Acquired hemophilia A: review and case report

2018 ◽  
Author(s):  
Н.Т. . Ватутин ◽  
Е.В. . . Склянная ◽  
М.А. Эль-Хатиб
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Successful Treatment ◽  
Hemophilia A ◽  
Coagulation Factors ◽  
Clotting Factor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Blood Disease

Резюме. Классическая гемофилия — наследственное заболевание крови, причиной развития которого является дефицит факторов свертывающей системы. Приобретенная же гемофилия относится к редким патологиям; в ее основе лежит продукция аутоантител, которые приводят к инактивации эндогенного фактора VIII. В статье представлен обзор литературы по этиологии, эпидемиологии, патогенезу, клинике, диагностике и лечению приобретенной гемофилии А. Приведено собственное клиническое наблюдение успешного лечения пациентки с приобретенной послеродовой гемофилией. Summary. Classical hemophilia is a hereditary blood disease caused by defi ciency of coagulation factors. Hemophilia A is a rare pathology caused by inactivation of plasma clotting factor VIII with autoantibodies. The review summarizes what is currently known about the epidemiology, pathogenesis, clinical peculiarities, diagnostics, treatment and prognosis of acquired hemophilia A. Case report of successful treatment of patient with acquired postpartum hemophilia is presented.

scholarly journals Clinical case of an acquired hemophilia

The Clinician ◽  
2020 ◽  
Vol 13 (3-4) ◽  
pp. 74-77
Author(s):  
P. N. Barlamov ◽  
E. R. Vasilyeva ◽  
M. E. Golubeva ◽  
V. G. Zhelobov ◽  
A. A. Shutylev ◽  
...  
Keyword(s):  
Factor Viii ◽  
Blood Clotting ◽  
Clinical Case ◽  
Hemophilia A ◽  
Clotting Factor ◽  
Clotting Factors ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Left Femur ◽  
Submandibular Region

The aim of the work is to describe the clinical case of formation, diagnosis and treatment of the acquired form of blood clotting factor VIII deficiency – of acquired hemophilia A.Material and methods. Patient R., 71 years, from April 2018, was found an acute hemorrhagic syndrome in the hematomic type of large hematomas manifested submandibular region, neck, chest, right breast, pubic and inguinal regions on the right, the anterior-medial surface of the left femur, anterior surface of left tibia. Standard laboratory tests, computed tomography of soft tissues of the neck, lungs, abdomen; coagulogram; blood clotting factors; inhibitor of factor VIII were evaluated in dynamics during the patient’s stay in the hospital; platelet aggregation function.Results. Typical gematomny type of bleeding, prolongation of coagulation indicators, the presence of the inhibitor factor VIII (7,0 BAA), the decrease in factor VIII (2 %) allowed diagnosis of acquired hemophilia A. Anti-inhibitory coagulant complex, fresh frozen plasma was successfully used for treatment. The patient is under observation in the regional Hematology center. The hematomas were not renewed.Conclusion. Our clinical observation demonstrates the features of the course, the algorithm of diagnosis and management of patients with of acquired hemophilia A.

Acquired Hemophilia A: A Case Report

2019 ◽  
Vol 33 (4) ◽  
pp. 562-566 ◽  
Author(s):  
Mark Shen ◽  
Shan Wang ◽  
Julia Sessa ◽  
Adel Hanna ◽  
Alexander Axelrad ◽  
...  
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Hemophilia A ◽  
Factor Viia ◽  
Spontaneous Mutation ◽  
Case Series ◽  
Rare Disorder ◽  
Bleeding Complications ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia

