•     19 yrs Male, from Chandrapur

Present complaints

•     Fever with chills   x  2 days

•     Pain in abdomen   x  2 days

•     Lump left hypochondria

Past H /O

•     Repeated episodes of Jaundice

•     Blood transfusion- once very year since birth

On Examination          

Pallor

Icterus

Spleenomegaly grade II

Liver    3cms

Laboratory profile

Hb                   5.5 gm/dl

Reticulocyte   12%

PS - Anisopoikilocytosis,

        Moderate hypochromia, microcytosis,

        Teardrop cells, polychromasia,

        NRBC 18 – 20 /100 WBC

        WBC leukocytosis with shift to left

Sr Bilirubin ­­

LDH - 1205 U/L  

Family Study of Hb electrophoresis

Father               AA2

Mother             AS

Elder Sister     Died with similar complaints at the age of 20 yrs

DIAGNOSIS?