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Project: Ghana Emergency Medicine Collaborative, 2013
Document Title: Hematologic and Oncologic Emergencies
Author(s): Joe Lex, MD, FACEP, FAAEM, MAAEM (Temple University)
License: Unless otherwise noted, this material is made available under the terms of the Creative Commons Attribution Share Alike-3.0 License: http://creativecommons.org/licenses/by-sa/3.0/
We have reviewed this material in accordance with U.S. Copyright Law and have tried to maximize your ability to use, share, and adapt it. These lectures have been modified in the process of making a publicly shareable version. The citation key on the following slide provides information about how you may share and adapt this material.
Copyright holders of content included in this material should contact [email protected] with any questions, corrections, or clarification regarding the use of content.
For more information about how to cite these materials visit http://open.umich.edu/privacy-and-terms-use.
Any medical information in this material is intended to inform and educate and is not a tool for self-diagnosis or a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional. Please speak to your physician if you have questions about your medical condition.
Viewer discretion is advised: Some medical content is graphic and may not be suitable for all viewers.
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Hematologic and Oncologic Hematologic and Oncologic EmergenciesEmergencies
Joe Lex, MD, FACEP, FAAEM, MAAEMJoe Lex, MD, FACEP, FAAEM, MAAEMAssociate Professor of Emergency Medicine, Associate Professor of Emergency Medicine,
Department of Emergency MedicineDepartment of Emergency Medicine
Temple University School of MedicineTemple University School of Medicine
Philadelphia, PA USAPhiladelphia, PA USA
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MenuMenu
• TransfusionsTransfusions
• Bleeding disordersBleeding disorders
• Platelet disordersPlatelet disorders
• Red cell disordersRed cell disorders
• White cell disordersWhite cell disorders
• Oncologic emergenciesOncologic emergencies
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QuestionsQuestions
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Who Needs Blood?Who Needs Blood?
• Most patients uncomfortable if Most patients uncomfortable if hemoglobin concentration <7 g/dLhemoglobin concentration <7 g/dL
• Patients with heart or pulmonary Patients with heart or pulmonary disease may need hemoglobin disease may need hemoglobin concentration of concentration of 10 g/dL to 10 g/dL to safeguard adequate oxygen safeguard adequate oxygen transporttransport
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WhatWhat’’s Available? s Available?
• Packed Red Blood Cells: Packed Red Blood Cells: centrifuged whole blood with 80% centrifuged whole blood with 80% of plasma removedof plasma removed
• Adult: 1 unit PRBC raises Adult: 1 unit PRBC raises hemoglobin by 1 gm/dLhemoglobin by 1 gm/dL
• Child: Each mL/kg raises Child: Each mL/kg raises hemoglobin by 1 gm/dLhemoglobin by 1 gm/dL
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WhatWhat’’s Available? s Available?
• Platelets: shelf life ~5 daysPlatelets: shelf life ~5 days– ABO / Rh checks are neededABO / Rh checks are needed
– ““Six packSix pack”” raises count by ~30,000 raises count by ~30,000
• Fresh Frozen Plasma: ~1 yearFresh Frozen Plasma: ~1 year– 1 unit / mL of each coagulation factor1 unit / mL of each coagulation factor
– No cellular componentsNo cellular components
– 10–15 mL / kg (3–4 250 mL bags)10–15 mL / kg (3–4 250 mL bags)
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Special NeedsSpecial Needs
• All blood products now leukocyte-All blood products now leukocyte-depleteddepleted
• Washed cell products in patients Washed cell products in patients with confirmed deficiency of IgAwith confirmed deficiency of IgA
• Irradiated cell products used to Irradiated cell products used to prevent graft-versus-host reactions prevent graft-versus-host reactions in immunosuppressed patientsin immunosuppressed patients
• Hypothermia from cold bloodHypothermia from cold blood
• Volume overloadVolume overload 20
Delayed HemolyticDelayed Hemolytic
• Extravascular: antibodies react to Extravascular: antibodies react to non-ABO antigensnon-ABO antigens
• Antibody-coated RBCs removed Antibody-coated RBCs removed from circulation by liver and spleen from circulation by liver and spleen before they are lysed before they are lysed extravascular extravascular no no hemoglobinemia, hemoglobinuria hemoglobinemia, hemoglobinuria
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Delayed HemolyticDelayed Hemolytic
• Typically asymptomatic, may have Typically asymptomatic, may have feverfever
• Same workup as acute Same workup as acute intravascular hemolytic reaction intravascular hemolytic reaction
• Acute respiratory distress, often Acute respiratory distress, often associated with fever, non-associated with fever, non-cardiogenic pulmonary edema, and cardiogenic pulmonary edema, and hypotensionhypotension
• ~1 in 2000 transfusions~1 in 2000 transfusions23
8.8.Your patient is profoundly anemic and Your patient is profoundly anemic and you believe she would benefit from a you believe she would benefit from a blood transfusion. In weighing benefits blood transfusion. In weighing benefits against risks, you tell her that the most against risks, you tell her that the most common adverse effect is: common adverse effect is:
a.a. hepatitis C transmission.hepatitis C transmission.b.b. hepatitis B transmission.hepatitis B transmission.c.c. human immunodeficiency virus (HIV) human immunodeficiency virus (HIV)
transmission.transmission.d.d. febrile nonhemolytic reaction. febrile nonhemolytic reaction. e.e. graft versus host reaction.graft versus host reaction.
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8.8.Your patient is profoundly anemic and Your patient is profoundly anemic and you believe she would benefit from a you believe she would benefit from a blood transfusion. In weighing benefits blood transfusion. In weighing benefits against risks, you tell her that the most against risks, you tell her that the most common adverse effect is: common adverse effect is:
a.a. hepatitis C transmission.hepatitis C transmission.b.b. hepatitis B transmission.hepatitis B transmission.c.c. human immunodeficiency virus (HIV) human immunodeficiency virus (HIV)
transmission.transmission.d.d. febrile nonhemolytic reaction. febrile nonhemolytic reaction. e.e. graft versus host reaction.graft versus host reaction.
