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[Budd-Chiari syndrome associated with chronic myeloproliferative syndromes: analysis of 6 cases]
F Cobo1, F Cervantes, J C García-Pagán
1Escuela de Hematología Farreras-Valentí, Departamento de Medicina, Hospital Clínic i Provincial, Universidad de Barcelona.
Insights
Chronic myeloproliferative disorders (CMPD) are a significant cause of Budd-Chiari syndrome. This study found CMPD in 6 of 14 Budd-Chiari patients, often presenting with hematologic abnormalities.
Area of Science:
- Hematology
- Hepatology
- Oncology
Context:
- Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction.
- Chronic myeloproliferative disorders (CMPD) are increasingly recognized as a primary cause of BCS, particularly in Western countries.
- Occult CMPD may underlie idiopathic hepatic vein thrombosis.
Purpose:
- To determine the frequency of CMPD in BCS patients.
- To analyze the clinical and hematologic features of BCS patients with CMPD.
- To investigate the association between CMPD and hepatic vein thrombosis.
Summary:
- A review of 14 BCS cases identified CMPD in 6 patients (43%).
- CMPD was suspected due to hematologic abnormalities like high hematocrit, leukocytosis, and thrombocytosis.
- The most common CMPD identified were polycythemia vera (5 patients) and idiopathic myelofibrosis (1 patient).
Impact:
- This study highlights the importance of screening for CMPD in BCS patients.
- Early detection and management of CMPD can potentially improve outcomes for BCS patients.
- Understanding this association aids in the diagnosis and treatment strategies for both conditions.
Abstract:
The chronic myeloproliferative disorders (CMPD) are considered the main etiology of Budd-Chiari syndrome in Western countries. Moreover, an occult CMPD has been recently identified in most patients with idiopathic hepatic vein thrombosis. In order to determine the frequency of the association between the above entities and to analyze the clinical and hematologic features of such patients, fourteen cases of Budd-Chiari syndrome diagnosed at a single institution over a five year period were reviewed. In 6 patients a CMPD was identified, with this being the first cause of the syndrome. Median age of the later six patients was 32 years (range: 14-54), and 4 were females. In all cases the CMPD was suspected due to the presence of hematological abnormalities, including a high hematocrit (5 cases), leucocytosis (4 cases) and thrombocytosis (3 cases). Five patients had polycythemia vera (PV) and one idiopathic myelofibrosis. In an additional Budd-Chiari patient with polycythemia, PV was ruled out on the basis of high serum erythropoietin and the absence of endogenous growth of erythroid colonies in the hematopoietic progenitor culture. The CMPD treatment included phlebotomies and hydroxiurea, whereas the Budd-Chiari syndrome was treated in most patients with transjugular intrahepatic portosystemic stent-shunt. One patient died from a gastrointestinal hemorrhage at 48 months from Budd-Chiari diagnosis, and the remaining five are alive after a median follow-up of 28 months.