Khawandanah Mohamad, Kraus Teresa, Cherry Mohamad
Hematology-Oncology Section, Department of Medicine, Stephenson Cancer Center, The University of Oklahoma Health Sciences Center, 800 NE 10th Street, Oklahoma City, OK 73104, USA.
Department of Pathology, The University of Oklahoma Health Sciences Center, 940 Stanton L. Young Boulevard, BMSB 451, Oklahoma City, OK 73104, USA.
Case Rep Hematol. 2014;2014:642868. doi: 10.1155/2014/642868. Epub 2014 May 13.
Cutaneous Hodgkin lymphoma is a rare condition. Specific neoplastic involvement can be primary (confined to the skin) or secondary to systemic involvement (metastatic). Cutaneous involvement by HL usually occurs late in the course and is associated with poor prognosis; however in some cases it can exhibit indolent behavior. Skin involvement with nonspecific cutaneous findings may represent a paraneoplastic syndrome. We describe a case of 46-year-old white male patient presented with rash and lymphadenopathy which led to the diagnosis of stage IVE mixed cellularity classical Hodgkin lymphoma with skin involvement. His disease was refractory to multiple lines of chemotherapy including (1) AVD (doxorubicin/bleomycin/dacarbazine), (2) brentuximab, and (3) bendamustine, he later achieved complete remission with (4) GCD (gemcitabine/carboplatin/dexamethasone) salvage regimen. Bleomycin was not given secondary to poor pulmonary function tests. His treatment was complicated after AVD with multiple pneumothoraces which unmasked the diagnosis of ZZ phenotype alpha-1 antitrypsin (ATT) deficiency. Simultaneous existence of Hodgkin lymphoma and ATT is rarely reported.
皮肤型霍奇金淋巴瘤是一种罕见疾病。特定的肿瘤累及可以是原发性的（局限于皮肤）或继发于全身累及（转移性）。HL的皮肤累及通常发生在病程晚期，且预后较差；然而在某些情况下，它可能表现为惰性病程。伴有非特异性皮肤表现的皮肤累及可能代表一种副肿瘤综合征。我们描述了一例46岁白人男性患者，其因皮疹和淋巴结病就诊，最终诊断为伴有皮肤累及的IV期E组混合细胞型经典型霍奇金淋巴瘤。他的疾病对多线化疗耐药，包括（1）AVD（阿霉素/博来霉素/达卡巴嗪）、（2）brentuximab和（3）苯达莫司汀，后来通过（4）GCD（吉西他滨/卡铂/地塞米松）挽救方案实现了完全缓解。由于肺功能检查结果不佳，未给予博来霉素。他在接受AVD治疗后出现了多次气胸，这使得ZZ表型α-1抗胰蛋白酶（ATT）缺乏症得以诊断，治疗变得复杂。霍奇金淋巴瘤和ATT同时存在的情况鲜有报道。
