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Acquired inhibitors to coagulation factors in patients with gastrointestinal diseases
Despina S Kyriakou1, Michael G Alexandrakis, Freda H Passam
1Department of Haematology, University Hospital of Heraklion, Crete, Greece.
Insights
Acquired coagulation inhibitors are more frequent in patients with gastrointestinal inflammatory and malignant diseases. This study identified specific factor deficiencies in these patients, with most experiencing no complications.
Area of Science:
- Hematology
- Gastroenterology
- Oncology
Background:
- Acquired coagulation inhibitors are rare but can complicate various diseases.
- Their incidence in gastrointestinal inflammatory and malignant conditions requires further investigation.
Purpose of the Study:
- To determine the frequency and specificity of acquired coagulation inhibitors in patients with gastrointestinal inflammatory and malignant diseases.
- To compare the incidence in the population of Crete with other regions.
Main Methods:
- A 10-year study of 511 patients with ulcerative colitis, Crohn's disease, gastrointestinal carcinoma, or lymphoma.
- Coagulation tests (prothrombin time, activated partial thromboplastin time) and mixing studies were performed.
- Specific coagulation factor assays identified the deficient factors.
Main Results:
- Fifteen patients developed inhibitors against factors IX, X, XII, VIII, XI, and V.
- Inhibitors were found in patients with ulcerative colitis, Crohn's disease, lymphoma, and carcinoma.
- Only one patient experienced severe bleeding; most had no complications and achieved remission after treatment.
Conclusions:
- Gastrointestinal inflammatory and malignant diseases are associated with an increased incidence of coagulation factor inhibitors.
- The population of Crete shows a higher incidence of these inhibitors compared to other areas.
Objectives:
To study the frequency and specificity of acquired coagulation inhibitors in inflammatory and malignant gastrointestinal diseases.
Methods:
In a 10-year period, 511 patients from the island of Crete in Greece were studied, 302 with ulcerative colitis, 112 with Crohn's disease, 82 with gastrointestinal carcinoma and 15 with gastrointestinal lymphoma. Prothrombin time and activated partial thromboplastin time were measured by routine methods. When prothrombin time and/or activated partial thromboplastin time were found to be prolonged, mixture experiments with 25%, 50% and 75% pooled normal human plasma were performed. If clotting times were inadequately corrected, the presence of an acquired inhibitor against a coagulation factor was suggested. Specific coagulation factor assays were then performed with deficient plasmas.
Results:
Fifteen patients acquired inhibitors to the following coagulation factors within the 10-year observation period: factor IX (four patients); factor X (three patients); factor XII (three patients); factor VIII (two patients); factor XI (two patients); and factor V (one patient). The activity of the above factors varied from < 1% to 10%. Five patients with ulcerative colitis, six with Crohn's disease, two with gastrointestinal lymphoma and two with gastrointestinal carcinoma developed an inhibitor. Only one patient with factor VIII inhibitor presented with severe bleeding and was treated with recombinant human activated factor VII, while the others had no complications. Remission was obtained in all patients after immunosuppressive therapy, chemotherapy or tumour resection.
Conclusion:
An increased incidence of coagulation factor inhibitors was found in patients with gastrointestinal inflammatory and malignant diseases compared to the healthy population. In addition, an increased incidence of these inhibitors was also found in the common population of Crete compared to that found in other areas.