副腎に著明な浸潤をきたし副腎不全を合併したマントル細胞リンパ腫 Mantle cell lymphoma markedly infiltrated into adrenal glands with adrenal insufficiency
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症例は66歳男性。平成14年5月より持続する上腹部痛と背部痛，体重減少を主訴に受診。表在リンパ節腫脹はなし。WBC 3430/μ<i>l</i>, Hb 13.0 g/d<i>l</i>, PLT 174000/μ<i>l</i>, LDH 1368IU/<i>l</i>, sIL-2R 2630U/m<i>l</i>, ACTH 132pg/m<i>l</i>, cortisol 7.4 μg/d<i>l</i>. CTで両側副腎腫瘤があり，Gaシンチで両側副腎への集積亢進を認めた。生検は出血傾向あり不能だったが，汎血球減少の進行あり骨髄穿刺を施行したところ，異型細胞浸潤を認めた。表面マーカーはCD5(+) cyclin D1(+) CD19(+) CD20(+) HLA-DR(+)。マントル細胞リンパ腫(stage IV B)と診断しhydrocortisone補充のうえCHOP療法を開始したところ腫瘤の縮小を得た。副腎不全を合併し，副腎に著明な浸潤をきたした悪性リンパ腫は稀であり，mantle cell lymphomaの組織型を有する症例の報告は見当たらない。文献的考察を加えて報告する。
A 66-year-old male was admitted to our hospital complaining of bilateral hypochondrial pain, back pain and loss of weight in May, 2002. Superficial lymph nodes were not palpable on admission. The leukocyte count was 3430/μ<i>l</i>, hemoglobin concentration, 13.0 g/d<i>l</i>, and platelet count, 174000/μ<i>l</i>. LDH, soluble IL-2 receptor, ACTH and cortisol values were out of the normal range (LDH 1368IU/<i>l</i>, sIL-2R 2630U/m<i>l</i>, ACTH 132pg/m<i>l</i>, cortisol 7.4μg/d<i>l</i>). Abdominal CT scan showed bilateral adrenal masses, and abnormal uptake of Ga-scintigraphy was seen correspondent with the bilateral adrenal masses. The histological diagnosis of bilateral adrenal masses cannot be performed because of the bleeding tendency, but atypical cells were observed in the patient's bone marrow aspirate. Surface marker analysis of atypical cells showed CD5+, cyclin D1+, CD19+, CD20+ and HLA-DR+. From these results we diagnosed this case as a mantle cell lymphoma (stage IV B) markedly infiltrated into the adrenal glands with adrenal insufficiency. The bilateral adrenal masses dramatically reduced in size after CHOP chemotherapy with hydrocortisone supplementation. We report on the present case and summarize the reports of adrenal grand-infiltrating lymphomas.
収録刊行物
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- 臨床血液 = The Japanese Journal of Clinical Hematology
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臨床血液 = The Japanese Journal of Clinical Hematology 45(7), 551-556, 2004-07-30
The Japanese Society of Hematology