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患者男,26岁。入院前4个月无明显诱因于颈项部、左侧鼻根部、肘窝出现数个暗红色、蚕豆大结节,无明显自觉症状,部分结节可自行消退。2个月后结节增多、扩大,累及躯干、四肢,部分表面溃烂。实验室检查示:血清LDH升高,EBV-Ig G(+),EBV-DNA定量﹤5.00+02 copies/m L(-)。外周血涂片及骨髓穿刺未见异常。彩超示:双侧锁骨上窝、腋窝、腹股沟及左侧腘窝淋巴结肿大。PET-CT示:右侧上颌窦、右肺上叶、心包右缘软组织结节影代谢活跃;左侧锁骨上区、纵膈主动脉弓旁、胸骨旁、右侧腋窝、腹腔及右侧腹股沟多发淋巴结肿大代谢活跃;全身皮下、肌间多发软组织结节或肿块影代谢活跃。皮肤结节组织病理示:真皮全层及皮下组织致密单一核细胞浸润,可见异型细胞及不典型核分裂相。免疫组织化学:CD20(-),CD3(+),CD43(+),CD30(+),ALK(+),EMA(+),CD56(+),TIA-1(+),Granzyme B(+),CD15(-),MPO(-),CK(-),Ki-67(80%+)。纵隔淋巴结活检与上述结果一致。诊断:ALK阳性间变性大细胞淋巴瘤,Ⅳ期。确诊后转入肿瘤病区治疗,患者随访中。
Male patient, 26 years old. Four months before admission, no obvious incentive for the neck, the left nasal root, elbow nest appeared several dark red, broad bean nodules, no obvious symptoms, some nodules can subside on their own. 2 months after the nodules increased, expanding, involving the trunk, limbs, some ulceration on the surface. Laboratory tests showed elevated serum LDH, EBV-Ig G (+), and EBV-DNA quantitation <5.00 + 02 copies / mL (-). No abnormalities in peripheral blood smear and bone marrow aspiration. Choi ultrasound show: bilateral supraclavicular fossa, armpits, groin and left popliteal lymph nodes. PET-CT showed: the right maxillary sinus, right upper lobe, pericardial right soft tissue nodules shadow metabolism; left supraclavicular, mediastinal aortic arch, parasternal, right axillary, abdominal and right groin multiple lymph nodes Swollen metabolism active; systemic subcutaneous, multiple soft tissue nodules or mass shadow metabolism active. Tissue and pathology of the skin nodules showed: dense mononuclear cells infiltrated all over the dermis and subcutaneous tissue, showing atypical cells and atypical mitotic phases. Immunohistochemistry: CD20 (-), CD3 (+), CD43 +, CD30 +, ALK +, EMA +, CD56 +, TIA- 1 +, Granzyme B + ), CD15 (-), MPO (-), CK (-), Ki-67 (80% +). Mediastinal lymph node biopsy consistent with the above results. Diagnosis: ALK positive anaplastic large cell lymphoma, stage Ⅳ. Into the tumor after diagnosis of disease treatment, patient follow-up.