慢性好中球性白血病の一例

Abstract

82 歳男性．節性濾胞辺縁帯リンパ腫 stage Ⅳに対し R-CVP 療法 6 コース施行後，完全寛解の治療効果を得た．治療終了約 2 年後，PET/CT 検査で骨髄への FDG 集積の亢進と末梢血中好中球数 57,000/?l までの増加を認めた．悪性リンパ腫の再燃は否 定的で，臨床所見と CSF3R 遺伝子変異を認めたことから慢性好中球性白血病（chronic neutrophilic leukemia：CNL ）と診断 した．Hydroxycarbamide と cytarabine ocfosphate hydrate の投与では病勢コントロールが困難であり , 経静脈 cytarabine 療 法を併用したが徐々に治療抵抗性となり，診断から 2 年後に死亡した．CNL はまれな疾患であり報告する．

A case of stage IV nodal marginal zone lymphoma in an 80-year-old male showed complete remission after 6 courses of R-CVP (rituximab. cyclophosphamide. vincristine sulfate and predinosone). Two years after the treatment, positron emission tomography/ computed tomography revealed increased accumulation of 2-deoxy-2-[18F]fluoroglucose to bone marrow and increase of neutrophils in peripheral blood to 57,000/μl. The possibility of recurrence of malignant lymphoma was considered low. The diagnosis of chronic neutrophilic leukemia (CNL) was made from the clinical findings and discovery of CSF3R gene mutation. The disease was difficult to control with hydroxycarbamide and cytarabine ocfosphate hydrate. Cytarabine therapy was applied in combination but resistance to treatment gradually developed, and the patient died 2 years after diagnosis. We report this rare case of CNL.