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8.8. Most common transfusion adverseMost common transfusion adverse
Febrile non-hemolytic reaction is Febrile non-hemolytic reaction is estimated to occur once for every 200 estimated to occur once for every 200 units transfused. During the units transfused. During the transfusion or within a few hours after transfusion or within a few hours after its completion, the patient has a its completion, the patient has a temperature elevation of at least 1°C temperature elevation of at least 1°C and usually has chills. The usual and usually has chills. The usual cause of this febrile reaction is an cause of this febrile reaction is an antigen-antibody reaction involving the antigen-antibody reaction involving the plasma, platelets, or white blood cells plasma, platelets, or white blood cells that are passively transfused to the that are passively transfused to the recipient along with the RBCs.recipient along with the RBCs. 30
8.8. Most common transfusion adverseMost common transfusion adverse
Once a febrile transfusion reaction is Once a febrile transfusion reaction is recognized, the current transfusion recognized, the current transfusion should be terminated because there is should be terminated because there is not sufficient clinical evidence to not sufficient clinical evidence to differentiate the simple febrile differentiate the simple febrile nonhemolytic reaction from the more nonhemolytic reaction from the more serious immediate hemolytic reaction.serious immediate hemolytic reaction.
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Thrombus FormationThrombus Formation
• Damaged epithelium Damaged epithelium collagen collagen and tissue factor (TF) exposed to and tissue factor (TF) exposed to flowing blood flowing blood adherence / adherence / activation of platelets (relies on von activation of platelets (relies on von Willebrand Factor [vWF])Willebrand Factor [vWF])
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Thrombus FormationThrombus Formation
• Tissue factor activates coagulation Tissue factor activates coagulation cascade cascade generates thrombin generates thrombin converts fibrinogen to fibrin converts fibrinogen to fibrin forms long, cross-linking strands forms long, cross-linking strands that trap platelets and RBCs to that trap platelets and RBCs to form insoluble clotform insoluble clot
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Thrombus FormationThrombus Formation
• Thrombin also triggers tissue Thrombin also triggers tissue plasminogen activator plasminogen activator activates activates plasminplasmin
• Plasmin, anti-thrombin III, protein C Plasmin, anti-thrombin III, protein C & protein S cause fibrinolysis& protein S cause fibrinolysis
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WarfarinWarfarin
• Inhibits Vitamin K-dependent Inhibits Vitamin K-dependent coagulation factors II, VII, IX, Xcoagulation factors II, VII, IX, X– Half-lives 7h (FVII) to 50h (FII)Half-lives 7h (FVII) to 50h (FII)
• Inhibits Vitamin K-dependent Inhibits Vitamin K-dependent anticoagulants Protein C & Santicoagulants Protein C & S– Half-lives up to 40hHalf-lives up to 40h
• 11stst few days: may be procoaguable few days: may be procoaguable
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Warfarin Overdose: No BleedWarfarin Overdose: No Bleed
• INR <5: omit next warfarin or INR <5: omit next warfarin or dosedose
• INR 5 – 9: omit next 1-2 doses, INR 5 – 9: omit next 1-2 doses, give oral vitamin K (1 – 2.5 mg)give oral vitamin K (1 – 2.5 mg)
• INR >9: omit warfarin, give oral INR >9: omit warfarin, give oral vitamin K (2.5 – 5 mg)vitamin K (2.5 – 5 mg)
• Avoid subcutaneous vitamin KAvoid subcutaneous vitamin K
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Warfarin Overdose: BleedWarfarin Overdose: Bleed
• Vitamin K 10 mg slow IV infusionVitamin K 10 mg slow IV infusion
• Fresh frozen plasma (FFP): start Fresh frozen plasma (FFP): start with 4 – 6 unitswith 4 – 6 units– Each ml of FFP contains 1unit of Each ml of FFP contains 1unit of
each coagulation factoreach coagulation factor
– INR will not correct beyond ~1.5INR will not correct beyond ~1.5
• Heparin activates antithrombin IIIHeparin activates antithrombin III
• Short half-life: stopping drip will Short half-life: stopping drip will normalize in few hoursnormalize in few hours
• Can reverse with protamine sulfate: Can reverse with protamine sulfate: 1mg IV for every 100u heparin 1mg IV for every 100u heparin unfractionated given in prior 2 hrsunfractionated given in prior 2 hrs– Works a little for LMWHWorks a little for LMWH
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Liver DiseaseLiver Disease
• Liver synthesizes nearly all clotting Liver synthesizes nearly all clotting factorsfactors
PT/INR do not correlate well PT/INR do not correlate well with risk of bleedingwith risk of bleeding
• Can reverse with FFP if active Can reverse with FFP if active bleeding, require invasive bleeding, require invasive procedureprocedure
• In ED, assume baseline plasma In ED, assume baseline plasma level to be zerolevel to be zero
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Replacement FactorsReplacement Factors
• Can be recombinant or derived Can be recombinant or derived from human plasmafrom human plasma
• Hemophilia A: mild to moderate Hemophilia A: mild to moderate start with DDAVPstart with DDAVP– Causes release of vWF and FVIII Causes release of vWF and FVIII
5.5.The initial dose of factor VIII required The initial dose of factor VIII required for a 60-kg male with severe for a 60-kg male with severe hemophilia A in whom you suspect a hemophilia A in whom you suspect a ruptured spleen is:ruptured spleen is:
a.a. 1,500 units1,500 unitsb.b. 2,850 units2,850 unitsc.c. 3,000 units3,000 unitsd.d. 6,000 units6,000 unitse.e. 5,700 units 5,700 units
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5.5.The initial dose of factor VIII required The initial dose of factor VIII required for a 60-kg male with severe for a 60-kg male with severe hemophilia A in whom you suspect a hemophilia A in whom you suspect a ruptured spleen is:ruptured spleen is:
a.a. 1,500 units1,500 unitsb.b. 2,850 units2,850 unitsc.c. 3,000 units3,000 unitsd.d. 6,000 units6,000 unitse.e. 5,700 units 5,700 units
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5.5. … …initial dose of factor VIII … severe initial dose of factor VIII … severe hemophilia A … ruptured spleen … 3,000 hemophilia A … ruptured spleen … 3,000 units.units.
