[Chronic neutrophilic leukemia: a long-term analysis of seven cases and review of the literature].

Chronic neutrophilic leukemia is an uncommon hematological entity. According to the WHO classification it is recognized as part of the family of myeloproliferative disorders. In the last 20 years seven patients have been diagnosed with chronic neutrophilic leukemia at our department. All but one had splenomegaly, two patients developed severe anaemia and in one case thrombocytosis was present at the time of diagnosis. White blood cell count ranged between 39 x 10(9)/1-71 x 10(9)/l with 80% of neutrophils and striking myeloid hyperplasia were present in the bone marrow without evidence of any dysplasia resembling chronic myelocytic leukemia. Granulocyte alkaline phosphatase scores were increased except one case and both cytogenetics (Philadelphia chromosome) and molecular biologic analysis (bcr/abl) revealed no alteration of any. Four patients have been followed up. Three of them died due to progression of chronic neutrophilic leukemia. One patient, initially receiving hydroxyurea + interferon therapy and showing progression, developed complete hematological remission with an eight week imatinib mesylate (Glivec) treatment. Beside of their own experiences the authors review the current literature and discuss differential diagnostic and therapeutic challenges, as well.