Myelokathexis is a very rare form of chronic hereditary
neutropenia resulting from impaired neutrophil releasing mechanism in the bone marrow. The recombinant human granulocyte-macrophage (
molgramostim) and granulocyte (
filgrastim,
lenograstim)
colony stimulating factors release the mature granulocytes from the bone marrow. We describe a 43-year-old woman suffering from myelokathexis, with the absolute neutrophil count ranging between 0.03 and 1.35 × 109/L. In the period before the introduction of
cytokines, the patient had more than 80 major infectious episodes. Since 1991,
infections in this patient have been treated with
cytokines, given in conjunction with
antibiotics. Initially, she received
molgramostim in a daily dose of 5 μg/kg subcutaneously, which stimulated the release of granulocytes from her bone marrow, thereby allowing successful treatment of
infection. After the development of
hypersensitivity,
molgramostim was substituted with
filgrastim. Finally,
lenograstim was given a trial. With all three
cytokines, the patient's neutrophil count always attained normal values already 4 hours after subcutaneous application of the
drug in a dose of 5 μg/kg, the highest neutrophil levels were measured at 24 hours post-injection, and the neutrophil count was again close to the baseline value 72 hours after the treatment. A slight
neutropenia was present 48 hours after the application of
filgrastim. We believe that all three
cytokines are equally effective in increasing the neutrophil count in venous blood of patients with myelokathexis.