Six month old with pallor
 
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Ira Shah
Medical Sciences Department, Pediatric Oncall, Mumbai

Address for Correspondence: 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 month old boy born of non consanguineous marriage presented with increasing pallor for 2 months and abdominal lump noticed 5 days back. There was no fever. The child is 3rd of three siblings and other two children are normal. Birth, immunization and milestones are normal. He is on breast feeds plus weaning diet. On examination, he had tachycardia, pallor, hepatosplenomegaly and cardiomegaly. Other systems were normal. Investigations showed:
• Hemoglobin = 3.4 gm, dl {MV = 67.6, MCH = 19.7, MCHC = 29.1}
• WBC count = 100,900, cumm {15 percent neutrophils, 83 percent lymphocytes}
• ESR = 70 mm at end of 1 hour
• Liver enzymes = Normal
• Lipemic serum with LDH = 4746 IU, L and uric acid = 12.8 mg percent
• Peripheral smear = nucleated RBCs.
• HIV = Negative



Question :
What is the diagnosis_?

Expert Opinion :
This child has anemia with high WBC count. The peripheral smear shows nucleated RBCs which may be counted as WBC on the coulter machine which may cause false elevation of the WBC count. Nucleated RBCs are immature red cells which are precursors of the mature RBCs. {In RBC maturation process in the bone marrow, the earlier erythrocytes have a nucleus which is lost as the cell matures into a reticulocyte and a mature RBC}. Thus, in peripheral blood, nucleated RBCs are usually not seen. Presence of nucleated RBCs is suggestive of increased destruction {hemolysis} of RBCs which makes the bone marrow throw immature cells into the circulation. This hemolysis can be in the spleen or the bone marrow. The lipemic serum with high LDH and uric acid is again suggestive of a high cell turnover and may be seen in leukemia or hemolysis. However, in leukemia, nucleated RBCs are not seen. Thus, in this child one would suspect hemolytic anemia. The child’s corrected WBC count was 15,900, cumm after excluding the nucleated RBCs and hemoglobin electrophoresis showed presence of beta thalassemia in the child {HbF = 70 percent, HbA2 = 1.8 percent} with both father {HbA2 = 6.3 percent} and mother HbA2 = 5.9 percent} being thalassemia carriers.
Thus, a WBC count of over 1,00,000, cumm is not always suggestive of leukemia or leukemoid reaction and examination of the peripheral smear is always a must to look for abnormality in morphology of RBC and WBC.

E-published: November 2009 Vol 6 Issue 11 Art No. 60


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