Epoprostenol-induced hypersplenism in portopulmonary hypertension.

Portopulmonary hypertension (POPH) is a not infrequent but serious complication of liver cirrhosis. Continuous intravenous epoprostenol infusion is a treatment option for this condition. Progressive splenomegaly with pancytopenia (hypersplenism) is associated with epoprostenol use in POPH. After recognizing a case of epoprostenol-induced hypersplenism that resolved upon stopping the drug, the authors retrospectively reviewed all patients treated with epoprostenol at the center for both POPH and pulmonary hypertension due to other causes. Five of 11 patients with POPH developed hypersplenism secondary to epoprostenol. In 1 patient, and possibly in a second, the hypersplenism resolved upon discontinuation of epoprostenol. None of 9 patients with pulmonary hypertension due to other causes developed splenomegaly. This report confirms hypersplenism as a complication of epoprostenol therapy for POPH. Furthermore, the authors demonstrate for the first time that hypersplenism may be reversed by stopping the medication and propose a mechanism for this phenomenon.

Thrombotic thrombocytopenic purpura and Graves disease.

Patients with an autoimmune disease have a propensity for development of a second autoimmune disease. We report the first instance of a patient with both idiopathic thrombotic thrombocytopenic purpura (TTP) and Graves disease. The TTP remitted with a combination of plasmapheresis and prednisone. Methimazole led to sustained remission of the hyperthyroid state within 6 weeks. Although hyperthyroidism may induce immune imbalance causing autoimmunity, it is unclear if this influenced the development of TTP in our patient and if treatment of hyperthyroidism alone could have resulted in the cure of both diseases.

Acute promyelocytic leukemia and HIV-1 infection: case report and review of the literature.

We report a 27-year-old man with HIV-1 infection who developed acute promyelocytic leukemia (APL) with a novel complex three-way chromosomal translocation t(15;16;17). Induction of remission and consolidation with all-trans-retinoic acid (ATRA)- and anthracycline-based chemotherapy was followed by maintenance therapy consisting of ATRA, 6-mercaptopurine (6-MP), and methotrexate (MTX). Highly active antiretroviral therapy (HAART) was continued with brief interruptions. He remains in complete remission 40 months after diagnosis.

Thymoma and myotonic dystrophy: successful treatment with chemotherapy and radiation: case report and review of the literature.

We present the case of a 42-year-old woman with myotonic dystrophy and thymoma. She was treated with combination chemotherapy followed by external beam radiation, and remains in remission 19 months after thymoma was diagnosed. The myotonic dystrophy is unchanged. Only six cases of this nature have been reported in the literature, and this patient is the first to be successfully treated with combined modality therapy.