脾臓摘出後, 両側腎に浸潤を認めた splenic marginal zone B-cell lymphoma [in Japanese] Splenic marginal zone B-cell lymphoma with bilateral renal invasion after splenectomy [in Japanese]
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症例は61才女性。肝脾腫及び全身のリンパ節腫脹とともに，血液検査にて貧血・血小板減少を認めた。末梢血，骨髄にκ+, CD5-, CD10-, CD23-, CD19+, CD20+の，絨毛様構造を有する異型リンパ球を認めた。脾臓摘出術を施行し，Splenic marginal zone B-cell lymphoma (SMZL), 臨床病期IV Aと診断。脾摘後，血液検査は正常化，ガリウムシンチで異常集積を認めなかった。しかし2ヶ月後，肝腫大の増大とともに，腎障害及びIgM-κ型M蛋白が出現。胸腹CTで両側腎が腫大し，骨髄，胃及び腎臓にリンパ腫細胞の浸潤を認めた。SMZLは緩慢な経過をとることが多く，これまでのところ両側性に腎へ浸潤した報告はない。R-CHOP療法を3コース施行し，完全寛解を得た。急激な増悪を認めてから6ヶ月経過した現在も寛解を維持している。
A 61-year-old woman presented with hepatosplenomegaly, systemic lymphadenopathy, anemia, and thrombocytopenia. Peripheral blood and bone marrow examination showed atypical lymphoid cells with villi. Immunophenotyping of these cells was CD19+ CD20+ CD5- CD10- CD23-, and light chain restriction (kappa) was positive. To confirm the diagnosis histologically, we performed a splenectomy and diagnosed the patient's disease as splenic marginal zone lymphoma (SMZL). She rapidly recovered normal hematological parameters and gallium-67 citrate scan showed no increased uptake. Two months after the splenectomy, however, she was readmitted with findings of 15% blasts in the peripheral blood and massive infiltration of the bone marrow by large blastoid cells. Laboratory evaluations were positive for monoclonal IgM-kappa protein. Under acute renal dysfunction, we performed a CT scan that showed bilateral enlargement of the kidneys with features suggestive of an infiltrative process besides systemic lymph node enlargement. A kidney biopsy established the diagnosis of lymphoma with renal infiltration. SMZL is characterized by an indolent clinical course, and no previous report has described SMZL with bilateral renal invasion. Complete remission was obtained after 3 cycles of chemothreapy (R-CHOP). She is undergoing 3 more courses and remains in remission 6 months after the rapid progress of her illness.
- Rinsho Ketsueki
Rinsho Ketsueki 49(1), 35-39, 2008-01-30
The Japanese Society of Hematology