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Question :
Posted On : 02/26/2019 12:30:24
A 15 YRS OLD BOY CAME TO MY OPD 4 MONTH BACK
WITH SEVERE PALLOR.ON EXAMINATION HER Hb
WAS 2Gm percent.S.Bilirubin 1.3, LIVER and SPLEEN
ENLARGED,XRAY SKULL SHOWED HAIR END ON
APPEARANCE.HIS GBP WAS S, O HEMOLTYTIC
ANAEMIA. BUT STRANGELY HE HAS NO PREVOIUS
HISTORY OF BLOOD TRANSFUSION.HE HAD NO
FEVER.3 UNITS OF BLOOD WAS TRANSFUSED AT
THAT TIME.NOW THAT AGAIN CAME TO TO ME HIS
Hb IS 3.5 ,S.Bilirubin IS 1.4.HE PASSES YELLOW
COLOURED URINE.HIS Hb ELECTROPHORESIS REPORT IS AS UNDER-
HbF-51,Peak2- 3.6,Hb Adult-41,HbA2-3.7,
CLINICALLY IT LOOK LIKE THALLESEMIA INTERMEDIA.WHY IT PRESENTED SO LATE ,AND IN THESE 4 MONTH WHY HIS HB IS FALLING SO FAST_?WHAT COULD BE THE OTHER POSSIBLE CAUSES._?
2
Expert Answer :
Short answer is YES. Thalassemia intermedia can present late. Median presentation age is 8 years but range is wider. Much also depends on when a person presents. In early ages they compensate by bony expansion and hepatosplenomegaly. I presume this child has bony deformities. It is a good idea to mention spleen size whenever asking for opinion {instead of splenomegaly}. I did not see growth, developmental delay as this can suggest chronicity of this condition.

Now there are 2 situations. One this child needs chronic transfusion regime {like a thalassemia major}. That is the reason that he is needing more transfusions at longer intervals. I deally these should be evenly spaced out. I suggest doing a CBC at 2-3 weeks interval to see hpw rapidly his Hb is falling.

Second differential is a bit tricky one. He is alloimmunised. In thalassemia {either}, the later the transfusions are started, higher is the chance of alloimmunisation. Unbelievable but true, if first transfusion is after age of 3 years then risk is 44 percent.
I suggest doing a direct antiglobulin {Coomb`s} test just before next transfusion is due. Ask the lab for grading of Coomb`s test if done by gel card method.

In either situation, a complete red cell antigen profile should be done so that this child gets best blood. This will probably cost Rs 1,500 and most private hospitals blood bank should be able to do it.

I will be interested in follow-up information on this child some weeks down the line so that we can plan better management for this child.
Answer Discussion :
A
Amelia A. Armas
g6pd deficiency
20 Days ago
M
Mahdi Tuji
malaria
21 Days ago



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