[Chronic neutrophilic leukemia clinicopathological investigation on Japanese cases: with the questionnaire]

The clinical picture and disease features of chronic neutrophilic leukemia (CNL), a rare hematological disorder, were investigated among 38 patients collected from Japanese reports. According to the diagnostic criteria, review of Japanese literature was carried out, using the questionnaire to the reporters of CNL cases. The rate of return for the questionnaire was 23/29 (79.3%). Of the 38 CNL patients, 26 were men and 12 were women. Their mean age at diagnosis was 65.2 years. The mean value of initial laboratory data were as follows; leukocyte count 54,000/mm3 with 86.9% mature neutrophils, hemoglobin 10.6g/dl, platelet count 22.0 X 10(4)/mm3, NCC from the bone marrow 44.9 X 10(4)/mm3, and NAP score 400.5. The CFU-C value was decreased in 17 of 24 cases examined. Two among the thirteen cases showed an increase of CSF activity in serum, and none was detected in urine of four cases. The clinical course in 33 cases were evaluated, and the median survival after the diagnosis was 21 months. Three cases terminated blastic crisis. Two had features similar to polycythemia during their course. The association with monoclonal gammopathy or multiple myeloma was found in 8 cases (21.8%), and this appears to be among the presenting clinical characteristics. Hemorrhagic diathesis was often fatal and was the most frequent cause of death (13 out of 28 cases died). Postmortem examination showed occasional systemic infiltration with neutrophils or leukemic cells in most organs including liver and spleen.