A 60-kg patient with a life threatening A 60-kg patient with a life threatening hemorrhage who requires 100% hemorrhage who requires 100% correction will need 50 mL/kg = 3000 correction will need 50 mL/kg = 3000 units of factor VIII units of factor VIII
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von Willebrand Diseasevon Willebrand Disease
• Mucocutaneous bleeding more Mucocutaneous bleeding more likelylikely
• Hemarthrosis less likelyHemarthrosis less likely
• DDAVP only effective in Type 1DDAVP only effective in Type 1
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15.15.A 38-year-old woman has von A 38-year-old woman has von WillebrandWillebrand’’s disease, Type I. She s disease, Type I. She complains of blood-tinged emesis and complains of blood-tinged emesis and epigastric pain. Her stool tests weakly epigastric pain. Her stool tests weakly positive for blood. Appropriate initial positive for blood. Appropriate initial therapy includes:therapy includes:
a.a. vitamin K.vitamin K.b.b. 6 units platelet concentrate.6 units platelet concentrate.c.c. factor IX concentrate.factor IX concentrate.d.d. desmopressin (DDAVP).desmopressin (DDAVP).e.e. plasmapheresis.plasmapheresis.
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15.15.A 38-year-old woman has von A 38-year-old woman has von WillebrandWillebrand’’s disease, Type I. She s disease, Type I. She complains of blood-tinged emesis and complains of blood-tinged emesis and epigastric pain. Her stool tests weakly epigastric pain. Her stool tests weakly positive for blood. Appropriate initial positive for blood. Appropriate initial therapy includes:therapy includes:
a.a. vitamin K.vitamin K.b.b. 6 units platelet concentrate.6 units platelet concentrate.c.c. factor IX concentrate.factor IX concentrate.d.d. desmopressin (DDAVP).desmopressin (DDAVP).e.e. plasmapheresis.plasmapheresis.
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15.15.von Willebrandvon Willebrand’’s disease, Type I ... s disease, Type I ... blood-tinged emesis … initial therapy…blood-tinged emesis … initial therapy…
Treatment of von WillebrandTreatment of von Willebrand’’s disease s disease depends on the type of disease that is depends on the type of disease that is present and the severity of bleeding. present and the severity of bleeding. Desmopressin (DDAVP) treatment has Desmopressin (DDAVP) treatment has benefit in patients with mild to benefit in patients with mild to moderately severe von Willebrandmoderately severe von Willebrand’’s s disease, but should be given in disease, but should be given in consultation with a hematologist. consultation with a hematologist. Factor VIII (cryoprecipitate) or fresh Factor VIII (cryoprecipitate) or fresh frozen plasma may be used in patients frozen plasma may be used in patients with severe bleeding. with severe bleeding.
• Clotting-predominant: heparinClotting-predominant: heparin– More common in chronic DICMore common in chronic DIC
– Not appropriate for acute DICNot appropriate for acute DIC
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3.3.The most helpful lab study in diagnosing The most helpful lab study in diagnosing disseminated intravascular coagulation is disseminated intravascular coagulation is the:the:
a.a. D-dimer, which is elevated.D-dimer, which is elevated.
b.b. partial thromboplastin time (PTT), which partial thromboplastin time (PTT), which is decreased.is decreased.
c.c. fibrinogen level, which is elevated.fibrinogen level, which is elevated.
d.d. prothrombin time, which is prolonged.prothrombin time, which is prolonged.
e.e. fibrin degradation products (FDP), which fibrin degradation products (FDP), which are diminished.are diminished.
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3.3.The most helpful lab study in diagnosing The most helpful lab study in diagnosing disseminated intravascular coagulation is disseminated intravascular coagulation is the:the:
a.a. D-dimer, which is elevated.D-dimer, which is elevated.
b.b. partial thromboplastin time (PTT), which partial thromboplastin time (PTT), which is decreased.is decreased.
c.c. fibrinogen level, which is elevated.fibrinogen level, which is elevated.
d.d. prothrombin time, which is prothrombin time, which is prolonged.prolonged.
e.e. fibrin degradation products (FDP), which fibrin degradation products (FDP), which are diminished.are diminished. 59
3.3.Useful labs in DICUseful labs in DIC
MOST USEFULMOST USEFUL::• PT / INR – PT / INR – • Platelet count – usually Platelet count – usually • Fibrinogen level – Fibrinogen level – HELPFULHELPFUL::• aPTT – usually aPTT – usually • Thrombin clot time – Thrombin clot time – • Fragmented RBCs – presentFragmented RBCs – present• FDP and D-dimers – FDP and D-dimers –
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10.10.The cornerstone of Emergency The cornerstone of Emergency Department management of DIC is:Department management of DIC is:
a.a. hemodynamic stabilization and hemodynamic stabilization and treatment of the underlying disorder.treatment of the underlying disorder.
b.b. rapid correction of thrombocytopenia.rapid correction of thrombocytopenia.c.c. aggressive resuscitation with colloid.aggressive resuscitation with colloid.d.d. pan-culture and broad-spectrum pan-culture and broad-spectrum
antibiotic coverage.antibiotic coverage.e.e. rapid intubation and hyperventilation.rapid intubation and hyperventilation.
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10.10.The cornerstone of Emergency The cornerstone of Emergency Department management of DIC is:Department management of DIC is:
a.a. hemodynamic stabilization and hemodynamic stabilization and treatment of the underlying treatment of the underlying disorder.disorder.
b.b. rapid correction of thrombocytopenia.rapid correction of thrombocytopenia.c.c. aggressive resuscitation with colloid.aggressive resuscitation with colloid.d.d. pan-culture and broad-spectrum pan-culture and broad-spectrum
antibiotic coverage.antibiotic coverage.e.e. rapid intubation and hyperventilation.rapid intubation and hyperventilation.
