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A 20-year-old male with chronic granulomatous disease (CGD) was admitted with multiple liver abscesses. He had already been diagnosed as CGD, type X91-, when he was 10 years old. He was successfully treated with antibiotics and granulocyte colony-stimulating factor (G-CSF) combined with continuous drainage of abscess. Employing flow cytometry, respiratory burst by individual neutrophils was measured using 2', 7'-dichlorofluorescein. The fluorescence intensity in all individual neutrophils from the patient under G-CSF treatment was higher than the one without G-CSF. G-CSF can be one of effective therapies for infection in some patients with CGD such as X91-.

A 67-year-old man was admitted to our hospital with abdominal distension due to hepatosplenomegaly. The peripheral blood revealed Hb content 6.5 g/dl, platelet count 4.7 x 10(4)/microliter, and WBC count 105.8 x 10(3)/microliter with 88% of mature neutrophils. The neutrophil alkaline phosphatase score was 421. Bone marrow aspiration revealed hypercellularity with increased megakaryocytes and myeloid hyperplasia. 46, XY, del 20(q 11) without Philadelphia chromosome was identified by cytogenetic study. The patient was diagnosed as having chronic neutrophilic leukemia and was successfully treated with busulfan, but he died of atypical mycobacteriosis about 20 months later. Analysis of neutrophil function demonstrated diminution of deformability, random mobility, and chemotaxis, but almost normal phagocytosis and bactericidal capacity. Southern analysis showed no rearrangements of breakpoint cluster region (bcr) gene and immunoglobulin heavy chain gene.