Bleeding disorders

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BLEEDING DISORDERS

Smrithi JP53 rd

• Idiopathic thrombocytopenic purpura• Hemophilia• Vitamin k deficiency• Neonatal alloimmune thrombocytopenia • Disseminated intravascular coagulation• Henoch- Schonlein Purpura

Idiopathic thrombocytopenic purpura

IDIOPATHIC THROMBOCYTOPENIC PURPURA

Pathogenesis• Overactive immune system

• Antigens- platelet glycoprotein IIa/ IIIb

• Platelets with antibodies- trapped in spleen- spleen macrophages remove them

• Directed against viral antigen

• Increased megakaryocytes in bone marrow

Clinical evaluationSite:• Appearance of mucosal

bleeding, bruises • Petechiae, ecchymosis

following trauma

Mode of onset:• Acute ITP: <6 months

• 60-75% patients

• Remission in 2-4 months

• Chronic ITP: >6 months• Relapsing course of c/c ITP • H/o any viral infection

Fanconi anemia

Thrombocytopenia with absent radii

EXAMINATION• Features of Fanconi anemia:

absent thumb, short stature, hyperpigmentation

• Thrombocytopenia with absent radii

• Hemangiomas• Splenomegaly and

lymphadenopathy

LAB DIAGNOSIS• CBC: decreased platelets• BT prolonged• CT, PT, aPTT normal• Peripheral smear: abnormal cells• RFT and LFT• LDH• Bone marrow• Other tests

MANAGEMENT

A/c ITP:• IVIG-1g/kg/d for 1-2 days• AntiD immunoglobulin 50-

75mg/kg• Steroids• Dexamethasone- 20mg/m2 for

4 days• Platelet transfusion• Supportive

C/C ITP

• Alternate low dose steroids

• Splenectomy• Danazole,vincristine, cyclosporine,azathioprine, rituximab

Hemarthrosis

HEMOPHILIA• Common hereditary clotting

disorder• X linked recessive• Hemophilia A: VIII deficiency• Hemophilia B: IX deficiency

• Presentation depends on the level of clotting factors

• Mild (5-40%)

bleeding after severe trauma

• Severe (<1%)

hemarthrosisintracranial bleeding

• Concentrate of factor VIII or IX• Cryoprecipitate• Fresh frozen plasma: 15U/kg 12-24

hrs for 1-2 days• Major bleeding: 50U/kg 8-12hrs 7-14

days• Alpha aminocaproic acid and

tranexemic acid

PROPHYLAXIS• Severe anemia: 2-3 times a week• Physiotherapy for joint• Counselling for injury prevention• Monitoring• Genetic counselling and prenatal

diagnosis

Vitamin K deficiency

Vitamin K deficiency

• Vitamin dependent clotting factors

• Green leafy vegetables, oil of soya bean

• New born: Low placental transmission Inadequate breast milk Sterile intestine, premature

liver

• Later :

Antibiotics Liver disease TPN Malabsorption

DIAGNOSIS

• PT and aPTT prolonged

• PIVKA

MANAGEMENT• Inj vit K 2-10 mg

PROPHYLAXIS• Inj vit k 1 mg s/c

Neonatal Alloimmune Thrombocytopenia

Neonatal Alloimmune Thrombocytopenia

• Fetal platelets destroyed by maternal antibodies

• HPA1, HPA 5b• 50% occur in first

pregnancy• Intracranial hemorrhage• Diagnosis of exclusion

MANAGEMENT• Transfusion of washed

maternal platelets• Close monitoring• Serial USG• Mother IVIG (1g/kg every 4

weeks)• Oral dexamethasone

DISSEMINATED INTRAVASCULAR COAGULATION

DISSEMINATED INTRAVASCULAR COAGULATION

• Acquired disorder of dysregulation of hemostasis

• Bleeding from multiple sites• Deposition of fibrin in micro

circulation• Consumption of platelets

and clotting factors

PATHOPHSIOLOGY

• Initiation of fibrin deposition• Amplification role of thrombin• Propagation of fibrin

deposition

• Wide spread deposition of fibrin• Ischemia• Microangiopathic hemolytic

anemia• Consumption• Activation of plasminogen

• Cleaves fibrin• Digest V and VIII• FDP inhibit platelet aggregation,fibrin

polymerisation,thrombin• Bleeding diathesis

Causes• Medical• Surgical• Transfusion medicine

Medical

• Septicemia• Fulminant hepatic failure• Heat stroke,burns• Malignancy• Reye syndrome• Hereditary protein c

deficiency• Snake bite• HUS• Kawasaki disease• Collagen Vascular disease

•Solid tumour•Liver cirrhosis•Kasabach Meritt Syndrome

•Bacterial•Viral•Parasitic•Mycotic•rickettsial

Surgical

• Severe renal graft rejection

• Severe trauma• Multiple fracture• Major operations• Brain injury

•Vascular tumour•Aortic aneurysm

Transfusion medicine

• Acute Transfusion reaction

• Massive transfusion

• Heparin induced thrombolysis

•Artificial surfaces

meningococcemia

Plasmodium falciparum

LABORATORY FINDING• Peripheral blood and

hemogram• Platelets decreased• PT, aPTT, TT prolonged• Fibrinogen decreased• D dimer increased

TREATMENT• Cause managed• IV fluids and O2

• Vitamin K• Replacement of deficient

compound• FFP, platelet concentrate,

cryopreciptate, packed blood cells

• Activated protein C

• Desirudin• Gabexate mesylate

Henoch- Schonlein Purpura

• Common vasculitides• Non thrombocytopenic purpura, transient arthralgia, abdominal symptoms

Manifestations

• Macular rash on lower extremities and buttocks

• Isolated hematuria, hypertension, nephritic/nephrotic syndrome• Intermittent colicky, periumbilical

abdominal pain• Vomiting

• Clinical diagnosis• Skin biopsy• Indirect immunofluorescence• USG abdomen

Treatment• Hydration and pain relief• Prednisolone Indications: Life Threatening

Intestinal Hemorrhage, Obstruction, Perforation, Intussusceptions

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