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  • Title: Clinical features of myelokathexis and treatment with hematopoietic cytokines: a case report of two patients and review of the literature.
    Author: Hord JD, Whitlock JA, Gay JC, Lukens JN.
    Journal: J Pediatr Hematol Oncol; 1997; 19(5):443-8. PubMed ID: 9329467.
    Abstract:
    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.
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