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Pediatric Oncall Journal

A 6 year old with thalessemia and increased blood transfusion requirement

A 6 year old with thalessemia and increased blood transfusion requirement

Dr Ira Shah.
Medical Sciences Department, Pediatric Oncall, Mumbai, India.

Dr Ira Shah, Medical Sciences Department, Pediatric Oncall, 1, B Saguna, 271, B St. Francis Road, Vile Parle {W}, Mumbai 400056.
Clinical Problem
A 6 years old boy born of non-consanguineous is a known case of thalassemia major diagnosed at 1 year of age. He has been on regular transfusions since then initially required a transfusion every 21 days but after the age of 4 years has required a transfusion every 15 days. Each time he requires packed cell transfusion at 15 cc/kg. However, since the past one month, his blood transfusion requirement has increased to once in every 7 days. He is on oral chelation but has stopped the same due to joint pain in the right knee since last 6 months. His serum ferritin done a year back was 3000 ng/dl and HIV, HCV and HBsAg by ELISA was negative at that time. On examination, he was malnourished (Height = 98 cm, Weight = 14 kg) and had thalassemic facies. He had splenohepatomegaly. His investigations showed Hemoglobin of 6.4 gm/dl, WBC count of 4,400/cumm and Platelet count of 1,22,000/cumm with reticulocyte count of 0.3%. His Direct and Indirect Coomb’s test were negative. He had elevated liver enzymes (SGOT = 235 IU/L, SGPT = 240 IU/L) with hypoalbuminemia (Serum albumin = 2.4 gm%). S. Bilirubin was normal. HIV, HBsAg and Anti HCV by ELISA were negative. He required packed cell transfusion every alternate day but his hemoglobin did not increase beyond 6.9 gm/dl though WBC count and platelet count improved.

What is the cause of his intractable anemia?
Expert’s opinion:- Dr Ira Shah

This child with thalassemia major has had an increased blood transfusion requirement since the past 2 years with packed cell requirement of over 390 cc/kg/year. This suggests either hypersplenism or bone marrow suppression. This child does not have any viral markers that are positive and hence chronic bone marrow suppression due to HIV, Hepatitis B or C seems unlikely. Thus, most likely, the long standing anemia may be due to hypersplenism. In such a case, the white cell count and platelet count will also be low which was seen in this child. Hence, it is quite likely that the child has an underlying hypersplenism. This can be proved by doing a bone marrow examination which in a case of hypersplenism may be of normal cellularity or may be hypercellular. One can also do an RBC tagged radionuclide scan which may show increased destruction of RBCs in the spleen. [In a case of thalassemia major, even though it is a hemolytic anemia, the anemia is due to dyserythropoietic anemia and not due to destruction in the spleen].
However, coming back to this intractable anemia since 1 month, hypersplenism cannot explain the severe anemia. In a multiply transfused child, autoimmune hemolytic anemia is a possibility. In such a case, Coomb’s test should be positive and there should be other evidence of hemolysis such as hemoglobinuria and indirect hyperbilirubinemia which this child did not have.
Thus, most likely the child has in addition a bone marrow suppression now. This child’s HIV, HBsAg, Anti HCV are negative. Also he seems to have predominant involvement of red cells. Hence an organism that has prediction for mainly RBCs should be suspected such as Parvovirus or Cytomegalovirus (CMV). CMV infection is common in multiple transfused thalassemia child but usually causes disease only if the child is immunosuppressed.
Parvovirus in patients with thalassemia is acquired through blood transfusion. Though it commonly causes a rash, it may be absent or minimal and in a child with hemolytic anemia can present with bone marrow suppression with predominant involvement of the red cells. This infection can be proved by doing Parvovirus ELISA or PCR. In this child, Parvovirus IgM was positive. Treatment for intractable anemia consists of Intravenous immunoglobulins (IVIg). However, sometimes, the infection resolves on its own. In this child, hemoglobin stabilized after 21 days and then he required blood transfusion again after 15 days interval.
Hence, in a child with hemolytic anemia with acute intractable drop in hemoglobin, one must suspect a Parvovirus infection.
Compliance with ethical standards
Funding:  None  
Conflict of Interest:  None
Cite this article as:
Shah I. A 6 year old with thalessemia and increased blood transfusion requirement. Pediatr Oncall J. 2006;3: 73.
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