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Hyperhomocysteinemia-associated Thrombosis in Patients With Pernicious Anemia
Thura W Htut1, Kyaw Z Thein2, Thein H Oo3
1Department of Haematology, Aberdeen Royal Infirmary, University of Aberdeen, NHS Grampian, Aberdeen, UK.
Insights
Pernicious anemia (PA) can cause hyperhomocysteinemia-associated thrombosis (HAT), often presenting at unusual sites with neuropsychiatric symptoms. Treatment with cobalamin replacement and antithrombotics showed a low rate of recurrent thrombosis.
Area of Science:
- Hematology
- Gastroenterology
- Neurology
Background:
- Cobalamin deficiency (CD) from pernicious anemia (PA) causes hyperhomocysteinemia, a known risk factor for thrombosis.
- The clinical characteristics and outcomes of hyperhomocysteinemia-associated thrombosis (HAT) linked to PA remain poorly understood.
Purpose of the Study:
- To elucidate the clinical features and outcomes of patients experiencing hyperhomocysteinemia-associated thrombosis (HAT) secondary to pernicious anemia (PA).
Main Methods:
- A comprehensive literature search was conducted across PubMed, Scopus, and Web of Science databases up to January 2024.
- Keywords included "pernicious anemia AND thrombosis" and related terms to identify relevant published studies.
- The review focused on extracting clinical data to characterize PA-related HAT.
Main Results:
- The study analyzed 20 patients with a median age of 54.5 years; 35% were elderly.
- Significant findings included thromboses at multiple or unusual sites (35% and 20%, respectively), and co-existing neuropsychiatric symptoms (45%).
- Eighty percent tested positive for anti-intrinsic factor (anti-IF) antibodies, and no recurrent thromboembolism was observed after treatment.
Conclusions:
- Pernicious anemia-related hyperhomocysteinemia-associated thrombosis is a rare condition characterized by a high incidence of unusual/multiple thromboses and associated neuropsychiatric symptoms.
- A high rate of anti-intrinsic factor antibody positivity was noted.
- Cobalamin replacement and antithrombotic therapy appear effective in preventing recurrent thromboembolic events.
Introduction:
Cobalamin deficiency (CD) due to pernicious anemia (PA) leads to hyperhomocystinemia, a risk factor for thrombosis. However, the clinical presentations and outcomes of hyperhomocystinemia-associated thrombosis (HAT) are not fully understood.
Methods:
We undertook a literature search using PUBMED, SCOPUS and WEB OF SCIENCE databases for the terms "pernicious anemia AND thrombosis", "pernicious anemia AND embolism", "pernicious anemia AND thromboembolism", "autoimmune gastritis AND thrombosis", "autoimmune gastritis AND embolism", "autoimmune gastritis AND thromboembolism" through January 2024 and reviewed the published literature. Our aim was to illustrate the clinical features of PA-related HAT.
Results:
Of 20 patients, the median age was 54.5 years (60 % males). 35 % of patients were elderly. The median serum homocysteine level was 67.75 umol/L. 35 % of patients developed thromboses at multiple locations while 20 % had thromboses at unusual sites. 45 % of patients had co-existing neuropsychiatric symptoms. 80 % of patients were positive for anti-IF (anti-intrinsic factor) antibodies. All patients received cobalamin replacement and the median duration of antithrombotic therapy was 6.5 months for reported cases. None developed recurrent thromboembolism.
Conclusion:
This rare condition is associated with high incidence of thromboses at unusual sites, multiple-site thromboses, co-existing neuropsychiatric symptoms, high positivity rate for anti-IF antibodies and low recurrent thrombosis rate.
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