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10.10.Cornerstone of managing DICCornerstone of managing DIC
The primary cause of the DIC needs to be The primary cause of the DIC needs to be determined and treated. The high determined and treated. The high mortality rate in severe DIC is primarily mortality rate in severe DIC is primarily due to the underlying disorder. Many due to the underlying disorder. Many patients with DIC require no specific patients with DIC require no specific therapy if there is no evidence of therapy if there is no evidence of bleeding, or if thrombosis and laboratory bleeding, or if thrombosis and laboratory studies are not deteriorating. The first studies are not deteriorating. The first principle of management is to stabilize principle of management is to stabilize the patient hemodynamically, providing the patient hemodynamically, providing oxygen, fluids, and life support as oxygen, fluids, and life support as needed.needed.
• Platelet count >50,000: minimal Platelet count >50,000: minimal needed to avoid bleeding in trauma needed to avoid bleeding in trauma / surgery/ surgery
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PlateletsPlatelets
• Aspirin blocks COX receptors for Aspirin blocks COX receptors for life of plateletlife of platelet
• Withhold aspirin for 1 day Withhold aspirin for 1 day ~25,000 active platelets~25,000 active platelets
• Withhold aspirin for 2 days Withhold aspirin for 2 days ~50,000 active platelets~50,000 active platelets
• NSAIDs block COX receptors for NSAIDs block COX receptors for life of NSAIDlife of NSAID (~4 – 8 hours) (~4 – 8 hours)
• Last resort: steroids, splenectomyLast resort: steroids, splenectomy
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6.6.A 44-year-old woman with a history of TTP, A 44-year-old woman with a history of TTP, in remission for 30 days, presents to the ED in remission for 30 days, presents to the ED complaining of lethargy. Laboratory results complaining of lethargy. Laboratory results would likely show:would likely show:
6.6.A 44-year-old woman with a history of TTP, A 44-year-old woman with a history of TTP, in remission for 30 days, presents to the ED in remission for 30 days, presents to the ED complaining of lethargy. Laboratory results complaining of lethargy. Laboratory results would likely show:would likely show:
In TTP, laboratory studies will show an In TTP, laboratory studies will show an anemia of variable degree, anemia of variable degree, LDH, LDH, reticulocytosis, reticulocytosis, indirect bilirubin, indirect bilirubin, negative Coombsnegative Coombs’’ test, and test, and schistocytes on peripheral smear. schistocytes on peripheral smear. Thrombocytopenia is often severe with Thrombocytopenia is often severe with the count <20,000/mL in 50% of the count <20,000/mL in 50% of patients. BUN and creatinine are patients. BUN and creatinine are typically typically . .
• Similar to TTP, but worse renal Similar to TTP, but worse renal dysfunction, less severe neurologic dysfunction, less severe neurologic symptomssymptoms– ~60% require hemodialysis~60% require hemodialysis
• ““TypicalTypical”” or or ““childhoodchildhood””: follows : follows acute bloody diarrheaacute bloody diarrhea– Most common: Most common: E coli O157:H7E coli O157:H7
– Usually 5 years and youngerUsually 5 years and younger76
TreatmentTreatment
• Plasma exchange not as effective Plasma exchange not as effective as with TTPas with TTP
• Avoid platelet transfusions as with Avoid platelet transfusions as with TTPTTP
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1.1. A patient with anemia, thrombocytopenia, A patient with anemia, thrombocytopenia, renal failure, normal coagulation tests and a renal failure, normal coagulation tests and a clear sensorium probably has:clear sensorium probably has:
e.e. autoimmune hemolytic anemia.autoimmune hemolytic anemia.
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1.1.A patient with anemia, thrombocytopenia, A patient with anemia, thrombocytopenia, renal failure, normal coagulation tests and a renal failure, normal coagulation tests and a clear sensorium probably has:clear sensorium probably has:
ITP generally presents with isolated ITP generally presents with isolated thrombocytopenia. TTP causes thrombocytopenia. TTP causes neurologic symptoms in addition to the neurologic symptoms in addition to the other symptoms. DIC will have other symptoms. DIC will have abnormal coagulation studies. abnormal coagulation studies. Autoimmune hemolytic anemia may Autoimmune hemolytic anemia may cause severe rapid anemia, which may cause severe rapid anemia, which may present with angina or congestive heart present with angina or congestive heart failure. failure.
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4.4.Hemolytic-uremic syndrome is most Hemolytic-uremic syndrome is most commonly seen in:commonly seen in:
a.a. neonates.neonates.b.b. infants and children 6 months to 4 infants and children 6 months to 4
years of age .years of age .c.c. adolescents.adolescents.d.d. women age 30 to 50.women age 30 to 50.e.e. both sexes, over age 75.both sexes, over age 75.
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4.4.Hemolytic-uremic syndrome is most Hemolytic-uremic syndrome is most commonly seen in:commonly seen in:
a.a. neonates.neonates.b.b. infants and children 6 months to infants and children 6 months to
4 years of age.4 years of age.c.c. adolescents.adolescents.d.d. women age 30 to 50.women age 30 to 50.e.e. both sexes, over age 75.both sexes, over age 75.
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4.4.Hemolytic-uremic syndrome is most Hemolytic-uremic syndrome is most commonly seen in infants and children.commonly seen in infants and children.
Hemolytic-uremic syndrome (HUS) is a Hemolytic-uremic syndrome (HUS) is a disease mainly of infancy and early disease mainly of infancy and early childhood, with a peak incidence childhood, with a peak incidence between 6 months and 4 years of age.between 6 months and 4 years of age.
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• More common with unfractionatedMore common with unfractionated
a.a. does not occur with low molecular does not occur with low molecular weight heparins.weight heparins.
b.b. requires a minimum number of units, requires a minimum number of units, so a heparin so a heparin ““flushflush”” is always safe. is always safe.
c.c. can paradoxically cause can paradoxically cause thrombosis, ischemia, and thrombosis, ischemia, and amputation.amputation.
d.d. never occurs during the first 24 hours never occurs during the first 24 hours of infusion.of infusion.
e.e. is easily treated with warfarin and is easily treated with warfarin and fresh-frozen plasma.fresh-frozen plasma.
