RBC Disorders Dr.CSBR.Prasad, M.D.,
RBC Disorders Dr.CSBR.Prasad, M.D.,
Clinical presentations
Fatigue
Pallor / Anemia
Spoon nails [Koilonychia]
Jaundice
Prolonged Neonatal jaundice
Hepatosplenomegaly
Hepatosplenomegaly
Delayed mile stones +organomegaly
Pigmented Gall stones in
young Persons
Ruddy facies of
polycytehmia
Some terms
• Anisocytosis • Poikilocytosis
– Schistocytes – Stomatocytes – Elliptocytes – Sickle cells – Spherocytes
• Polychromasia • Normocyte • Microcyte • Macrocyte • Rouleaux formation • RBC Agglutinates
Normocyte
Microcyte
Macrocyte
Spherocyte
Anisopoikilocytosis
Polychromatic cells
Hypochromia
RBC agglutinates
Rouleaux formation
The nucleated RBC in the center contains basophilic stippling of the cytoplasm. This suggests a toxic injury to the bone marrow. Such stippling may also appear with severe megaloblastic anemia.
Sickle cell anemia
RBC Indices
• HGB
• RBC count
• HCT
• MCV
• MCH
• MCHC
• RDW
Here is data from a CBC in a person with iron deficiency anemia. Note the low hemoglobin (HGB). Microcytosis is indicated by the low MCV (mean corpuscular volume). Hypochromia correlates here with the low MCH (mean corpuscular hemoglobin).
The CBC here shows a markedly increased MCV, typical for megaloblastic anemia. The MCV can be mildly increased in persons recovering from blood loss or hemolytic anemia, because the newly released RBC's, the reticulocytes, are increased in size over normal RBC's, which decrease in size slightly with aging.
This CBC demonstrates findings suggestive of spherocytosis, a condition in which the RBC's are small and round (rather than the normal biconcave appearance) with increased hemoglobin content. This is indicated here by the increase MCHC (mean corpuscular hemoglobin concentration). There is a rare condition known as hereditary spherocytosis. Also, RBC's in the condition of autoimmune hemolytic anemia can also appear similarly.
Anemias
Symptoms of Anemia
• Fatigue
• Shortness of breath
• Dizziness
• Pale or yellowish skin
• Swollen tongue that may appear dark red
• Weight loss
• Diarrhea
• Numbness or tingling in your hands and feet
• Muscle weakness
• Irritability
• Unsteady movements
• Mental confusion or forgetfulness
Anemia
Definition:
Decrease in hemoglobin concentration below the normal value for the age, sex
and for the given individual
Classification of anemias
There are many classifications of anemia
• Based on underlying mechanisms
• Morphologic characteristics
Anemias due to BLOOD LOSS
Mechanism Specific Examples BLOOD LOSS Acute blood loss Trauma Chronic blood loss Gastrointestinal tract lesions,
gynecologic disturbances
INCREASED RED CELL DESTRUCTION (HEMOLYSIS)
Inherited genetic defects Red cell membrane disorders Hereditary spherocytosis, hereditary elliptocytosis Enzyme deficiencies Hexose monophosphate shunt enzyme deficiencies
G6PD deficiency, glutathione synthetase deficiency
Glycolytic enzyme deficiencies Pyruvate kinase deficiency, hexokinase deficiency Hemoglobin abnormalities Deficient globin synthesis Thalassemia syndromes Structurally abnormal globins (hemoglobinopathies)
Sickle cell disease, unstable hemoglobins
Acquired genetic defects Deficiency of phosphatidylinositol-linked glycoproteins
Paroxysmal nocturnal hemoglobinuria
Antibody-mediated destruction Hemolytic disease of the newborn (Rh disease), transfusion reactions, drug-induced, autoimmune disorders
Mechanical trauma
Microangiopathic hemolytic anemias Hemolytic uremic syndrome, disseminated intravascular coagulation, thrombotic thrombocytopenia purpura
Cardiac traumatic hemolysis Defective cardiac valves
Repetitive physical trauma Bongo drumming, marathon running, karate chopping
Infections of red cells Malaria, babesiosis Toxic or chemical injury Clostridial sepsis, snake venom, lead poisoning Membrane lipid abnormalities Abetalipoproteinemia, severe hepatocellular
liver disease Sequestration Hypersplenism
DECREASED RED CELL PRODUCTION Inherited genetic defects Defects leading to stem cell depletion
Fanconi anemia, telomerase defects
Defects affecting erythroblast maturation
Thalassemia syndromes
Nutritional deficiencies
Deficiencies affecting DNA synthesis
B12 and folate deficiencies
Deficiencies affecting hemoglobin synthesis
Iron deficiency anemia
Erythropoietin deficiency Renal failure, anemia of chronic disease
Immune-mediated injury of progenitors
Aplastic anemia, pure red cell aplasia
Inflammation-mediated iron sequestration
Anemia of chronic disease
Primary hematopoietic neoplasms Acute leukemia, myelodysplasia, myeloproliferative disorders ( Chapter 13 )
Space-occupying marrow lesions Metastatic neoplasms, granulomatous disease
Infections of red cell progenitors Parvovirus B19 infection Unknown mechanisms Endocrine disorders, hepatocellular
liver disase
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Dr.CSBR.Prasad, M.D.,
Associate Professor of Pathology,
Sri Devaraj Urs Medical College,
Kolar-563101,
Karnataka,
INDIA.