A VARIANT OF MYELOKATHEXIS WITH HYPOGAMMAGLOBULINEMIA: Lymphocytes as Well as Neutrophils may Reverse in Response to Infections

A 7-year-old boy with prolonged and marked leukopenia diagnosed at 6 months of age is described. The polymorphonuclear cells presented no hypersegmented nuclei or concentrated nuclear chromatin, although vacuolated myeloid cells appeared in bone marrow smears. Neutrophils reversed in response to adm...

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Bibliographic Details
Published in:Pediatric hematology and oncology 2000, Vol.17 (2), p.171-176
Main Authors: Arai, Jun-Ichi, Wakiguchi, Hiroshi, Hisakawa, Hiroaki, Kubota, Haruo, Kurashige, Takanobu
Format: Article
Language:English
Subjects:
Agammaglobulinemia - complications
Agammaglobulinemia - diagnosis
Agammaglobulinemia - drug therapy
AIDS/HIV
Antibodies, Viral - blood
Antigens, Viral - blood
Biological and medical sciences
Bone Marrow - pathology
Capsid - immunology
CD4-CD8 Ratio
Child
Combined Immunodeficiency
Cytoplasm - pathology
Cytoplasm - ultrastructure
Fever
Genetic Variation
Granulocyte Colony-Stimulating Factor - administration & dosage
Herpesvirus 4, Human - chemistry
Humans
Hydrocortisone - administration & dosage
Hyperplasia - complications
Hypogammaglobulinemia
Immunodeficiencies
Immunodeficiencies. Immunoglobulinopathies
Immunoglobulin A - blood
Immunoglobulin G - blood
Immunoglobulin M - blood
Immunoglobulins - blood
Immunopathology
Leukocyte Count
Leukopenia
Leukopenia - complications
Leukopenia - diagnosis
Leukopenia - drug therapy
Lymphocyte Subsets
Male
Medical sciences
Myelokathexis
Neutropenia - complications
Neutropenia - diagnosis
Neutropenia - genetics
Neutrophils - drug effects
Neutrophils - pathology
Neutrophils - ultrastructure
Vacuoles
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Description
Summary:A 7-year-old boy with prolonged and marked leukopenia diagnosed at 6 months of age is described. The polymorphonuclear cells presented no hypersegmented nuclei or concentrated nuclear chromatin, although vacuolated myeloid cells appeared in bone marrow smears. Neutrophils reversed in response to administration of G-CSF. His leukocyte counts were 400-1000/muL during afebrile periods and increased to 2000-3000/muL in response to infections. The increased leukocyte was usually neutrophils, but lymphocytes also increased at EB-virus infection. The serum IgG decreased gradually and was 364 mg/dL at 7 years of age. Antibody responses were normal and recurrent otitis media has been the patient's only problem. Granulocytopenia with hypogammaglobulinemia of this patient mimics myelokathexis with hypogammaglobulinemia, and lymphocytes also increased at viral infections.
ISSN:0888-0018
1521-0669
DOI:10.1080/088800100276532