Hemophilia A, also known as factor VIII deficiency, is a rare disorder caused by an insufficient level of factor VIII, an essential clotting protein. Hemophilia A can be inherited or acquired. Inherited hemophilia A is caused by a mutation to the factor VIII gene on the X chromosome, which is commonly passed down from parents to children. However, in about one-third of cases, the cause is a spontaneous mutation in that gene. Acquired hemophilia A is due to an autoantibody to factor VIII, which is termed an inhibitor. This rare disorder can cause life-threatening bleeding complications. Management relies on a rapid and accurate diagnosis, control of bleeding episodes, and eradication of the inhibitor by immunosuppression therapy. Most treatment strategies are centered around anecdotal reports or small case series. This case report summarizes the successful treatment of a patient with acquired hemophilia A and major bleeding following a surgical procedure, with the use of desmopressin, recombinant factor VIIa, repeated doses of recombinant factor VIII, rituximab, and prednisone.

scholarly journals Diagnosis dan Tata Laksana Acquired Hemophilia A (AHA) dengan Pemfigoid Bulosa

2017 ◽  
Vol 3 (4) ◽  
pp. 218
Author(s):  
Rasco Sandy Sihombing ◽  
Henry Ratno Diono Silalahi ◽  
Hamzah Shatri ◽  
Lugyanti Sukrisman ◽  
Ikhwan Rinaldi ◽  
...  
Keyword(s):  
Case Report ◽  
Factor Viii ◽  
Bullous Pemphigoid ◽  
Skin Disorder ◽  
Hemophilia A ◽  
Coagulation Factor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Faktor Viii ◽  
Autoantibody Formation

Acquired hemophilia A merupakan kondisi dimana faktor koagulasi VIII menjadi tidak aktif akibat pembentukan autoantibodi. Kondisi ini dikaitkan dengan kehamilan, keganasan, dan penyakit auitoimun dengan kelainan kulit. Pada kasus ini, seorang wanita berusia 66 tahun, datang dengan keluhan perdarahan paska tindakan yang disertai dengan lesi kulit. Pasien didiagnosis dengan acquired hemophilia A dengan ditemukannya inhibitor faktor VIII terkait dengan pemfigoid bulosa.Kata Kunci: Acquired hemophilia A, diagnosis, pemfigoid bulosa, tata laksana  Diagnosis and Treatment of Acquired Hemophilia A (AHA) with Bullous PemphigoidAcquired hemophilia A is a condition in which coagulation factor VIII become inactive due to autoantibody formation. This condition is related to pregnancy, malignancy, and autoimmune disease with skin disorder. In this case report, a 66 years woman with a post procedural bleeding with skin disorder. Later on, patient diagnosed with acquired hemophilia A with a factor VIII inhibitors related to bullous pemphigoid. Keywords : Acquired hemophilia A, bullous pemphigoid, diagnosis, treatment

scholarly journals Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report

2019 ◽  
Vol 77 (2) ◽  
pp. 179-183 ◽  
Author(s):  
Élise Sourdeau ◽  
Sylvain Clauser ◽  
Romain Prud’Homme ◽  
Valérie Bardet ◽  
Leyla Calmette
Keyword(s):  
Multiple Myeloma ◽  
Case Report ◽  
Bullous Pemphigoid ◽  
Hemophilia A ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia

Acquired hemophilia A in chronic lymphocytic leukemia: A case report

2019 ◽  
Vol 58 (4) ◽  
pp. 447-448
Author(s):  
Volkan Karakuş ◽  
Egemen Kaya ◽  
Yelda Dere ◽  
Erdal Kurtoğlu
Keyword(s):  
Case Report ◽  
Chronic Lymphocytic Leukemia ◽  
Hemophilia A ◽  
Lymphocytic Leukemia ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia

scholarly journals Perioperative Management of Acquired Hemophilia A: A Case Report and Review of Literature

2013 ◽  
Vol 3 (3) ◽  
Author(s):  
Yashar Ilkhchoui ◽  
Eugene Koshkin ◽  
Jimmy J Windsor ◽  
Timothy R Petersen ◽  
Matthew Charles ◽  
...  
Keyword(s):  
Case Report ◽  
Perioperative Management ◽  
Hemophilia A ◽  
Review Of Literature ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia
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