Abstract
Mesenteric venous thrombosis (MVT) presents with a wide range of clinical presentations depending on the vessel involved, degree of thrombosis, and the extent of bowel wall ischemia. MVT usually has an insidious presentation and is often a forerunner of an underlying disorder. Essential thrombocythemia (ET) presenting itself as MVT along with splenic infarction is a rare presentation. Here, we report the case of a 54-year-old female with massive splenomegaly, thrombocytosis, and acute splenic and portal venous thrombosis along with multiple splenic infarcts. Bone marrow suggested ET with JAK2V617F mutation positivity. She was managed conservatively and made an uneventful recovery.
Highlights
Essential thrombocythemia (ET) is an acquired myeloproliferative disorder (MPD) characterized by a persistent elevation of platelet count
Hematological conditions involving thrombophilia should be kept under consideration in the evaluation of thrombotic episodes, such as prothrombin gene mutation and factor V Leiden gain-of-function mutations or hematopoietic system-related clonal disorders like MPD which include chronic myeloid leukemia (CML) and BCR-ABL negative disorders
BCR-ABL negative MPD is associated with a somatic mutation in JAK2 which has been identified in almost all cases of PV (>95% positive), in 50-70% of ET and 50-60% of PMF patients [6]
Summary
Essential thrombocythemia (ET) is an acquired myeloproliferative disorder (MPD) characterized by a persistent elevation of platelet count. Around 8-15% of the cases are idiopathic and the remaining have a direct relation with various conditions, MPD [2] Diagnosis at these unusual sites is crucial in patients with ET as thrombosis significantly affects the disease outcome and is associated with severe organ damage and high mortality [3]. Bone marrow examination revealed hypercellular marrow (80-85% cellularity), megakaryocytic hyperplasia with focal clustering, and the presence of staghorn nuclei. These changes were suggestive of a myeloproliferative neoplasm, possibly ET (Figure 2). Portal vein thrombosis was managed with heparin followed by warfarin with international normalized ratio (INR) monitoring daily while the patient was admitted and every three to five days after discharge. On follow-up evaluation six weeks later, she was asymptomatic with a platelet count of 95 x 109/L and a total leucocyte count of 4.5 x 10 9/L
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