The patient, one year and ten month old girl, whose diagnosis had been made as chronic benign neutropenia in childhood (CBN-childhood) at the age of seven month, was readmitted to Department of Pediatrics, Fukushima Medical College Hospital because of suddenly appeared hemorrhagic diathesis. The diagnosis of CBN-childhood had been made on the basis of following hematological findings; RBC 449×10
4/cmm, Hb 11.6 g/d
l, Hct 35.5%, WBC 7,000/cmm (band 1%, segment 1%, monocyte 13%, lymphocyte 77.5%, absophil 0.5%, eosinophil 7%), platelet 41.4×10
4/cmm and bone marrow cellularity 7.99×10
4/cmm (myeloblast 1.4%, promyelocyte 2.2%, myelocyte 6.2%, metamyelocyte 13.4%, band 10.4%, segment 1.2%, lymphocyte 43.4%, erythroblast 15.0%, others 6.8%).
Laboratory findings on readmission revealed RBC 558×10
4/cmm, Hb 10.3 g/d
l, Hct 32.9%, WBC 5,600/cmm (band 1%, segment 0%, monocyte 3.5%, lymphocyte 90.5%, basophil 3%, eosinophil 1.5%, erythroblast 0.5%), platelet 1.3×10
4/cmm and bone marrow cellularity 28.9×10
4/cmm with megakaryocyte 113/cmm with marked increase of immature megakaryocytes. On the diagnosis of CBN-childhood associated with thrombocytopenic purpura, oral administration of betamethasone (3 mg/day, total amount 100 mg) was performed for the purpose of reduction in hemorrhagic diathesis, which resulted in the hematological improvement not only in platelet (31.7×10
4/cmm at 18th day), but in neutrophil (10,675/cmm; band 4%, segment 57% at 12th day).
In this report, the possible mechanisms of steroid induced remissionin this case was discussed, and it was strongly suggested that this case belongs to the group of autoimmune neutropenia, though the classical test for detection of anti-neutrophil antibodies were not detected in our case.
To our knowledge, CBN-childhood associated with thrombocytopenic purpura was not reported previously.
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