Key result
Early initiation of chemotherapy, systemic steroids, and sildenafil led to dramatic clinical improvement in 1 young woman with severe pulmonary hypertension due to suspected PTTM.
Why the study?
Pulmonary tumour thrombotic microangiopathy is an exceedingly rare, rapidly fatal complication of malignancy often diagnosed postmortem, with difficult ante-mortem diagnosis.
Case Report (n=1)
Early initiation of chemotherapy, systemic steroids, and sildenafil may provide clinical benefit in patients with pulmonary tumour thrombotic microangiopathy presenting with severe pulmonary hypertension.
May support early multimodal therapy in suspected pulmonary tumour thrombotic microangiopathy with severe pulmonary hypertension; hypothesis-generating and requires prospective validation.
Pulmonary tumour thrombotic microangiopathy (PTTM) and pulmonary tumour emboli (PTE) are distinct but related complications of malignancy. The incidence of each is exceedingly rare, unfortunately often being diagnosed postmortem. Patients with PTTM and PTE typically present with dyspnoea associated with a rapid onset of hypoxia due to pulmonary hypertension (PH), and respiratory failure that is almost certain to be fatal. The prognosis is grim due to the rapidity of the clinical decline and difficulty in establishing an ante-mortem diagnosis. We present a case of new-onset severe PH in a young woman with a recently discovered breast mass. She presented with shortness of breath and experienced rapid deterioration of her cardiopulmonary status which we attributed to PTTM. With early initiation of chemotherapy, systemic steroids and sildenafil, the patient dramatically improved. Case reports have identified early use of steroids, phosphodiesterase inhibitors and other alternative therapies as providing possible benefit in PTTM.
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Tran et al. (2019) conducted a case report in Pulmonary tumour thrombotic microangiopathy (PTTM) with pulmonary hypertension (n=1). Chemotherapy, systemic steroids, and sildenafil was evaluated on Clinical improvement. Early initiation of chemotherapy, systemic steroids, and sildenafil led to dramatic clinical improvement in 1 young woman with severe pulmonary hypertension due to suspected PTTM.
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