累及皮肤的套细胞淋巴瘤一例

Blastoid mantle cell lymphoma involving the skin： a case report

患者男，53岁，躯干四肢紫红色结节、斑块半月，眶周肿胀4d。体检：全身多处浅表淋巴结肿大，脾肿大。血钙3．12mmol／L，LDH853U／L，血免疫固定电泳示单克隆免疫球蛋白IgM阳性（K链型），正电子发射断层显像／）（线计算机体层成像检查示全身多处淋巴结肿大，咽后壁肿块，脾脏大。颈部皮肤结节组织病理检查示皮下脂肪组织内大量异形淋巴样细胞弥漫浸润，体积中等偏大，核圆形，核仁较明显，核分裂易见。免疫组化标记示异形细胞L26、CD79a、Bcl-2、细胞周期蛋白D1均阳性，多发性骨髓瘤原癌基因1部分阳性，Ki-67阳性率〉80％，CD5、CD21、CD23、CD38、CD3、CDl0、Bel-6、CD45RO、末端脱氧核苷酸转移酶（TdT）、髓过氧化物酶（MPO）、CD30、间变性淋巴瘤激酶（ALK）、CDll7、CD34均阴性。荧光原位杂交检测示t（11；14）CCNDl／IGH融合基因阳性。诊断：伴皮肤和眶周受累的套细胞淋巴瘤（母细胞变异型）合并高钙血症。治疗：给予利妥昔单抗注射液联合环磷酰胺、长春新碱、多柔比星、地塞米松，交替以大剂量甲氨蝶呤及阿糖胞苷静脉注射，第3天血钙降至正常，第6天病情得到迅速控制，皮损及眶周肿胀明显消退，但随访治疗1个月后，患者最终死于严重肺部感染。

A 53-year-old man presented with prunosus nodules and plaques on the trunk and extremities for half a month and with periorbital swelling for 4 days. Hematological examination in a local hospital showed thrombocytopenia, and pulsed cortieosteroid therapy did not work. On physical examination, there were splenomegaly and multiple enlarged superficial lymphnodes. Serum calcium and l-lactate dehydrogenase （LDH） were increased to 3.12 mmol/L and 853 U/L, respectively. Serum immunofixation electrophoresis evidenced the presence of a monoclonal immunoglobulin IgM （K chain）. Positron emission tomography （PET-CT） showed abnormal uptake in multiple lymph nodes, back wall of the pharynx, and spleen. The biopsy of a nodule in the neck revealed a diffuse infiltration of numerous atypical lymphoid cells in the subcutaneous fat tissue, which were medium-sized with round nuclei, obvious nueleoli and karyokinesis. Immunophenotyping of the abnormal lymphoeytes indicated positive reactions for L26, CD79a, Bcl-2, cyclin D1, muhiple myeloma oneogene 1 （partly）, Ki-67 （〉80%）, but negative for CD5, CD21, CD23, CD38, CD3, CDIO, Bcl-6, CD45RO, terminal deoxynucleotidyl transferase （TdT）, myeloperoxidase （MPO）, CD30, anaplastic lymphoma kinase （ALK）, CDll7, or CD34. Fluorescence in situ hybridization （FISH） revealed the presence of a fusion gene （t（11：14） CCND1/IGH） in the abnormal lymphocytes. Based on the above findings, the diagnosis was made as blastoid mantle cell lymphoma with skin and periorbital involvement complicated by hypercalcemia. After treatment with rituximab injection, cyclophosphamide, vincristine, doxorubicin, dexamethasone, and intermittent treatment with intravenous high dose of methotrexate and cytarabine （R-Hyper-CVAD）, serum calcium returned to a normal level three days later, and the patient made a quick and excellent recovery on the 6th day, with the regression of skin lesions and periorbital swelling. Unfortunately, the patient eventually died of severe pulmonary infection one month later.