Key result
Heparin and sildenafil temporarily reverse acute cor pulmonale from myeloma-associated PTTM.
Why the study?
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare cause of acute cor pulmonale typically linked to advanced solid tumors, but its occurrence as the initial presentation of multiple myeloma has not been described.
Case Report (n=1)
No
In patients with CTPA-negative acute cor pulmonale and unexplained thrombocytopenia, an elevated globulin level may be a crucial early indicator of a plasma cell disorder causing pulmonary tumor thrombotic microangiopathy.
May support heparin-sildenafil trial for temporary stabilization in myeloma-PTTM cor pulmonale; hypothesis-generating and leaves open prospective validation.
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 × 10 9 /L preoperatively to 30 × 10 9 /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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Cui et al. (2026) conducted a case report in Multiple myeloma with acute cor pulmonale due to presumed pulmonary tumor thrombotic microangiopathy (n=1). Unfractionated heparin and sildenafil was evaluated on Clinical recovery of cardiac structure/function and platelet count. Treatment with heparin and sildenafil temporarily normalized cardiac function in a 45-year-old woman presenting with acute cor pulmonale from myeloma-associated PTTM before death from myeloma progression.
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