%0 Journal Article
%T Severe pulmonary arterial hypertension as a predominant manifestation of angioimmun- oblastic T-cell lymphoma: a case report
%A Jian Hua
%A Yasunobu Iwaki
%A Morihiro Inoue
%A Masao Hagihara
%J Journal of Hematological Malignancies
%D 2012
%I 
%R 10.5430/jhm.v2n3p26
%X 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.
%U http://www.sciedu.ca/journal/index.php/jhm/article/view/1023