Ubenimex treatment in congenital cyclic neutropenia.
A female patient with congenital cyclic neutropenia was presented. The cycle of neutropenia and the duration of neutropenia of less than 500/microliters was approximately three weeks and two weeks, respectively. There were also oscillations of monocytes and eosinophils with the peak level at the neutropenic phase. The severity and incidence of infections tended to diminish when the patient was pregnant. There were no abnormalities of lymphocyte subpopulations including CD4, CD8, CD4/CD8 ratio, and CD16. Administration of the immunomodulator ubenimex (Bestatin), 30 mg/day, reduced the severity of infections during the neutropenic periods, although the cyclic of neutropenia was not altered.
Aihara
M
M
First Department of Internal Medicine, Hirosaki University School of Medicine.
Sasaki
K
K
Yoshida
Y
Y
D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D015994:Incidence; D007930:Leucine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D007958:Leukocyte Count; D008213:Lymphocyte Activation; D009503:Neutropenia / Q000151:congenital / Q000188:drug therapy*; D016176:T-Lymphocyte Subsets / Q000187:drug effects
D000900:Anti-Bacterial Agents; D007930:Leucine; C012211:ubenimex