Chart-3

A 2 year old boy presented with failure to thrive and abdominal distension.

O/E: Pallor+, frontal bossing and hepatosplenomegaly.

X ray of Skull- Crew cut (Hair on end) appearance. 

Laboratory findings:

Hemoglobin: 4gm/dl

Reticulocyte count: 7 %

Peripheral blood smear:

A. Interpret the peripheral blood smear with diagnosis. 

• RBCs: are microcytic hypochromic, marked anisopoikilocytosis with many target cells and polychromatophilic RBC’s. 

• WBCs normal in number, morphology and distribution 

• Platelets adequate 

Diagnosis: Thalassemia

B. Why is this X-ray appearance due to? 

Widening of bone marrow (diploe) of skull in infants and children with severe chronic hemolysis is due to increased erythropoiesis and hair on end appearance is  due to accentuated vertical trabeculae between the inner and outer tables of the skull because of excessive bone marrow hyperplasia. 

C. Name the test to confirm this condition. 

Hemoglobin Electrophoresis