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Portal venous thrombosis in a young patient with idiopathic myelofibrosis and intrahepatic extramedullary hematopoiesis: a difficult diagnosis, prognosis and management

Leukemia volume 21, pages 2373–2375 (2007)Cite this article

Chronic myeloproliferative syndromes (MPSs) occur much less commonly in children than in adults. Idiopathic myelofibrosis (IMF) is characterized by stem cell-derived clonal myeloproliferation and shows constitutional symptoms, increasing splenomegaly, bone marrow fibrosis, extramedullary hematopoiesis, progressive anemia and cytopenias; hemorrhages and above all thrombosis are frequent complications.1

Portal vein thrombosis (PVT) is associated with liver cirrhosis, hepatocellular carcinoma, intra-abdominal infections, hemodynamic factors, hypercoagulability states and thrombophilic disorders, including MPS.2 Chronic PVT can be often recognized casually, after a fortuitous diagnosis of hypersplenism or portal hypertension or while having biliary symptoms related to cholangiopathy or only initial ascites; moreover, acute PVT can present with life-threatening events such as gastrointestinal hemorrhage and intestinal infarction. The PVT can result from the concurrence of several acquired factors, with or without the presence of genetic ones. Cirrhotic PVT patients usually have an advanced liver disease; in these patients, the concomitant mutation G20210A of the prothrombin gene increases more than fivefold the risk.3 About 70% of patients with PVT have a general risk factor, most commonly MPS, while local precipitating risk factors, such as an abdominal inflammatory focus, can be identified in about 30%. A moderate hyperhomocysteinemia is associated with heightened risk,4 and PVT has been reported in patients with antiphospholipid syndrome.5 PVT in children or young adults is reported anecdotically.4

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Acknowledgements

We wish to thank AIL, Italian Association against Leukaemia, Lymphoma and Myeloma, Pescara Section.

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Authors and Affiliations

  1. Department of Hematology, ‘Spirito Santo’ Hospital, Pescara, Italy

    S Pulini, A Spadano & G Fioritoni

  2. Department of Internal Medicine, Liver Unit, ‘Spirito Santo’ Hospital, Pescara, Italy

    E D'Amico

  3. Department of Radiology, ‘SS Annunziata’ Hospital, University of Chieti, Chieti, Italy

    R Basilico & M Mereu

  4. Institute of Hematology and Clinical Oncology, L. and A. Seragnoli, Hematology and Hematopathology Units, St Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy

    F Bacci

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  1. S Pulini
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  2. E D'Amico
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  3. R Basilico
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Correspondence to S Pulini.

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Pulini, S., D'Amico, E., Basilico, R. et al. Portal venous thrombosis in a young patient with idiopathic myelofibrosis and intrahepatic extramedullary hematopoiesis: a difficult diagnosis, prognosis and management. Leukemia 21, 2373–2375 (2007). https://doi.org/10.1038/sj.leu.2404789

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