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Medicine

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Hematology

Question
3 out of 17
 

Haptoglobin levels are decreased in: (AIPG 2011)



A A mismatched transfusion reactions

B PNH

C G 6PD deficiency

D All of the above

Ans. D

All of the above (Ref. Hari- 17th ed., Pg. 652)

Haptoglobin is a type of alpha-2 globulin

Hemolysis is said to occur when the mean RBC survival is less than 120 days.

Extra Edge:

1. All intracorpuscular causes of hemolysis are inherited (Hemoglobinopathy, Enzyme defects, membrane defects) except PNH which is due to acquired somatic mutation

2. All extracorpuscular causes of hemolysis are acquired except heredity hemolytic uremic syndrome (where there is excess compliment activation)

Table - Classification of Hemolytic Anemias (Ref. Hari-18th Pg. 872, table 106.1)

Intracorpuscular Defects

Extracorpuscular Factors

Hereditary

Hemoglobinopathies

Familial hemolytic uremic syndrome (HUS)

Enzymopathies

Membrane-cytoskeletal defects

Acquired

PNH

Mechanical destruction (microangiopathic)

Toxic agents

Drugs

Infectious

Autoimmune

Extra Edge: Hereditary causes correlate with intracorpuscular defects because these defects are due to inherited mutations. The one exception is PNH because the defect is due to an acquired somatic mutation. Similarly, acquired causes correlate with extracorpuscular factors because mostly these factors are exogenous. The one exception is familial hemolytic uremic syndrome (HUS; often referred to as atypical HUS) because here an inherited abnormality allows complement activation to be excessive, with bouts of production of membrane attack complex capable of destroying normal red cells.

Extra Edge: Features of hemolytic anemia

1. Reticulocyte count (Most important criteria)

2. Unconjugated bilirubin

3. LDH

4. Splenomegaly

5. Hypercellular BM

6. Hb level is reduced

7. MCH , MCV

8. Urobilinogen +

9. P/S polychromasia increase nucleated RBC, Macrocytes.

10. Erythroid hyperplasia in marrow (Meyloid erythroid ratio is decreased)

11. Cr 51 labeled RBC is used to assess the life span.

12. Gall stone (Pigment)

13. Intra vascular hemolysis Hemoglobinuria can cause Hemosiderinuria IDA

14. Extra vascular hemolysis = Blood Transfusion Fe overload Secondary Hemochromatosis can cause CHF

Extra Edge:

Compensated Hemolytic anemia = Hemoglobin normal = due to increase erythropoietin

Decompensate (Hb reduced) a. Pregnancy b. Renal failure c. Folic acid deficiency d. Parovo virus infection

Extra Edge:

1. The increase or decrease of one cell lineage (myeloid vs. erythroid) compared to another is obtained by a differential count of nucleated cells in a bone marrow smear [the myeloid/erythroid (M/E) ratio].

2. A patient with a hypoproliferative anemia and a reticulocyte production index < 2 will demonstrate an M/E ratio of 2 or 3:1.

3. In contrast, patients with hemolytic disease and a production index > 3 will have an M/E ratio of at least 1:1. Maturation disorders are identified from the discrepancy between the M/E ratio and the reticulocyte production index (Ref. Hari- 18th Pg. 452).

4. As haptoglobin is indeed an acute-phase protein, any inflammatory process (infection, extreme stress, burns, major crush injury, allergy, etc.) may increase the levels of plasma haptoglobin.

Hematology Flashcard List

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