A 70-year-old male patient had been treated as diabetes mellitus and hypertension at Kobe Kyodo Hospital. Blood examination revealed leukocytosis (20,600/μl, 95% mature granulocytes) by chance in 1973. On physical examination, mild hepatosplenomegaly was found. Elevation of serum uric acid, vitamine B12, and neutrophile alkaline phosphatase were detected. No Ph1 chromosome or colony stimulating factor was found. Based on these findings, the patient was diagnosed as having chronic neutrophilic leukemia.
In 1977, electrophoretic pattern showed polyclonal gammopathy with 2,300 mg/dl of serum IgG In 1979, a small spike was obsered in the γ-region on electrophoresis. In 1982, this spike became conspicuous with the appearance of Bence Jones protein in the urine. The patient died of uremia in 1984 and in autopsy bone marrow and liver were found infiltrated with mature neutrophils. However, no other malignancies including multiple myeloma were observed.
This case, with a 11-year follow-up, is the longest surviving case of chronic neutrophilic leukemia, ever reported ln Japan. The mechanisms of association with monoclonal gammopathy were also discussed.
