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Dysgranulopoietic neutropenia and abnormal monocytes in childhood vitamin B 12 deficiency
Author(s) -
Crist William M.,
Parmley Richard T.,
Holbrook C. Tate,
Castleberry Robert P.,
Denys Francis R.,
Malluh Ahmad
Publication year - 1980
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830090110
Subject(s) - myelopoiesis , granulopoiesis , vacuole , biology , bone marrow , myeloid , monocyte , immunology , pathology , medicine , progenitor cell , cytoplasm , microbiology and biotechnology , stem cell
Neutropenia and/or leukopenia (associated with elevated serum lysozyme levels) in three children with vitamin B 12 deficiency were evaluated using soft agar culture and ultrastructural and cytochemical techniques. In two patients a marked increase in peripheral blood myeloid colony forming cells (CFC) was observed; whereas a marginal increase in CFC was present in the third, less symptomatic, patient. Marrow CFC was normal or slightly increased. Serum colony stimulating activity (CSA) was normal but elaboration of CSA by white blood cells was low. Normal maturation of the progenitors was present in vitro and serum inhibitors of myelopoiesis were absent. Megaloblastic neutrophils and monocytes with nuclear‐cytoplasmic asynchrony were observed ultrastructurally in directly sampled marrow specimens. These cells contained autophagic and/or heterophagic vacuoles and an increase in cytoplasmic granules. Both monocytes and neutrophils also contained enlarged‐disrupted centrioles. Many marrow macrophages contained phagocytic vacuoles, which enclosed disrupted neutrophils and cellular debris. We conclude that although a large number of dysmorphic myeloid cells degenerate in the marrow, sufficient marrow CFC are present and peripheral blood CFC are elevated, suggesting abnormal release and/or maturation failure. All of these abnormalities are corrected by administration of vitamin B 12 .