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This study focuses on patients who have a condition called extrahepatic portal vein obstruction (EHPVO), where a blood clot blocks the portal vein outside the liver. This blockage can cause problems like an enlarged spleen, bleeding from swollen veins in the digestive system, and low blood cell counts. Many of these patients may have hidden blood disorders that increase the risk of clotting, such as myeloproliferative neoplasms (MPNs), antiphospholipid syndrome (APS), or paroxysmal nocturnal hemoglobinuria (PNH). This study will collect and analyze blood test results-such as complete blood count (CBC), liver function tests (LFTs), and clotting tests-from patients with EHPVO. The aim is to find patterns that may suggest an underlying blood disorder, even if the patient doesn't show obvious symptoms.By understanding these patterns early, doctors may be able to diagnose and treat the root causes of clotting in these patients more accurately, helping prevent complications and improve outcomes.
Extrahepatic portal vein obstruction (EHPVO) is a non-cirrhotic cause of portal hypertension that results from a thrombotic blockade in the portal vein outside the liver. The preserved hepatic parenchyma distinguishes EHPVO from intrahepatic causes of portal hypertension. However, complications like variceal bleeding, hypersplenism, and splenomegaly remain common. Hematological abnormalities frequently observed in EHPVO include thrombocytopenia, anemia, and leukopenia, often attributed to hypersplenism. Yet, in a subset of patients, especially those with concurrent myeloproliferative neoplasms (MPNs), these cytopenias may be masked due to increased production of blood elements. Coagulation profiles may also show subtle abnormalities or evidence of underlying thrombophilia.A growing body of literature suggests a strong association between EHPVO and underlying prothrombotic conditions such as MPNs (often linked to JAK2V617F mutation), antiphospholipid syndrome (APS), and paroxysmal nocturnal hemoglobinuria (PNH).
Early identification of these disorders may significantly impact clinical management, prognosis, and long-term surveillance. The current study is a cross-sectional observational analysis aiming to investigate the hematological spectrum in patients with EHPVO at Assiut University Hospital. Data will be collected retrospectively from medical records, including CBC with differential, liver function tests, coagulation parameters, and specific thrombophilia workups when available. The primary objective is to correlate these laboratory findings with underlying hematological disorders. Secondary aims include evaluating the association between these abnormalities and clinical outcomes such as variceal bleeding, transfusion needs, and prognosis. The study may offer valuable insights into risk stratification and highlight the importance of thrombophilia screening in this patient population.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| EHPVO Patients | Patients diagnosed with extrahepatic portal vein obstruction (EHPVO) based on imaging (Doppler ultrasound, CT, or MRI) and with preserved liver function. This group will be assessed for hematological abnormalities, including cytopenias and coagulation disturbances, using retrospective laboratory data. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| No intervention (Observational Study) | Other | This is a retrospective observational study with no assigned intervention. Data will be collected from patient medical records only |
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| Measure | Description | Time Frame |
|---|---|---|
| Frequency of hematological disorders in EHPVO patients | The proportion of EHPVO Patients showing hematological disorders including cytopenias ( thrombocytopenia , anemia , leukopenia ) and coagulation disturbances based on retrospective laboratory data | Over a retrospective period from 2020 to 2024 |
| Frequency of hematological disorders in EHPVO patients | Over a retrospective period from 2020 to 2024 |
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Inclusion Criteria:
Exclusion Criteria:
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Adult patients diagnosed with EHPVO between 2020 and 2024 in a tertiary care hospital in Cairo. Data will be collected retrospectively from medical records
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Rehab N Dr Rehab Nady Ali | Contact | +201099703970 | reeehabnady@gmail.com |
| Name | Affiliation | Role |
|---|---|---|
| Mohamed Q Professor of internal medicine | Assiut University | Principal Investigator |
| Maha M Dr Maha Mohamed Abdelaziz, Dr at Internal medicine | Assiut University | Principal Investigator |
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| Label | URL |
|---|---|
| Related Info | View source |
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De-identified individual participant data, including complete blood count (CBC), liver function tests (LFTs), and coagulation profile results, will be shared. No personally identifiable information will be included.
IPD and supporting documents will be available starting 6 months after publication of the primary results and will remain available for up to 5 years after publication.
Access to the de-identified individual participant data (IPD), including CBC, LFTs, and coagulation profiles, will be granted to qualified researchers for academic and non-commercial purposes.
Requests should be submitted to the Principal Investigator and will be reviewed on a case-by-case basis. A data use agreement may be required before access is granted.
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| ID | Term |
|---|---|
| D019851 | Thrombophilia |
| D009196 | Myeloproliferative Disorders |
| D016736 | Antiphospholipid Syndrome |
| D006457 | Hemoglobinuria, Paroxysmal |
| D013921 | Thrombocytopenia |
| D000740 | Anemia |
| D007970 | Leukopenia |
| D006971 | Hypersplenism |
| D006402 | Hematologic Diseases |
| ID | Term |
|---|---|
| D006425 | Hemic and Lymphatic Diseases |
| D001855 | Bone Marrow Diseases |
| D001327 | Autoimmune Diseases |
| D007154 | Immune System Diseases |
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| ID | Term |
|---|---|
| D019370 | Observation |
| ID | Term |
|---|---|
| D008722 | Methods |
| D008919 | Investigative Techniques |
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| D000743 | Anemia, Hemolytic |
| D009190 | Myelodysplastic Syndromes |
| D001791 | Blood Platelet Disorders |
| D000095542 | Cytopenia |
| D007960 | Leukocyte Disorders |
| D013158 | Splenic Diseases |
| D008206 | Lymphatic Diseases |