Chronic Granulomatous Disease

4th Pediatric Infectious Diseases Conference

ISSN 0973 - 0958

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Rakesh Kumar, Binay Ranjan, Manjul Vijay Department of Pediatrics, Katihar medical college, Katihar...

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	Specialist Answers
Question
A 4 year-old boy, known to have Chronic Granulomatous Disease, is on Co-trimoxazole prophylaxis. He was admitted 3 weeks ago with herpetic stomatitis, secondary to HSV type I. During admission the child was febrile, and had petechial lesions & herpetic stomatitis. No lymphadenopathy & no hepatosplenomegaly was seen. 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 & coaggulation 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 immunodeficient host, through 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 he showed 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 that is 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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