Diagnostic Dilemma

Acute hemolysis


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Question
A twelve years old male child born of third degree consanguineous marriage presented with fever for 10 days and high colored urine for 5 days. On examination, he had pallor, icterus and splenohepatomegaly. He had bradycardia and basal crepitations suggestive of congestive cardiac failure. He had been treated with Chloroquine 5 days ago along with Primaquine by a private practitioner for his fever. A clinical diagnosis of pyrexia with acute hemolysis was considered. His hemoglobin was 4.4 gm% with a WBC count of 10,400/cumm and platelet count of 4,72,000/cumm. Reticulocyte count was 5.1% with corrected reticulocyte count of 1.7%. Serum bilirubin was 1.5 mg/dl with indirect bilirubin of 0.9 mg/dl. Liver enzymes were normal with direct coombs test and indirect coombs test being negative. The child did not have any further fever in the hospital stay.

What is the cause of hemolysis?
Expert Opinion :
The child has indirect hyperbilirubinemia and anemia with increased reticulocyte count suggestive of acute hemolysis. The cardiac failure may be related to the acute anemia. There is no external blood loss. Thus the destruction of RBCs may be in the spleen due to infected RBCs or due to drug related hemolysis. The hemolysis may be due to infection such as malaria. Since the child has responded to Chloroquine with no further fever episode, malaria induced destruction seems unlikely. Also Coombs test is negative ruling out autoimmune anemia. Since the child was given Primaquine and also is a male child G-6-PD deficiency is a possibility. He was screened for G-6-PD deficiency, which was low. The parents were negative for G-6-PD deficiency.
G-6-PD (Glucose-6-phosphate dehydrogenase) is an enzyme present in the RBC. Deficiency of this enzyme leads to shortening of the red-cell life span and hereditary non-spherocytic hemolytic anemia. It is inherited as an X-linked disorder. The life span (particularly of older RBCs) is shortened particularly during drug administration and infection. Drug induced hemolysis in G-6-PD deficient cells is accompanied by formation of Heinz bodies due to inability to reduce NADP+ to NADPH at a normal rate. Hence hemoglobin is denatured and destroyed by the spleen. The major clinical feature is hemolytic anemia in males usually the anemia is episodic. Drugs to be avoided in G-6-PD deficiency are – Furazolidone, Nalidixic acid, Nitrofurantoin, Phenylhydrazine, Primaquine, sulfacetamide, sulfamethoxazole, sulfapyridine and chloramphenicol. Chemicals to be avoided are Methylene blue, Naphthalene, TNT.
Answer Discussion :
S
Sunil Shukla
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G6pd deficiency
9 months ago
E
Elsa Lima Teixeira
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G6PDH deficiency
9 months ago

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