Severe pulmonary arterial hypertension as a predominant manifestation of angioimmun- oblastic T-cell lymphoma: a case report

Angioimmunoblastic T-cell lymphoma is a rare and unique disease with peculiar clinical features among malignant lymphomas. We report here a case of severe pulmonary arterial hypertension appearing as the main manifestation of angioimmunoblastic T-cell lymphoma in a 55-year-old woman. The patient was treated with the THP-COP regimen, consisting of pirarubicin, cyclophosphamide, vincristine, and prednisolone for 1 cycle, followed by the CHOP regimen, including doxorubicin instead of pirarubicin, for 5 cycles. Soon after the initiation of chemotherapy, all the patient’s symptoms and abnormal findings, including an elevated pulmonary artery pressure, completely disappeared. As of 3 months after the end of therapy, the patient remains in complete remission. To our knowledge, this is the first report of pulmonary arterial hypertension secondary to angioimmunoblastic T-cell lymphoma.