Key result
Imatinib dramatically lowers mPAP and alleviates PH symptoms in a patient with PTTM.
Why the study?
Pulmonary tumour thrombotic microangiopathy is a rare, often lethal cancer complication causing progressive pulmonary hypertension, with limited therapeutic options.
Does imatinib improve pulmonary hypertension in patients with pulmonary tumour thrombotic microangiopathy?
Case Report (n=1)
Does imatinib improve pulmonary hypertension in patients with pulmonary tumour thrombotic microangiopathy?
Imatinib may be an effective therapeutic option for severe pulmonary hypertension secondary to pulmonary tumour thrombotic microangiopathy.
Imatinib may improve PH in PTTM; extends sparse case reports but leaves open need for controlled trials before practice change.
Pulmonary tumour thrombotic microangiopathy (PTTM) is a rare complication of cancer, which can be lethal due to progressive pulmonary hypertension (PH). Several case reports have demonstrated that imatinib, a platelet-derived growth factor receptor-tyrosine kinase inhibitor, can improve severe PH in patients with PTTM.We describe the case of a 56-year-old woman. Her mean pulmonary arterial pressure (mPAP) was 47 mm Hg, and her dyspnoea worsened rapidly over several days. Although pulmonary embolism was not observed on CT, enlargement of the para-aortic lymph nodes was detected. Gastro-oesophageal endoscopy revealed signet-ring cell carcinoma. We diagnosed her as having PTTM based on her clinical course, and started treatment with imatinib. Five days after its administration, her mPAP decreased dramatically. She was discharged and lived without symptoms of PH until her death due to systemic metastasis of carcinoma. In some cases of PTTM, imatinib may be an effective therapeutic option for PH.
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Kubota et al. (2017) conducted a case report in Pulmonary tumour thrombotic microangiopathy (PTTM) induced by gastric cancer (n=1). Imatinib was evaluated on Mean pulmonary arterial pressure (mPAP) and symptoms of pulmonary hypertension. Imatinib dramatically decreased mean pulmonary arterial pressure and alleviated symptoms of pulmonary hypertension in a patient with pulmonary tumour thrombotic microangiopathy.
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