以肺动脉高压为首要表现的原发性浆细胞白血病一例报道并文献复习

Pulmonary hypertension as a dominant clinical picture of primary plasma cell leukemia： a case report and literature review

目的探讨浆细胞白血病患者肺动脉高压可能的发生机制。方法介绍我院收治的1例以肺动脉高压为首要表现的原发性浆细胞白血病的临床特点和实验室检查结果，并结合国内外文献，分析其肺动脉高压的发生机制。结果患者男性，24岁，以胸闷、气喘伴右侧胸腔积液起病，超声心动图发现肺动脉高压（肺动脉收缩压：52mm Hg），外周血白细胞最高达82．80×10^9／L，分类不明细胞占89％，伴贫血和血小板减少，血γ-球蛋白、IgG及血、尿免疫球蛋白L轻链明显升高，骨髓检查确诊为原发性浆细胞白血病，经COMEP方案化疗。随自血病病情缓解，肺动脉高压也迅速缓解。国外文献报道6例多发性骨髓瘤伴肺动脉高压，大多与自体干细胞移植和沙立度胺化疗有关。结论原发性浆细胞白血病以肺动脉高压起病，国内外罕见。白细胞淤滞、高黏滞及高凝状态、纤溶活性降低均可能参与其发生机制。

Objective To explore the possible pathogenesis of pulmonary hypertension （PH） in a patient with primary plasma cell leukemia（PPCL）. Methods The clinical features and laboratory tests of a patient with PPCL in which PH has been the onset manifestation were analyzed,and the relevant literatures were reviewed. Results A 24-year-old previously healthy male presented to our hospital with a history of dyspnea,tachypnea and right pleural effusion, transthoracic echocardiography revealed a PH （PASH = 52 mm Hg）, laboratory tests showed WBC （ 43.46-82.80 ） × 10^9/L, unclassified cell 89%, anemia, thrombocytopenia, and markedly elevated levels of gammaglobulin, IgG, Bence-Jones proteinurin, bone marrow aspirate led ultimately to a diagnosis of PPCL. Successful treatment of his PPCL with a ＂COMEP＂ chemotherapy cycle resulted in dramatic and significant improvement of＂ his PH. 6 cases with multiple myeloma accompanied by PH were reported in foreign literatures, most of them were probably associated with peripheral blood stem cell transplantation and/or thalidomide chemotherapy. Conclusions As far as known,it is very rare that PH has been the dominant clinical picture of PPCL. Leukostasis, hyperviscosity and hypercoagulable state,decreased fibrinolytic activity may play important roles in the pathophysiology of the disease.