Jugular vein thrombosis: a rare presentation of atypical chronic myeloproliferative disorder in a young woman

Venous thromboembolism is common in subjects with chronic myeloproliferativ e disorders and is a recognized presenting feature of occult myeloprolifera tion. We report the case of a young woman who presented with acute thrombos is in the right jugular vein and pulmonary embolism. Splenomegaly and myelo id proliferation with bone marrow fibrosis, in the absence of the criteria for typical myeloproliferative disorders, allowed a diagnosis of an atypica l form of chronic myeloproliferative disorder. This form carries a high ris k of thrombosis and venous thromboembolism can be the presenting feature, t hough the course Is often indolent. Acute thrombosis in the right jugular v ein has not been so far described in these subjects. The outcome of young p eople with myelofibrosis is unpredictable, but a normal level of hemoglobin and the absence of blast cells and constitutional symptoms at presentation identifies subjects with a low probability of rapid disease progression. ( C) 1999 Ferrata Storti Foundation.