Lymphoma with specific affinity to endocrine organs
Lymphoma with specific affinity to endocrine organs
复制标题
与内分泌器官具有特异性亲和力的淋巴瘤
DOI：
10.1007/s00277-011-1372-3
复制
发表时间：
2011
期刊：
影响因子：
--
通讯作者：
森田剣
中科院分区：
文献类型：
--
作者：
Y. Sawai;H. Murata;M. Horii;K. Koto,T. Matsui;N. Horie;Y. Tsuji;E. Ashihara;T. Maekawa;T. Kubo;S. Fushiki;森田剣
亲爱的编辑，淋巴瘤只影响一个内分泌器官是罕见的。不用说，同时累及两个或两个以上的内分泌器官的情况会少得多。在此，我们报告一例垂体和双侧肾上腺的非霍奇金淋巴瘤。54岁日本男性，以头痛、发热、复视、右上睑下垂2个月为临床表现。他过去的病史一般。神经学检查证实有右动眼神经麻痹。实验室数据显示轻度贫血，血清乳酸脱氢酶升高687 U/L，可溶性白细胞介素-2受体升高1297 U/mL。促甲状腺素水平0.01 mIU/L（参考范围0.4-4.3 mIU/L），游离甲状腺素8.0 pmol/L (10.6-21.0 pmol/L)，促黄体生成素0.1 IU/L (1.0-8.0 IU/L)，促卵泡激素2.3 IU/L (2.1-18.6 IU/L)，促肾上腺皮质激素1.5 pmol/L (1.6-13.9 pmol/L)，皮质醇63 nmol/L （150-600 nmol/L）。然后开始用甲状腺素和皮质类固醇进行激素替代治疗。脑磁共振成像（MRI）显示鞍区肿块延伸至右侧海绵窦（图1a）。计算机断层扫描（CT）显示肾上腺肿瘤。未见淋巴结病变和肝脾肿大。F-18氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）联合CT显示垂体（图1b）和双侧肾上腺（图1c）的摄取增加。经鼻内镜对鞍区肿块进行活检。组织病理学显示大淋巴样细胞弥漫性增殖，CD20和bcl-2阳性，CD3、CD10和CD56阴性。Epstein-Barr病毒编码RNA-1原位杂交结果为阴性。免疫球蛋白重链基因单克隆重排。骨髓活检显示相同免疫表型的大淋巴样细胞浸润。骨髓抽吸的细胞遗传学研究显示所有中期核型正常。脑脊液正常。因此我们诊断为弥漫性大B细胞淋巴瘤（DLBCL），累及垂体、双侧肾上腺和骨髓。患者接受6个周期的利妥昔单抗、环磷酰胺、阿霉素、长春新碱和强的松龙（R-CHOP）方案治疗，完全缓解（图1d-f）。神经系统症状完全消失，垂体功能减退部分改善。令人惊讶的是，这种特殊分布的淋巴瘤是复发性的。Li等人报道了首例77岁男性被诊断为垂体、双侧肾上腺和骨髓弥漫性大细胞淋巴瘤的病例。Ogilvie等人随后报道了一例累及垂体和双侧肾上腺的59岁男性DLBCL患者。包括我们在内的三个病例都有一个共同的特点，即垂体前叶和
Dear Editor, Lymphoma affecting only one endocrine organ is rare. Needless to say, simultaneous involvement of two or more endocrine organs would occur much less frequently. Here, we describe a case of non-Hodgkin’s lymphoma of the pituitary and bilateral adrenal glands. A 54-year-old Japanese male presented with headache, fever, diplopia, and right ptosis for 2 months. His past medical history was unremarkable. Neurological examination confirmed the presence of right oculomotor nerve palsy. Laboratory data were notable for mild anemia, elevated serum lactate dehydrogenase level of 687 U/L, and soluble interleukin-2 receptor level of 1,297 U/mL. Anterior hypopituitarism was demonstrated by thyrotropin level of 0.01 mIU/L (reference range, 0.4–4.3 mIU/L), free thyroxine 8.0 pmol/L (10.6–21.0 pmol/L), luteinizing hormone 0.1 IU/L (1.0–8.0 IU/L), follicle-stimulating hormone 2.3 IU/L (2.1–18.6 IU/L), adrenocorticotropic hormone 1.5 pmol/L (1.6–13.9 pmol/L), and cortisol 63 nmol/L (150–600 nmol/L). Hormone replacement therapy was then initiated with thyroxine and corticosteroid. Brain magnetic resonance imaging (MRI) revealed a sellar mass extending to the right cavernous sinus (Fig. 1a). Computed tomography (CT) scan showed tumors on the adrenal glands. Lymphadenopathy and hepatosplenomegaly were not noted. F-18 fluorodeoxyglucose-positron emission tomography (FDG-PET) combined with CT exhibited increased uptakes in the pituitary (Fig. 1b) and bilateral adrenal glands (Fig. 1c). Endoscopic transnasal biopsy of the sellar mass was performed. Histopathology revealed diffuse proliferation of large lymphoid cells that were positive for CD20 and bcl-2 and negative for CD3, CD10, and CD56. Epstein-Barr virus encoded RNA-1 in situ hybridization resulted in a negative study. Immunoglobulin heavy chain gene was monoclonally rearranged. Bone marrow biopsy disclosed infiltration of large lymphoid cells of the same immunophenotype. Cytogenetic study of the bone marrow aspirate showed normal karyotype in all metaphases examined. The cerebrospinal fluid was normal. We therefore made a diagnosis of diffuse large B cell lymphoma (DLBCL) involving the pituitary, bilateral adrenal glands, and bone marrow. The patient received 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) regimen and achieved complete remission (Fig. 1d–f). Neurologic symptoms thoroughly disappeared and hypopituitarism partially improved.Surprisingly enough, lymphoma with such specific distribution is recurrent. Li et al. described the first case of a 77-year-old male that was diagnosed as diffuse large cell lymphoma of the pituitary, bilateral adrenal glands, and bone marrow [1]. Ogilvie et al. subsequently reported a 59-year-old male patient of DLBCL involving the pituitary and bilateral adrenal glands [2]. The three cases including ours shared the common feature that the anterior pituitary and