Pulmonary tumor thrombotic microangiopathy successfully treated with corticosteroids: a case report

Abstract

BackgroundPulmonary tumor thrombotic microangiopathy is a special type of tumor thromboembolism. We report the case of a patient who developed pulmonary tumor thrombotic microangiopathy with alveolar hemorrhage. Almost all patients with pulmonary tumor thrombotic microangiopathy die within 1 week of the onset of dyspnea; however, the prognosis in this case was better, with 10 weeks of survival from presentation.Case presentationA 62-year-old Japanese man was referred to our hospital with a 4-week history of dyspnea on exertion and severe pulmonary hypertension. Five years previously, he had undergone distal gastrectomy for gastric cancer. He was afebrile, normotensive, and hypoxemic. A physical examination was unremarkable except for purpura on his upper extremities and trunk. Blood tests showed anemia and disseminated intravascular coagulation. Chest computed tomography revealed diffuse ground-glass opacities with emphysema in his upper lungs, moderate pleural effusions, mediastinal lymphadenopathy, and enlargement of the right ventricle and main pulmonary artery. A computed tomography pulmonary angiogram showed no evidence of pulmonary embolism. Lung perfusion scintigraphy showed multiple segmental defects. Although recurrence of gastric cancer was confirmed from the results of bone marrow biopsy, bronchoscopy was not performed due to bleeding diathesis. He was treated with corticosteroids, antibiotics, and platelet transfusion, following which resolution of the abnormal lung shadows and right ventricular pressure overload along with partial alleviation of respiratory failure was observed. Because of his poor performance status, he was eventually transited to palliative care and died 6 weeks after admission. Necropsy of the lung confirmed the diagnosis of pulmonary tumor thrombotic microangiopathy with alveolar hemorrhage.ConclusionsPulmonary tumor thrombotic microangiopathy should be considered in the differential diagnosis of patients with cancer who present with severe pulmonary hypertension. In pulmonary tumor thrombotic microangiopathy, local inflammation in pulmonary microvasculature may contribute to pulmonary hypertension, and regulation of inflammation using corticosteroids may help improve the prognosis.

Pulmonary hypertension in metastatic breast cancer: a case of pulmonary tumour thrombotic microangiopathy

“…TKIs are known to block PDGF and thereby might improve PTTM-associated PH. In an experimental animal model, TKIs were shown to reverse pulmonary vascular changes and cor pulmonale 20. Imatinib has been shown, in various cases reports, to improve PTTM-associated PH 15 16.…”

How to Diagnose and Treat Pulmonary Tumor Thrombotic Microangiopathy

“…6,8) Other authors have proposed steroid therapy to inhibit the inflammatory reaction between disseminated tumor cells and the pulmonary micro-vasculature. 9) Several molecular targeted agents or anti-pulmonary hypertension agents have been reported, including imatinib (an inhibitor of platelet-derived growth factor receptor) and bevacizumab (an inhibitor of vascular endothelial growth factor), [10][11][12] although they are off-label uses. We could not perform more intensive therapies beyond conventional anti-coagulation therapy due to the extensive deterioration of the systemic conditions.…”

Pulmonary tumour thrombotic microangiopathy: a rare but underappreciated complication of malignancy

“…He died within hours of his presentation. A subsequent autopsy revealed a diagnosis of pulmonary tumour thrombotic microangiopathy, which is mostly associated with carcinomas and is characterised by microemboli formation in the pulmonary vasculature . The histopathology from autopsy showed an arteriole with a tumour embolus associated with the fibrin (Figure, A) and fibrous intimal hyperplasia nearly occluding the lumen of these vessels (Figure, B).…”

“…The histopathology from autopsy showed an arteriole with a tumour embolus associated with the fibrin (Figure, A) and fibrous intimal hyperplasia nearly occluding the lumen of these vessels (Figure, B). The acute and rapid nature of the condition makes antemortem diagnosis a significant challenge, although present in 3.3% of cases of carcinomas at post mortem examination . While there are no guidelines on management, case series describe imatinib, chemotherapy or targeted therapy aimed at the primary malignancy, or corticosteroid therapy as potential options …”

“…The acute and rapid nature of the condition makes antemortem diagnosis a significant challenge, although present in 3.3% of cases of carcinomas at post mortem examination . While there are no guidelines on management, case series describe imatinib, chemotherapy or targeted therapy aimed at the primary malignancy, or corticosteroid therapy as potential options …”