Chronic Granulomatous Disease in pediatriconcall.com

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Rakesh Kumar, Puja Bhagat Department of Pediatrics, Katihar Medical College, Katihar, Bihar, India ...

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	Specialist Answers
Question
4 year-old boy, known case of Chronic Granulomatous Disease, on Co-Trimoxazole prophylaxis. He was admitted 3 weeks ago with herpetic stomatitis, secondary to HSV type I. Upon admission the child was febrile, and having petechial lesions & herpetic stomatitis. There was no lymphadenopathy & no hepatosplenomegaly. His CBC showed Pancytopenia (WBC 1700, absolute neutrophil count 260, RBC 2.6, hemoglobin 7, platelets 20,000). Retcis 5.4% .Peripheral blood film showed slight spherocytes, target cells, shistiocytes and mild hypochromia. Renal function test, liver function test & coagulation profile were normal. He was given a course of Ceftazidim, Cloxacillin (for 10 days) & Acyclovir IV for 4 days. His herpetic stomatitis resolved, fever subsided, and blood culture had no growth. The possibility of viral or drug-related bone marrow suppression was the initial impression, but being an immunodeficienthost, thuoghto our knowledge CGD is not associated with leukemias, and with a ? family history of leukemia, malignancy had to be ruled out. Bone marrow aspiration showed hypercellular marrow with no abnormal cells. So the investigations were directed to possible autoimmune process with ? Evans syndrome. Direct Coombs' tests done twice were negative. ANA and anti-platelet antibodies were negative. Other investigations showed a total bilirubin 23 umol/L, direct 4. Haptoglobin <0.06 g/L (NR 1-2.3), serum ferritin 179.9 (NR 30-400), triglyceride 1.32. Ham test weakly positive. Urine analysis was normal. The patient was given 2 doses of IV immunoglobulin but the pancytopenia persisted. He was given GC-SF, and the neutropenia responded. He also received Prednisolone 2 mg/kg/day for 6 days but with no response regarding the platelet count, and so it was discontinued. His CBC after this 3 weeks period showed WBC 5940, ANC 2140, RBC 1.61, hemoglobin 5.1, platelet 13,000, retics 32.4 % . His general condition remains stable & afebrile, with no new findings on his physical examination. What is your impression? Your suggestions are welcome to best_pedia@yahoo.com . Thanks for your time and for helping this child.
Answer
This is what our Consultant Hematologist had to say: The initial presentation was s/o viral induced BM suppression,which is corroborated by a hypercellular marrow done during the resolution phase of the disease process. The PS still suggests some hemolysis. Also,with the child clinically well & recovery of white cells with persistence of anemia(with Normal retic) & thrombocytopenia,it does feel like Evans syndrome. I feel that the test really needed is anticardiolipin antibodies. This would explain the destruction of RBC's & platelets with Normal DCT & antiplatelet antibodies. Do inform us of any developments in this case. Back to All Questions

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