Correspondence
Treatment of Bleeding in Hereditary Hemorrhagic Telangiectasia with Aminocaproic Acid
N Engl J Med 1994; 331:1236November 3, 1994
- Article
To the Editor:
We read the Brief Report by Saba et al. (June 23 issue)1 on the treatment of bleeding in hereditary hemorrhagic telangiectasia with aminocaproic acid with a mixture of hope and concern. Although Saba and colleagues had success with aminocaproic acid, we know of three patients with hereditary hemorrhagic telangiectasia in whom aminocaproic acid failed to decrease bleeding.
Patient 1 was a 66-year-old man whose primary manifestation of hereditary hemorrhagic telangiectasia was chronic gastrointestinal bleeding from gastric and small-bowel telangiectasias. His bleeding persisted despite trials of estrogen and progesterone, danazol, testosterone, and oral norepinephrine in addition to multiple sessions of endoscopic laser therapy. He required at least 4 units of packed red cells each month in order to maintain his hemoglobin concentration above 8 g per deciliter. Aminocaproic acid at a dose of 1 g twice a day was begun in January 1994 in conjunction with estrogen and progesterone and was continued for one month. During this period, the patient's hemoglobin concentration continued to decrease at the same rate and his transfusion requirement did not change.
Patient 2 was a 67-year-old man whose primary manifestations were frequent epistaxis and gastrointestinal bleeding refractory to endoscopic laser treatments and therapy with estrogen and progesterone. He was treated with gradually increasing doses of aminocaproic acid (final dose, 6 g per day) for three months. His bleeding was unaffected, and he required more than 25 units of packed red cells during this period.
The third patient was a 37-year-old woman with severe epistaxis who was referred to us and who had been taking aminocaproic acid (1 g twice a day) for three weeks with no diminution in the duration or severity of bleeding. She had daily headaches that she attributed to the aminocaproic acid.
Although aminocaproic acid may prove useful in some patients with bleeding caused by hereditary hemorrhagic telangiectasia, our results indicate that it is not always successful.
1 ReferencesJoshua R. Korzenik, M.D.
Mark D. Topazian, M.D.
Robert White, M.D.
Yale University School of Medicine, New Haven, CT 065201
Saba HI ,Morelli GA ,Logrono LA . Treatment of bleeding in hereditary hemorrhagic telangiectasia with aminocaproic acid. N Engl J Med 1994;330:1789-1790
Full Text | Web of Science | Medline
Author/Editor Response
The authors reply:
To the Editor: We thank Dr. Korzenik and his colleagues for sharing their experience with aminocaproic acid in patients with hereditary hemorrhagic telangiectasia. The lack of efficacy of aminocaproic acid in some patients is not surprising, because the pathogenesis of the bleeding diathesis in this disorder is probably multifactorial. Among the suggested causes are a form of disseminated intravascular coagulation,1 platelet dysfunction,2 and abnormalities of the synthesis and release of factor VIII and von Willebrand factor in the telangiectatic vascular endothelium.3 None of these, however, were found in our two patients who responded to aminocaproic acid, an inhibitor of fibrinolysis. It is likely that locally enhanced fibrinolysis had a dominant role in their bleeding diathesis. This possibility is supported by the demonstration of increased plasminogen-activator activity in the walls of the telangiectatic vessels in some patients.4,5 Since the publication of our report, we have learned of other patients in whom aminocaproic acid slowed bleeding. We hope to collect more information to determine accurately the value of this therapy.
5 ReferencesHussain I. Saba, M.D., Ph.D.
Genevieve Morelli, B.A.
Luis Logrono, M.D.
University of South Florida College of Medicine, Tampa, FL 336121
Bick RL . Hereditary hemorrhagic telangiectasia and disseminated intravascular coagulation: a new clinical syndrome. Ann N Y Acad Sci 1981;370:851-854
CrossRef | Medline2
Larsson SO . Osler's disease with impaired adhesion and aggregation of platelets. Acta Med Scand 1974;196:133-136
CrossRef | Web of Science | Medline3
Conlon CL ,Weinger RS ,Cimo PL ,Moake JL ,Olson JD . Telangiectasia and von Willebrand's disease in two families. Ann Intern Med 1978;89:921-924
Web of Science | Medline4
Kwaan HC ,Silverman S . Fibrinolytic activity in lesions of hereditary hemorrhagic telangiectasia. Arch Dermatol 1973;107:571-573
CrossRef | Web of Science | Medline5
Watanabe M ,Hanawa S ,Morishima T . Fibrinolytic activity in cutaneous lesions of hereditary hemorrhagic telangiectasia. Nippon Hifuka Gakkai Zasshi 1985;95:11-16
Medline
- Citing Articles (5)
Citing Articles
1
Carmelo Morales-Angulo, Alfonso Pérez del Molino, Roberto Zarrabeitia, África Fernández, Francisco Sanz-Rodríguez, Luisa María Botella. (2007) Tratamiento de las epistaxis en la telangiectasia hemorrágica hereditaria (enfermedad de Rendu-Osler-Weber) con ácido tranexámico. Acta Otorrinolaringológica Española 58:4, 129-132
CrossRef2
Ronald Concha, Rafael Amaro, Jamie S. Barkin. (2007) Obscure Gastrointestinal Bleeding. Journal of Clinical Gastroenterology 41:3, 242-251
CrossRef3
Carmelo Morales-Angulo, Alfonso Pérez del Molino, Roberto Zarrabeitia, África Fernández, Francisco Sanz-Rodríguez, Luisa María Botella. (2007) Treatment of Epistaxes in Hereditary Haemorrhagic Telangiectasia (Rendu-Osler-Weber Disease) With Tranexamic Acid. Acta Otorrinolaringologica (English Edition) 58:4, 129-132
CrossRef4
Sabbà, Carlo, Gallitelli, Mauro, Palasciano, Giuseppe, . (2001) Efficacy of Unusually High Doses of Tranexamic Acid for the Treatment of Epistaxis in Hereditary Hemorrhagic Telangiectasia. New England Journal of Medicine 345:12, 926-926
Full Text5
Joyce M Annichino-Bizzacchi, Roberta M Facchini, Márcia Z Torresan, Valder R Arruda. (1999) Hereditary Hemorrhagic Telangiectasia Response to Aminocaproic Acid Treatment. Thrombosis Research 96:1, 73-76
CrossRef
