Acute Cor Pulmonale Caused by Pulmonary Tumor Thrombotic Microangiopathy as the Initial Presentation of Multiple Myeloma: A Case Report

Jianwei Cui, Bingzhen Zhao, Xin Ding
https://doi.org/10.1002/pul2.70425

Abstract

Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of acute cor pulmonale, typically associated with advanced solid tumors; its occurrence as the initial presentation of multiple myeloma (MM) has not been described. A 45-year-old woman developed acute right heart failure on postoperative day 1 after uterine myomectomy. Echocardiography showed a pulmonary artery systolic pressure of 86 mmHg with a D-shaped left ventricle and right ventricular dysfunction (TAPSE 10 mm); CTPA excluded pulmonary embolism but revealed bilateral ground-glass opacities. Platelet count fell from 115 × 109/L preoperatively to 30 × 109/L. An overlooked preoperative globulin of 67 g/L prompted serum protein electrophoresis and immunofixation, which identified an IgG κ M-protein; bone marrow aspiration revealed 14% morphologically abnormal plasma cells. Presumed MM-associated PTTM was treated with heparin and sildenafil. The platelet count recovered, and a follow-up echocardiogram on day 7 showed normalized cardiac structure and function. The patient self-discontinued rivaroxaban 1 week after discharge and died 3 months later from progression of untreated MM. In CTPA-negative acute cor pulmonale with unexplained thrombocytopenia, an elevated globulin on routine biochemistry may be the only early clue to a plasma cell disorder, and timely anticoagulation with pulmonary vasodilation may buy time for definitive therapy.

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