[Polyclonal hypergammaglobulinemia, pulmonary hypertension, hyperviscosity and coagulopathy in a patient with Rhupus]

We present the case of a 29-year-old male diagnosed with Rhupus syndrome, hospitalized for severe pulmonary hypertension refractory to standard drugs. On admission, he presented clinical findings compatible with blood hyperviscosity in the framework of extreme polyclonal hypergammaglobulinemia. Due to the rapid coagulation of the samples and the lack of clot retraction, it was not possible to obtain serum to measure the initial serum viscosity, although it was found to be elevated in the course of treatment. Coagulation tests showed prolongation of prothrombin (PT), activated partial thromboplastin (APTT) and thrombin (TT) times, with decreased coagulation factors except factor VIII, in addition to hypofibrinogenemia. PT and TT corrected with normal plasma, but not APTT, the late probably due to an interference phenomenon secondary to hypergammaglobulinemia. Elevated D-dimer and shortened euglobulin lysis time were observed as well as an expression of hyperfibrinolysis. He also presented transitory ascites with a seroascitic gradient greater than 1.1. The transjugular liver biopsy showed moderate steatosis, although the sample was scarce. After receiving four pulses of intravenous cyclophosphamide, right ventricular function improved, pulmonary arterial pressure was reduced, coagulation tests were normalized, and gamma globulin values were halved. Concomitantly, the general condition improved. It was interpreted that blood hyperviscosity caused by hypergammaglobulinemia acted as an aggravating factor for pulmonary hypertension The coagulation disorders were ascribed to chronic liver disease. The favorable response to treatment with cyclophosphamide is highlighted.