MYELOPROLIFERATIVE NEOPLASMS AND SPLANCHNIC VEIN THROMBOSIS: CONTEMPORARY DIAGNOSTIC AND THERAPEUTIC STRATEGIES.
Jean-Jacques KiladjianBruno CassinatPublished in: American journal of hematology (2023)
Myeloproliferative neoplasms are the most common etiologies of primary splanchnic vein thrombosis, present in almost forty percent of patients with Budd-Chiari syndrome or portal vein thrombosis. Diagnosis of myeloproliferative neoplasms can be difficult in these patients because key characteristics such as elevated blood cell counts and splenomegaly are confounded by portal hypertension or bleeding complications. In recent years, diagnostic tools have improved to provide more accurate diagnosis and classification of myeloproliferative neoplasms. Although bone marrow biopsy findings remain a major diagnostic criterion, molecular markers are playing an increasing role not only in diagnosis but also in better estimating prognosis. Therefore, though screening for JAK2V617F mutation should be the starting point of the diagnostic workup performed in all patients with splanchnic vein thrombosis, a multidisciplinary approach is needed to accurately diagnose the subtype of myeloproliferative neoplasm, recommend the useful additional tests (bone marrow biopsy, search for an additional mutation using targeted next-generation sequencing), and suggest the best treatment strategy. Indeed, providing a specific expert care pathway for patients with splanchnic vein thrombosis and underlying myeloproliferative neoplasm is crucial to determine the optimal management to reduce the risk of both hematological and hepatic complications. This article is protected by copyright. All rights reserved.
Keyphrases
- pulmonary embolism
- bone marrow
- mesenchymal stem cells
- inferior vena cava
- end stage renal disease
- newly diagnosed
- healthcare
- blood pressure
- risk factors
- chronic kidney disease
- palliative care
- single cell
- ejection fraction
- atrial fibrillation
- ultrasound guided
- quality improvement
- deep learning
- stem cells
- prognostic factors
- fine needle aspiration
- drug delivery
- gene expression
- high resolution
- case report
- combination therapy
- mass spectrometry
- dna methylation
- genome wide
- arterial hypertension
- high grade