Clinical features of myelokathexis and treatment with hematopoietic cytokines: a case report of two patients and review of the literature.

Autor:	Hord JD; Department of Pediatrics, University of Pittsburgh School of Medicine, Pennsylvania, USA., Whitlock JA, Gay JC, Lukens JN
Jazyk:	angličtina
Zdroj:	Journal of pediatric hematology/oncology [J Pediatr Hematol Oncol] 1997 Sep-Oct; Vol. 19 (5), pp. 443-8.
DOI:	10.1097/00043426-199709000-00007
Abstrakt:	Purpose: To define the features and course of myelokathexis, a rare congenital neutropenia resulting from impaired release of granulocytes from bone marrow. Methods: The clinical features, granulocyte function, lymphocyte function, and response to granulocyte colony-stimulating factor (G-CSF) of two patients (mother/son) with myelokathexis were studied. This experience and 14 previous reports lead to a composite description of myelokathexis. Results: Both patients had chronic neutropenia, recurrent pulmonary infections, bone marrow consistent with myelokathexis, hypogammaglobulinemia, and elevated endogenous G-CSF. Patient 15 had normal granulocyte function, a rise in absolute neutrophil count (ANC) with epinephrine and hydrocortisone, and normal numbers of T- and B-lymphocytes; she also had numerous warts during childhood. Both patients experienced a transient increase in ANC with infection, a significant increase in ANC within 5 hours following a single dose of G-CSF, and fewer infections with daily G-CSF. Conclusions: Based on 16 cases, myelokathexis occurs more often in females and frequently affects multiple members of a family. The usual number of circulating granulocytes is low although function is normal. Mature marrow granulocytes are mobilized with infection, corticosteroids, epinephrine, G-CSF, and granulocyte-macrophage colony-stimulating factor (GM-CSF). Lymphocyte number is normal but lymphocyte function is abnormal as evidenced by hypogammaglobulinemia and papillomavirus infection.
Databáze:	MEDLINE
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