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11.11.HIT thrombosisHIT thrombosis
Heparin-induced thrombocytopenia Heparin-induced thrombocytopenia (HIT) is due to an antibody, usually IgG, (HIT) is due to an antibody, usually IgG, which attaches to and stimulates which attaches to and stimulates platelets. This platelet activation platelets. This platelet activation produces both thrombocytopenia and a produces both thrombocytopenia and a tendency for thrombosis. The tendency for thrombosis. The incidence of HIT is between 1 and 3% incidence of HIT is between 1 and 3% in patients treated with unfractionated in patients treated with unfractionated heparin but significantly less in patients heparin but significantly less in patients treated with LMW products.treated with LMW products.
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11.11.HIT thrombosisHIT thrombosis
The onset of HIT is usually 5 to 12 days The onset of HIT is usually 5 to 12 days after heparin treatment is started but after heparin treatment is started but may be sooner for patients who may be sooner for patients who developed the antibody from a previous developed the antibody from a previous exposure. Thrombosis may involve the exposure. Thrombosis may involve the skin (similar to warfarin-induced skin (similar to warfarin-induced cutaneous necrosis), the arteries (e.g., cutaneous necrosis), the arteries (e.g., femoral artery thrombosis), or the veins femoral artery thrombosis), or the veins (e.g., recurrent DVT or PE). (e.g., recurrent DVT or PE).
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• Acute in children (age 2 – 6)Acute in children (age 2 – 6)– Viral prodromeViral prodrome
– Self-limited: remission in 90%Self-limited: remission in 90%
• Diagnosis: thrombocytopenia on Diagnosis: thrombocytopenia on CBCCBC
Signs and SymptomsSigns and Symptoms
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• Steroids (?)Steroids (?)
• If refractory: splenectomy or If refractory: splenectomy or immunosuppressive therapyimmunosuppressive therapy
• Platelet transfusion ONLY if serious Platelet transfusion ONLY if serious bleedingbleeding
• Intravenous immunoglobulin G Intravenous immunoglobulin G (IVIG) in children with intracranial (IVIG) in children with intracranial hemorrhage (rare)hemorrhage (rare)
TreatmentTreatment
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14.14.A 5-year-old girl complains of weakness A 5-year-old girl complains of weakness and fatigue. Two weeks ago she saw her and fatigue. Two weeks ago she saw her family doctor and was diagnosed with an family doctor and was diagnosed with an upper respiratory infection. She received no upper respiratory infection. She received no medicine at that time. Physical examination medicine at that time. Physical examination shows only a scattered petechial rash shows only a scattered petechial rash located in areas where her clothing is snug located in areas where her clothing is snug against her skin, such as underwear elastic against her skin, such as underwear elastic lines. Laboratory studies show a white lines. Laboratory studies show a white blood cell count 11,000/mmblood cell count 11,000/mm33 , Hgb 10.5 , Hgb 10.5 mg/dL, and platelet count of 16,000/mmmg/dL, and platelet count of 16,000/mm33. . Appropriate management of this patient Appropriate management of this patient should be: should be: 93
14.14. Recent URI … petechial rash … Recent URI … petechial rash … platelet count of 16,000/mmplatelet count of 16,000/mm3.3.
a.a. platelet concentrate transfusion.platelet concentrate transfusion.b.b. discharge home with instructions to discharge home with instructions to
limit contact sports.limit contact sports.c.c. admit for salicylate therapy.admit for salicylate therapy.d.d. admit for splenectomy.admit for splenectomy.e.e. admit for observation.admit for observation.
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14.14. Recent URI … petechial rash … Recent URI … petechial rash … platelet count of 16,000/mmplatelet count of 16,000/mm3.3.
a.a. platelet concentrate transfusion.platelet concentrate transfusion.b.b. discharge home with instructions to discharge home with instructions to
limit contact sports.limit contact sports.c.c. admit for salicylate therapy.admit for salicylate therapy.d.d. admit for splenectomy.admit for splenectomy.e.e. admit for observation.admit for observation.
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14.14. Recent URI … petechial rash … Recent URI … petechial rash … platelet count of 16,000/mmplatelet count of 16,000/mm3.3.
Acute idiopathic thrombocytopenic Acute idiopathic thrombocytopenic purpura (ITP) is seen most often in purpura (ITP) is seen most often in children 2 to 6 years old. A viral children 2 to 6 years old. A viral prodrome is common, usually within 3 prodrome is common, usually within 3 weeks of the onset. The platelet count weeks of the onset. The platelet count falls, usually to <20,000/mmfalls, usually to <20,000/mm33. The . The course is self-limited, with a greater course is self-limited, with a greater than 90% rate of spontaneous than 90% rate of spontaneous remission. Morbidity and mortality are remission. Morbidity and mortality are low. Treatment is supportive. Steroid low. Treatment is supportive. Steroid therapy does not alter disease course. therapy does not alter disease course.
• Proliferation of lymphoid cells from Proliferation of lymphoid cells from lymph nodes OR lymphatic tissue lymph nodes OR lymphatic tissue other than bone marrowother than bone marrow
• B-Cell, T-Cell, NK-Cell: can be B-Cell, T-Cell, NK-Cell: can be indolent or aggressiveindolent or aggressive
• Bone marrow diseases: myelo-Bone marrow diseases: myelo-dysplasia, myelofibrosis, some dysplasia, myelofibrosis, some leukemias and lymphomasleukemias and lymphomas
• Pancytopenia with hypocellular Pancytopenia with hypocellular bone marrowbone marrow– Acquired: marrow stem cells Acquired: marrow stem cells
damaged by drugs, radiation, virus, damaged by drugs, radiation, virus, chemical, immune-relatedchemical, immune-related
– Inherited: FanconiInherited: Fanconi’’s anemia, s anemia, dyskeratosis congenitadyskeratosis congenita
Aplastic AnemiaAplastic Anemia
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• Bleeding, easy bruisingBleeding, easy bruising
• Fatigue, malaise, anemia Fatigue, malaise, anemia short short of breathof breath
• Infection commonInfection common
Signs & SymptomsSigns & Symptoms
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• CBC, reticulocyte countCBC, reticulocyte count
• Bone marrow biopsyBone marrow biopsy
• Immediate: depends on severity of Immediate: depends on severity of anemia / thrombocytopenia / anemia / thrombocytopenia / neutropenia ± complicationsneutropenia ± complications
• Bone marrow transplantBone marrow transplant
Diagnosis / TreatmentDiagnosis / Treatment
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Red Blood Cells (RBC)Red Blood Cells (RBC)
• Normal life: ~120 daysNormal life: ~120 days
• Marrow releases ~1% total count Marrow releases ~1% total count dailydaily– ““baby red cellsbaby red cells”” = nucleated = = nucleated =
reticulocytesreticulocytes
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7.7.The most helpful laboratory study to The most helpful laboratory study to differentiate poor red blood cell production differentiate poor red blood cell production from increased red cell destruction is the:from increased red cell destruction is the:
a.a. sedimentation rate.sedimentation rate.b.b. sideroblast level.sideroblast level.c.c. serum iron level.serum iron level.d.d. total to direct bilirubin ratio.total to direct bilirubin ratio.e.e. reticulocyte count.reticulocyte count.
