表现为神经淋巴瘤病的外周T细胞淋巴瘤一例

Peripheral T-cell lymphoma presenting as neurolymphoma: a case report

31岁男性患者,全身反复出现红斑、结节伴肢端麻木感8年。体格检查:全身皮肤散在直径1～5 cm淡红至紫红色斑片和结节。双侧颈部及腹股沟可触及数个直径<1 cm的淋巴结。双手尺侧及双足深浅感觉减退,双下肢腱反射减弱。四肢肌电图检查示:获得性多灶性感觉运动神经病。PET-CT提示淋巴瘤。右肘尺神经组织病理示:弥漫增生的淋巴样细胞;免疫组化染色示:肿瘤细胞CD3、CD5、TIA-1均阳性,Ki-67约40%～70%阳性。TCR克隆性基因重排阳性。左小腿皮肤结节组织病理:表皮和真皮浅层、皮肤附属器及部分血管周围较多异形淋巴样细胞浸润;免疫组化染色示:CD3、CD5、GrB、TIA-1均阳性,Ki-67约40%阳性。诊断为:(1)非霍奇金淋巴瘤IVB期;(2)外周T细胞淋巴瘤,非特指型;(3)神经淋巴瘤病。治疗:先后采取吉西他滨+培门冬酶、DICE、CHOP、EPOCH+来那度胺、BECOP+西达苯胺等化疗方案,目前口服西达苯胺治疗,原皮损逐渐消退,但双小腿仍有新发结节,总体病程缓慢进展。

A 31-year-old male patient presented with recurrent erythema, nodules throughout the body and acral numbness for 8 years, without pain or itching. Physical examination: light red to purple-red patches and nodules 1-5 cm in diameter scattered over the skin of the whole body, bilateral cervical and inguinal lymph nodes less than 1 cm in diameter could be touched. Both the deep and superficial sensation of ulnar side of both hands and feet was decreased, and the tendon reflex of both lower limbs was weakened. The electromyogram of extremities showed acquired multifocal sensorimotor neuropathy. PET-CT suggested lymphoma. Histopathology of the right elbow ulnar nerve tissue showed diffuse proliferation of lymphoid cells, immunohistochemical staining showed that the tumor cells were positive for CD3, CD5 and TIA-1, Ki-67 was about 40%-70%.TCR clonal gene rearrangement was positive. Histopathology of the cutaneous nodule in the leg: more heteromorphic lymphoid cells infiltrated in the epidermis and superficial dermis, around skin appendages and part of perivascular areas. Immunohistochemical staining showed that CD3, CD5, GrB and TIA-1 were all positive, and Ki-67 was about 40%. The diagnosis was(1)Non-Hodgkin’s lymphoma IVB;(2)Peripheral T-cell lymphoma, NOS;(3)Neurolymphomatosis. Therapy: Gemcitabine+Pempermutase, DICE, CHOP, EPOCH+Lenadomide, BECOP+Cedaraniline and other chemotherapy regimens had been adopted successively. With the current treatment of oral Cedaraniline, the original lesions gradually subsided, but there were still new lesions appeared in both lower extremities, which showed a slow progression.