Effects of molgramostim, filgrastim and lenograstim in the treatment of myelokathexis.
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 x 10(9)/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 microg/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 microg/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.
Cernelc
P
P
Department of Haematology, University Medical Centre Ljubljana, Slovenia.
Andoljsek
D
D
Mlakar
U
U
Pretnar
J
J
Modic
M
M
Zupan
I P
IP
Zver
S
S
D000276:Adjuvants, Immunologic / Q000627:therapeutic use*; D000328:Adult; D002908:Chronic Disease; D005260:Female; D000069585:Filgrastim; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D000078224:Lenograstim; D007958:Leukocyte Count; D009503:Neutropenia / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D009504:Neutrophils / Q000473:pathology; D011994:Recombinant Proteins / Q000627:therapeutic use*
D000276:Adjuvants, Immunologic; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D000078224:Lenograstim; D016178:Granulocyte-Macrophage Colony-Stimulating Factor; C082430:molgramostim; D000069585:Filgrastim