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7.7.The most helpful laboratory study to The most helpful laboratory study to differentiate poor red blood cell production differentiate poor red blood cell production from increased red cell destruction is the:from increased red cell destruction is the:
a.a. sedimentation rate.sedimentation rate.b.b. sideroblast level.sideroblast level.c.c. serum iron level.serum iron level.d.d. total to direct bilirubin ratio.total to direct bilirubin ratio.e.e. reticulocyte count.reticulocyte count.
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7.7. production vs production vs destruction destruction
Reticulocytes are RBCs of intermediate Reticulocytes are RBCs of intermediate maturity. They are an index of the maturity. They are an index of the production of mature RBCs by the bone production of mature RBCs by the bone marrow (reported as a percent of total marrow (reported as a percent of total RBCs). RBCs). reticulocyte count reflects reticulocyte count reflects impaired RBC production; seen with impaired RBC production; seen with low levels of iron, vitamin B12, folate, low levels of iron, vitamin B12, folate, bone marrow failure. bone marrow failure. reticulocyte reticulocyte count reflects accelerated count reflects accelerated erythropoeisis, the normal marrow erythropoeisis, the normal marrow response to anemia; seen with blood response to anemia; seen with blood loss and hemolytic anemias. loss and hemolytic anemias. 109
Aplastic AnemiaAplastic Anemia
• Normal MCV with Normal MCV with reticulocyte reticulocyte
• Can be RBC aplasia aloneCan be RBC aplasia alone
• New infiltrate on chest x-ray + New infiltrate on chest x-ray + fever, cough, fever, cough, sputum, dyspnea, sputum, dyspnea, tachypnea, hypoxiatachypnea, hypoxia
• Many causes: fat embolism from Many causes: fat embolism from marrow ischemia, pulmonary vaso-marrow ischemia, pulmonary vaso-occlusion, infection, venous occlusion, infection, venous thromboembolism, pulmonary thromboembolism, pulmonary edemaedema
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Acute Chest SyndromeAcute Chest Syndrome
• Children: fever & cough most Children: fever & cough most commoncommon
• Adults: fever & chest pain as Adults: fever & chest pain as presenting complaintpresenting complaint– Some develop 2 – 3 days into Some develop 2 – 3 days into
hospital stayhospital stay
• ± hypoxemia± hypoxemia
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Acute Chest SyndromeAcute Chest Syndrome
• Chest x-ray normal in ~50%Chest x-ray normal in ~50%
• Priapism: low-flow due to corpora Priapism: low-flow due to corpora cavernosa occlusioncavernosa occlusion
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2.2.The viral agent implicated in an aplastic The viral agent implicated in an aplastic crisis of patients with sickle cell disease is:crisis of patients with sickle cell disease is:
a.a. adenovirus (atypical).adenovirus (atypical).
b.b. herpes simplex.herpes simplex.
c.c. parvovirus.parvovirus.
d.d. coxsackie virus.coxsackie virus.
e.e. HTLV-IV.HTLV-IV.
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2.2.The viral agent implicated in an aplastic The viral agent implicated in an aplastic crisis of patients with sickle cell disease is:crisis of patients with sickle cell disease is:
Aplastic crises can be precipitated by Aplastic crises can be precipitated by viral infections (particularly parvovirus viral infections (particularly parvovirus B19), folic acid deficiency, or the B19), folic acid deficiency, or the ingestion of bone marrow toxins such ingestion of bone marrow toxins such as phenylbutazone. Bone marrow as phenylbutazone. Bone marrow erythropoiesis is slowed or stopped. erythropoiesis is slowed or stopped. The hematocrit falls to as low as 10%, The hematocrit falls to as low as 10%, and the reticulocyte count falls to as and the reticulocyte count falls to as low as 0.5%. The white blood cell low as 0.5%. The white blood cell count and platelet counts usually count and platelet counts usually remain stable. remain stable. 124
9.9.A 12-year-old girl with sickle cell disease is A 12-year-old girl with sickle cell disease is brought by her mother after she passed out brought by her mother after she passed out twice. She was kept home from school the twice. She was kept home from school the last few days for a last few days for a ““cold.cold.”” When you ask the When you ask the child to stand, you must catch her to prevent child to stand, you must catch her to prevent her from falling to the ground. This is her from falling to the ground. This is suspicious for:suspicious for:a.a. salmonella sepsis.salmonella sepsis.b.b. sequestration crisis.sequestration crisis.c.c. acute chest syndrome.acute chest syndrome.d.d. aplastic crisis.aplastic crisis.e.e. hemolytic crisis.hemolytic crisis.
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9.9.A 12-year-old girl with sickle cell disease is A 12-year-old girl with sickle cell disease is brought by her mother after she passed out brought by her mother after she passed out twice. She was kept home from school the twice. She was kept home from school the last few days for a last few days for a ““cold.cold.”” When you ask the When you ask the child to stand, you must catch her to prevent child to stand, you must catch her to prevent her from falling to the ground. This is her from falling to the ground. This is suspicious for:suspicious for:a.a. salmonella sepsis.salmonella sepsis.b.b. sequestration crisis.sequestration crisis.c.c. acute chest syndrome.acute chest syndrome.d.d. aplastic crisis.aplastic crisis.e.e. hemolytic crisis.hemolytic crisis.
