Case: Thalassemia with severe anaemia with pericardial effusion

I was called in a clinic to see a girl of 5 years of consanguinous parents of Rohingya origin,the family staying in a Refugee camp in Tekhnaf, waiting for migration to Ireland.

The patient has growth failure(wt 10 kg),severe pallor, mild respiratory distress, malar prominence, huge hepatospenomegaly and intermittant fever of 22 days with no history of contact with tuberculosis. Clinically she also seems to have cardiomegaly, a soft systolic murmer, no basal creps and the liver was not tender.

Her investigation shows Hb-2.7 gm/dl, ESR-60, TC,DC-normal range. TT, Urine R/E is noram. Hb electrophoresis shows Hb-E Beta Thalassemia disease. X-ray has huge cardiomegaly-pear shaped with oligamic lung field, Echo shows gross pericardial effusion.




She was given two unit of blood, antibiotic and oral steroid. Fever was subsided after 7 days of traatment,Hb eleveated and respiratory distress subsided.

For pericardiocentesis and further evaluation he was refered to a cardiac centre. The delay takes place because of beaucratic complicacy of the sponsoring agencies.

badrud doza

Post script: The patient was untimately given antitubercular drug.

Case: Spleenectomy with postoperative convulsion and impaired consciousness in a case of Thalassemia

A boy of 11 years was suffering from Thalassemia major. He was advised for spleenectomy as the huge spleen was causing respiratory embarrassment.

He was operated without any trouble. But he developed convulsion in the mid night which was focal in the right hand. He was given anticonvulsants and Inj Calcium. His electrolytes and the calcium show normal level. His convulsion was continuing intermittently. His convulsion become generalized and he GCS falls to 8. He was given Inj phenobarbitone and Sodium Valproate orally.

Convulsions were controlled and his consciousness was gradually improving. His speech was initially slurred which was also later improved.

We had a plan for CT scan but as the patient improved completely patient took the discharge.

We level the case as Thalassemia major with post operative convulsion due to emboli.

Dr. Badrud Doza
Dr. Shoma Chowdhury
Related link:
1) Thalassemia Foundation
2)International Thalasemia Foundation

Thalassemia with jaundice

At this momont, we have two patients of thalassemia with jaundice. Both are male, age 7 yrs, both are from poor socioeconomic class and both are thalassemia intermedia.
The first case has taken blood transfusion 1 year back, the second case was on hydroxyurea.
Both are HBsAg negative.
The first one develops jaundice due to acute hepatitis-nonB virus.In second the jaundice is induced by the hepatoxicity of the hydroxyurea.