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Kostmann's Syndrome - 4.

Kostmann's Syndrome - 4.
#00002962
Author: John Lazarchick
Category: Myeloid Disorders > Congenital Neutropenia syndromes
Published Date: 10/29/2012

The patient is a 10 month old Caucasian male with a history of recurrent upper respiratory infections and otitis media since birth. His CBC at this time included a H/H of 10.9g/dl/32.4%, platelet count of 280,000/ul and a WBC of 3840/ul with a differential of 25% monocytes, 70% lymphocytes, 4% eosinphils, 1% basophils and 1% neutrophils. His ANC was 38/ul. After an extensive evaluation he was diagnosed as having congenital neutropenia consistent with Kostmann's syndrome. The peripheral smear confirmed the leukopenia and almost complete absence of neuutrophils. In this view, erythroid precursors and eosinophils are prominent. Note also several basophils. Myeloid maturation is absent. The prominent lymphocytosis is again seen. Many of these cells have the morphologic appearance of hematogones.