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9.9.Sequestration crisisSequestration crisis
Sequestration crisis occurs primarily in Sequestration crisis occurs primarily in children and is the second most children and is the second most common cause of death in children with common cause of death in children with SCD under the age of 5 years. Often SCD under the age of 5 years. Often preceded by viral infections, sickled preceded by viral infections, sickled cells block the splenic outflow, causing cells block the splenic outflow, causing pooling of peripheral blood and sickled pooling of peripheral blood and sickled cells in the spleen. Such patients cells in the spleen. Such patients present in hypovolemic shock with an present in hypovolemic shock with an enlarged spleen. enlarged spleen.
• Reticulocytes should be Reticulocytes should be • Haptoglobin: binds free HgbHaptoglobin: binds free Hgb • Bilirubin Bilirubin • LDH LDH (released from RBCs) (released from RBCs)
iron iron iron deficiency iron deficiency globin globin thalassemia thalassemia porphyrin porphyrin sideroblastic, lead sideroblastic, lead
toxicitytoxicity• Chronic diseaseChronic disease
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13.13. A healthy 12-year-old African-A healthy 12-year-old African-American female complains of American female complains of weakness and fatigue 3 days after weakness and fatigue 3 days after starting a course of trimethoprim-starting a course of trimethoprim-sulfamethoxasole and pyridium for sulfamethoxasole and pyridium for a urinary tract infection. Her a urinary tract infection. Her hemoglobin is 4.8 mg/dl, and her hemoglobin is 4.8 mg/dl, and her urine is tea-colored, but you see no urine is tea-colored, but you see no red blood cells on microscopic red blood cells on microscopic exam. She probably has exam. She probably has undiagnosed:undiagnosed:
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13.13.12-year-old African-American 12-year-old African-American female…weakness and fatigue… female…weakness and fatigue… TMP/SMZ … hgb 4.8 mg/dl … no TMP/SMZ … hgb 4.8 mg/dl … no RBCs in urine. Undiagnosed:RBCs in urine. Undiagnosed:a.a. hemolytic uremic syndrome.hemolytic uremic syndrome.b.b. G6PD deficiency.G6PD deficiency.c.c. idiopathic thrombocytopenic purpura.idiopathic thrombocytopenic purpura.d.d. thrombotic thrombocytopenic thrombotic thrombocytopenic
Deficiency of the RBC enzyme Deficiency of the RBC enzyme glucose-6-phosphate dehydrogenase glucose-6-phosphate dehydrogenase (G-6-PD) is the most common human (G-6-PD) is the most common human enzyme defect, affecting nearly one-enzyme defect, affecting nearly one-tenth of the worldtenth of the world’’s population. The s population. The RBC is unable to protect itself against RBC is unable to protect itself against oxidant stress. Acute hemolytic crises oxidant stress. Acute hemolytic crises occur that are incited by bacterial and occur that are incited by bacterial and viral infections, exposure to oxidant viral infections, exposure to oxidant drugs, metabolic acidosis (such as drugs, metabolic acidosis (such as diabetic ketoacidosis), and ingestion of diabetic ketoacidosis), and ingestion of fava beans in some patients.fava beans in some patients.
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13.13.G6PDG6PD
Within 1 to 3 days following oxidant Within 1 to 3 days following oxidant stress, the patient can develop stress, the patient can develop hemoglobinuria and the potential for hemoglobinuria and the potential for vascular collapse. These hemolytic vascular collapse. These hemolytic crises are generally well tolerated and crises are generally well tolerated and self-limited because only the older self-limited because only the older RBCs will hemolyze. The drugs most RBCs will hemolyze. The drugs most commonly associated with oxidant commonly associated with oxidant stress are sulfa drugs, antimalarials, stress are sulfa drugs, antimalarials, phenazopyridine, and nitrofurantoin. phenazopyridine, and nitrofurantoin.
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Diagnosis / TreatmentDiagnosis / Treatment
serum iron levelserum iron level serum ferritin levelserum ferritin level total iron binding capacity total iron binding capacity
(TIBC)(TIBC)• Treatment: iron supplements, Treatment: iron supplements,
• Polycythemia vera: Polycythemia vera: production production RBCs, WBCs, plateletsRBCs, WBCs, platelets– Can progress to myelofibrosis, Can progress to myelofibrosis,
RBCs, RBCs, WBCs, WBCs, plateletsplatelets• Confirmed by non-ED studiesConfirmed by non-ED studies• Treatment: phlebotomyTreatment: phlebotomy
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16.16. A 68-year-old man complains of A 68-year-old man complains of headache, dizziness, and blurred headache, dizziness, and blurred vision. His blood pressure is vision. His blood pressure is 190/118 mmHg. He has a florid 190/118 mmHg. He has a florid face, normal fundi, and marked face, normal fundi, and marked splenomegaly. His hematocrit is splenomegaly. His hematocrit is 67%. Reasonable therapy 67%. Reasonable therapy includes:includes:
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16.16. … … florid face … splenomegaly … florid face … splenomegaly … hematocrit = 67%. Therapy:hematocrit = 67%. Therapy:
16.16. … … florid face … splenomegaly … florid face … splenomegaly … hematocrit = 67%. Therapy:hematocrit = 67%. Therapy:
Emergency treatment of any form of Emergency treatment of any form of symptomatic polycythemia is symptomatic polycythemia is phlebotomy. Usually not more than phlebotomy. Usually not more than 500 ml of blood is slowly removed as 500 ml of blood is slowly removed as the volume is replaced with a the volume is replaced with a comparable amount of normal saline. comparable amount of normal saline.
