Chart-4

A 15 year old girl presented with weakness and yellowish discouration of sclera.

O/E: Pallor, moderate splenomegaly and mild jaundice +

Laboratory findings

Hemoglobin             - 8 gm%

MCV                            - 90 fl

MCH                           - 28 pg

MCHC                         - 38%

PCV                            - 23%

Reticulocyte count  - 8%

Serum unconjugated bilirubin   - Increased

Osmotic fragility     - Increased

Peripheral blood smear:

A. Interpret the peripheral smear findings in this case? 

• RBCs are normocytic normochromic, polychromatophilic RBCs and many microspherocytes are seen 

• WBCs are normal in number, morphology and distribution 

• Platelets are adequate 

B. What is the diagnosis? 

Hereditary Spherocytosis 

C. Which protein is deficient in this condition? 

Spectrin deficiency is the most common abnormality