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Hepatic circulatory diseases associated with chronic myeloid disorders
Vijayrama Poreddy1, Laurie D DeLeve
1Division of Gastrointestinal and Liver Diseases, University of Southern California Keck School of Medicine, 2011 Zonal Avenue, HMR 603, Los Angeles, CA 90293, USA.
Insights
This study examines liver circulatory diseases, including Budd-Chiari syndrome, portal vein thrombosis (PVT), sinusoidal obstruction syndrome (SOS), nodular regenerative hyperplasia (NRH), and peliosis hepatis. It highlights myeloproliferative disorders as a key cause of prothrombotic conditions leading to these liver issues.
Area of Science:
- Hepatology
- Vascular Biology
- Hematology
Background:
- Liver circulatory diseases encompass Budd-Chiari syndrome, portal vein thrombosis (PVT), sinusoidal obstruction syndrome (SOS), nodular regenerative hyperplasia (NRH), and peliosis hepatis.
- Myeloproliferative disorders are frequently associated with prothrombotic conditions, contributing to Budd-Chiari syndrome and PVT.
- SOS, a complication of hematopoietic stem cell transplantation, involves damage to sinusoidal endothelial cells (SECs) and subsequent blockage.
Purpose of the Study:
- To elucidate the underlying mechanisms and associations of various liver circulatory diseases.
- To identify common etiological factors, such as myeloproliferative disorders and SEC damage.
- To differentiate the causes and patient populations affected by these distinct liver conditions.
Main Methods:
- Review of existing literature on liver circulatory diseases.
- Analysis of etiological factors including myeloproliferative disorders, transplantation regimens, and patient conditions.
- Pathophysiological examination of sinusoidal lining cell (SEC) damage and circulatory blockage.
Main Results:
- Myeloproliferative disorders are a primary cause of prothrombotic states leading to Budd-Chiari syndrome and PVT.
- SOS results from SEC damage and embolism, often linked to transplantation conditioning.
- NRH is uncommonly associated with myeloproliferative disorders, potentially due to perfusion issues, while peliosis hepatis is linked to SEC damage in immunosuppressed or ill patients.
Conclusions:
- Liver circulatory diseases share common pathways involving vascular compromise and endothelial cell damage.
- Understanding the specific causes, like myeloproliferative disorders and SEC injury, is crucial for diagnosis and management.
- These conditions highlight the vulnerability of hepatic circulation to systemic disorders and iatrogenic complications.
Abstract:
These liver diseases are diseases of the hepatic circulation. Myeloproliferative disorders are among the most common prothrombotic disorders that lead to Budd-Chiari syndrome and PVT. SOS, previously known as hepatic veno-occlusive disease, is mainly seen in North America and Western Europe as a complication of the conditioning regimen for hematopoietic stem cell transplantation. SOS is caused by damage to SECs, and the initiating circulatory blockage occurs because of the embolism of sinusoidal lining cells. Myeloproliferative disorders are an uncommon cause of NRH, which is believed to be caused by uneven perfusion of the liver at the venous or sinusoidal level. Peliosis hepatis is believed to result from damage to SECs and is seen mainly in immunosuppressed patients, patients with a wasting illness, or patients with a drug toxicity.