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MethemoglobinemiaMethemoglobinemia
production or production or reductionreduction• Causes discussed in toxicologyCauses discussed in toxicology• Cyanosis with normal oxygen Cyanosis with normal oxygen
saturationsaturation• Treatment:Treatment:
– Congenital: usually noneCongenital: usually none– Toxic Toxic symptomatic symptomatic treat with treat with
intravenous methylene blueintravenous methylene blue
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LeukemiaLeukemia
production undifferentiated production undifferentiated hematopoietic stem cellshematopoietic stem cells
– Related to abnormal cells crowding Related to abnormal cells crowding out normal cells out normal cells RBC, RBC, WBC, WBC, plateletsplatelets
– Bacterial infections in 1/3 at time of Bacterial infections in 1/3 at time of diagnosisdiagnosis
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LeukemiaLeukemia
• Chronic Lymphocytic (CLL): most Chronic Lymphocytic (CLL): most common in patients >50 yearscommon in patients >50 years
• Often no symptoms: may have Often no symptoms: may have fatigue, large lymph nodes, fatigue, large lymph nodes, infections (esp. respiratory)infections (esp. respiratory)
• Abnormal Abnormal plasma cellsplasma cells• Fatigue from anemiaFatigue from anemia• Bone pain from osteolytic lesions or Bone pain from osteolytic lesions or
pathologic fracturespathologic fractures• Diagnosis: Diagnosis: calcium, calcium, creatininecreatinine• Bone marrow: >10% plasma cellsBone marrow: >10% plasma cells• Serum / urine protein electro-Serum / urine protein electro-
phoresis (SPEP, UPEP)phoresis (SPEP, UPEP)
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TreatmentTreatment
• ChemotherapyChemotherapy• Plasmapheresis for hyperviscosity Plasmapheresis for hyperviscosity
syndromesyndrome• If comatose: temporize by removing If comatose: temporize by removing
1 liter blood, replace with normal 1 liter blood, replace with normal salinesaline
calcium: IV saline, steroidscalcium: IV saline, steroids
abnormal serum proteinsabnormal serum proteins• Waldenstrom macroglobulinemiaWaldenstrom macroglobulinemia• Multiple myeloma (less common)Multiple myeloma (less common)• Most common symptoms: Most common symptoms:
neurologic, visualneurologic, visual• May see mucosal or GI bleedingMay see mucosal or GI bleeding• CHF from CHF from plasma volume plasma volume• Plasmapheresis, exchangePlasmapheresis, exchange
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LeukostasisLeukostasis
• WBC sludging in microcirculationWBC sludging in microcirculation• Usually acute leukemiaUsually acute leukemia• Can be seen with chronic, NHLCan be seen with chronic, NHL• Neurologic symptomsNeurologic symptoms• Can see respiratory failureCan see respiratory failure• Treatment: leukapheresis, Treatment: leukapheresis,
• Acute renal failure: possible Acute renal failure: possible hemodialysis; do NOT alkalinize hemodialysis; do NOT alkalinize urine, worsens urine, worsens phosphorus and phosphorus and calciumcalcium
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OthersOthers
• Hypercalcemia: see Endocrine-Hypercalcemia: see Endocrine-Metabolic EmergenciesMetabolic Emergencies
12.12.A 42-year-old woman with adult T-cell A 42-year-old woman with adult T-cell lymphoma-leukemia complains of back pain, lymphoma-leukemia complains of back pain, abdominal pain, and confusion. Laboratory abdominal pain, and confusion. Laboratory evaluation shows a total calcium of 15.8 evaluation shows a total calcium of 15.8 mg/dl. Appropriate management of this mg/dl. Appropriate management of this patient should include:patient should include:
a.a. plasmapheresis.plasmapheresis.b.b. IV bicarbonate.IV bicarbonate.c.c. IV hypertonic saline / oral IV hypertonic saline / oral
Kayexalate®.Kayexalate®.d.d. IV normal saline and IV furosemide.IV normal saline and IV furosemide.e.e. glucagon.glucagon.
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12.12.A 42-year-old woman with adult T-cell A 42-year-old woman with adult T-cell lymphoma-leukemia complains of back pain, lymphoma-leukemia complains of back pain, abdominal pain, and confusion. Laboratory abdominal pain, and confusion. Laboratory evaluation shows a total calcium of 15.8 evaluation shows a total calcium of 15.8 mg/dl. Appropriate management of this mg/dl. Appropriate management of this patient should include:patient should include:a.a. plasmapheresis.plasmapheresis.b.b. IV bicarbonate.IV bicarbonate.c.c. IV hypertonic saline / oral Kayexalate®.IV hypertonic saline / oral Kayexalate®.
d.d. IV normal saline and IV IV normal saline and IV furosemide.furosemide.
e.e. glucagon.glucagon.
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12.12.Hypercalcemia of malignancyHypercalcemia of malignancy
Patients with severe hypercalcemia Patients with severe hypercalcemia (>14 mg/dl) require immediate (>14 mg/dl) require immediate treatment regardless of symptoms. treatment regardless of symptoms. The four basic goals of therapy areThe four basic goals of therapy are
12.12.Hypercalcemia of malignancyHypercalcemia of malignancy
Isotonic saline is the first step. Once Isotonic saline is the first step. Once volume is restored, the calcium will volume is restored, the calcium will usually have usually have by 1.6 to 2.4 mg/dl, but by 1.6 to 2.4 mg/dl, but hydration alone rarely leads to hydration alone rarely leads to complete normalization. Loop diuretics complete normalization. Loop diuretics inhibit resorption of calcium in the thick inhibit resorption of calcium in the thick ascending loop of Henle, ascending loop of Henle, the the calciuric effect of hydration. Volume calciuric effect of hydration. Volume expansion must precede administration expansion must precede administration of furosemide, because the drugof furosemide, because the drug’’s s effect depends on the delivery of effect depends on the delivery of calcium to the distal nephron. calcium to the distal nephron. 168
PearlsPearls
• The first step in managing any The first step in managing any transfusion reaction to to stop the transfusion reaction to to stop the transfusiontransfusion
• Hemarthrosis in hemophiliacs: Hemarthrosis in hemophiliacs: factor replacement, never factor replacement, never arthrocentesisarthrocentesis
• Minor head injury in hemophilia: Minor head injury in hemophilia: replace factor, head CT, admit for replace factor, head CT, admit for observationobservation
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PearlsPearls
• Initial treatment in mild-moderate Initial treatment in mild-moderate hemophilia A: DDAVPhemophilia A: DDAVP
• NO PLATELET TRANSFUSION in NO PLATELET TRANSFUSION in patients with TTP, HUSpatients with TTP, HUS
• Once a patient has HIT, no heparin Once a patient has HIT, no heparin can ever again be used, including can ever again be used, including LMWHLMWH