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

Objective To explore the possible pathogenesis of pulmonary hypertension (PH) in apatient with primary plasma cell leukemia(PPCL). Methods The clinical features and laboratory tests of apatient with PPCL in which PH has been the onset manifestation were analyzed,and the relevant literatureswere reviewed. Resaits A 24-year-old previously healthy male presented to our hospital with a history ofdyspnea,tachypnea and right pleural effusion, transthoraeic echocardiography revealed a PH (PASH=52mmHg), laboratory tests showed WBC (43.46-82.80)×10 /L, unclassified cell 89%, anemia,thrombocytopenia, and markedly elevated levels of gammaglobulin, IgG, Benee-Jones proteinurin, bonemarrow 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 multiplemyeloma accompanied by PH were reported in foreign literatures, most of them were probably associatedwith peripheral blood stem cell transplantation and/or thalidomide chemotherapy. Conclusions As far asknown,it is very rare that PH has been the dominant clinical picture of PPCL. Leukostasis, hyperviscosity andhypercoagulable state,decreased fibrinolytic activity may play important roles in the pathophysiology of the disease. Key words: Pulmonary hypertension; Plasma cell leukemia/multiple myeloma