Effects of molgramostim, filgrastim and lenograstim in the treatment of myelokathexis

Myelokathexis is a very rare form of chronic hereditary neutropenia resulti ng from impaired neutrophil releasing mechanism in the bone marrow. The rec ombinant human granulocyte-macrophage (molgramostim) and granulocyte (filgr astim, lenograstim) colony stimulating factors release the mature granulocy tes from the bone marrow. We describe a 43-year-old woman suffering from my clokathexis, with the absolute neutrophil count ranging between 0.03 and 1. 35 x 10(9)/L. In the period before the introduction of cytokines, the patie nt had mon: than XO major infectious episodes. Since 1991, infections in th is patient have been treated with cytokines, given in conjunction with anti biotics. Initially, she received molgramostim in a daily dose of 5 mu g/kg subcutaneously, which stimulated the release of granulocytes from her bone marrow, thereby allowing successful treatment of infection. After the devel opment of hypersensitivity, molgramostim was substituted with filgrastim. F inally, lenograstim was given a trial. With all three cytokines, the patien t's neutrophil count always attained normal values already 4 hours alter su bcutaneous application of the drug in a dose of 5 mu g/kg, the highest neut rophil levels were measured at 24 hours post-injection, and the neutrophil count was again close to die baseline value 72 hours alter the treatment. A slight neutropenia was present 48 horns after the application of filgrasti m. We believe that all three cytokines are equally effective in increasing the neutrophil count in venous blood of patients with myelokathexis.