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ARTICLE |

Hematology FREE

Jeanne M. Lusher, MD; Sharada Sarnaik, MD
JAMA. 1996;275(23):1814-1815. doi:10.1001/jama.1996.03530470042025
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Von Willebrand disease (vWD) affects an estimated 1% of the population, yet the condition is undiagnosed in many affected persons. Originally described by the Finnish physician Erik von Willebrand in 1926, this hereditary disorder is characterized by mucous membrane bleeding (epistaxis, gum bleeding, menorrhagia), excessive bleeding from superficial lacerations, surgery, or invasive dental procedures, and easy bruisability. Although inherited as an autosomal dominant trait, vWD presents more problems for young women, who often have excessive menstrual bleeding.

Until recently, the pathogenesis of vWD was poorly understood, available diagnostic tests were controversial and difficult to interpret, and treatment options were limited. Fortunately, this has changed. We have a much clearer understanding of vWD,1-3 better diagnostic tests, and safer, easy-to-use treatment modalities. These developments—especially for treatment—make it imperative that clinicians at least think of the possibility of vWD when seeing and evaluating patients with mucous membrane—type bleeding, with or without excessive

REFERENCES

Ruggeri ZM.  Pathogenesis and classification of von Willebrand disease. Haemostasis . 1994;;24:264-275.
Ruggeri ZM, Zimmerman TS.  Variant von Willebrand disease: characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets. J Clin Invest . 1980;;65:1318-1325.
Lethagen S.  von Willebrand's disease: pathogenesis and clinical aspects. Crit Rev Oncol Hematol . 1993;;15:1-11.
Lusher JM.  Response to 1-deamino-8-D-arginine vasopressin (DDAVP) in von Willebrand disease. Haemostasis . 1994;;24:276-284.
Rick M.  Diagnosis and management of von Willebrand's syndrome. Med Clin North Am . 1994;;78:609-623.
Lusher M, Kessler CM, Laurian Y, Pierce G.  Viral contamination of blood products. Lancet . 1994;;344:405-406.
Centers for Disease Control and Prevention.  Hepatitis A among persons with hemophilia who received clotting factor concentrate: United States, September-December, 1995. MMWR Morb Mortal Wkly Rep . 1996;;45:29-32.
Eaton WA, Hofrichter J.  Hemoglobin S gelation and sickle cell disease. Blood . 1987;;70:1245-1266.
Powars DR, Chan L, Schroeder WA.  The influence of fetal hemoglobin on the clinical expression of sickle cell anemia. Ann N Y Acad Sci . 1987;;565:262-278.
Letvin NL, Linch DC, Beardsley GP, et al.  Augmentation of fetal hemoglobin production in anemic monkeys by hydroxyurea. N Engl J Med . 1984;;310: 869-874.
Charache S, Terrin ML, Moore D, et al.  Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med . 1995;;332:1317-1322.
Rogers GP.  Spectrum of fetal hemoglobin responses in sickle cell patients treated with hydroxyurea: the National Institutes of Health experience. Semin Oncol . 1992;; 19( (suppl 9) ):67-73.
ZengY-T, Huang S-Z, Ren Z-R, et al.  Hydroxyurea therapy in beta thalassemia intermedia: improvement in hematological parameters due to enhanced beta globin synthesis. Br J Haematol . 1995;;90:557-563.
Voskaridou E, Kalotychou V, Loukopoulous D.  Clinical and laboratory effects of long-term administration of hydroxyurea to patients with sickle-cell/beta thalassemia. Br J Haematol . 1995;;89:479-484.

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Ruggeri ZM.  Pathogenesis and classification of von Willebrand disease. Haemostasis . 1994;;24:264-275.
Ruggeri ZM, Zimmerman TS.  Variant von Willebrand disease: characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets. J Clin Invest . 1980;;65:1318-1325.
Lethagen S.  von Willebrand's disease: pathogenesis and clinical aspects. Crit Rev Oncol Hematol . 1993;;15:1-11.
Lusher JM.  Response to 1-deamino-8-D-arginine vasopressin (DDAVP) in von Willebrand disease. Haemostasis . 1994;;24:276-284.
Rick M.  Diagnosis and management of von Willebrand's syndrome. Med Clin North Am . 1994;;78:609-623.
Lusher M, Kessler CM, Laurian Y, Pierce G.  Viral contamination of blood products. Lancet . 1994;;344:405-406.
Centers for Disease Control and Prevention.  Hepatitis A among persons with hemophilia who received clotting factor concentrate: United States, September-December, 1995. MMWR Morb Mortal Wkly Rep . 1996;;45:29-32.
Eaton WA, Hofrichter J.  Hemoglobin S gelation and sickle cell disease. Blood . 1987;;70:1245-1266.
Powars DR, Chan L, Schroeder WA.  The influence of fetal hemoglobin on the clinical expression of sickle cell anemia. Ann N Y Acad Sci . 1987;;565:262-278.
Letvin NL, Linch DC, Beardsley GP, et al.  Augmentation of fetal hemoglobin production in anemic monkeys by hydroxyurea. N Engl J Med . 1984;;310: 869-874.
Charache S, Terrin ML, Moore D, et al.  Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med . 1995;;332:1317-1322.
Rogers GP.  Spectrum of fetal hemoglobin responses in sickle cell patients treated with hydroxyurea: the National Institutes of Health experience. Semin Oncol . 1992;; 19( (suppl 9) ):67-73.
ZengY-T, Huang S-Z, Ren Z-R, et al.  Hydroxyurea therapy in beta thalassemia intermedia: improvement in hematological parameters due to enhanced beta globin synthesis. Br J Haematol . 1995;;90:557-563.
Voskaridou E, Kalotychou V, Loukopoulous D.  Clinical and laboratory effects of long-term administration of hydroxyurea to patients with sickle-cell/beta thalassemia. Br J Haematol . 1995;;89:479-484.
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