Hematological Disorders in EHPVO Patients (EHPVO-HEM)

Rahab Nady

Status: Not yet enrolling

Conditions: Anemia; Paroxysmal Nocturnal Hemoglobinuria (PNH); Thrombocytopenia; Thrombophilia; Antiphospholipid Syndrome (APS); Leukopenia; Extrahepatic Portal Vein Obstruction (EHPVO); Myeloproliferative Neoplasms (MPN)

Treatments: Other: No intervention (Observational Study)

Study type: Observational

Funder types: Other

Identifiers

NCT07108023

EHPVO-02

Details and patient eligibility

About

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.

Full description

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.

Enrollment: 115 estimated patients

Sex: All

Ages: 18 to 75 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Age 18 years or older . Diagnosis of extra hepatic portal vein obstruction based on imaging ( Doppler, CT , MRI ) preserved liver function . Available complete medical records including CBC , LFTs and coagulation profile .

Exclusion criteria

• Patients with cirrhosis or intrahepatic portal hypertension • Incomplete or missing medical records • Patients with known hematologic malignancies or undergoing chemotherapy

Trial design

115 participants in 1 patient group

EHPVO Patients

Description:

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.

Treatment:

Other: No intervention (Observational Study)