scholarly journals Postpartum Acquired Hemophilia: A Rare Cause of Postpartum Hemorrhage

2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
Srikanth Seethala ◽  
Sumit Gaur ◽  
Elizabeth Enderton ◽  
Javier Corral
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Factor Vii ◽  
Normal Vaginal Delivery ◽  
Factor Viii Inhibitor ◽  
Activity Levels ◽  
Factor Viii Activity ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Viii Inhibitor

A 36-year-old female started having postpartum vaginal bleeding after normal vaginal delivery. She underwent hysterectomy for persistent bleeding and was referred to our institution. An elevation of PTT and normal PT made us suspect postpartum acquired hemophilia (PAH), and it was confirmed by low factor VIII activity levels and an elevated factor VIII inhibitor. Hemostasis was achieved with recombinant factor VII concentrates and desmopressin, and factor eradication was achieved with cytoxan, methylprednisolone, and plasmapheresis.

scholarly journals Acquired hemophilia A: A rare cause of gross hematuria

2015 ◽  
Vol 9 (11-12) ◽  
pp. 905
Author(s):  
Gregory W Hosier ◽  
Ross J Mason ◽  
K Sue Robinson ◽  
Gregory G Bailly
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Rare Condition ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Viii Inhibitor

Acquired hemophilia A is a rare condition caused by spontaneous development of factor VIII inhibitor. This condition most commonly presents with multiple hemorrhagic symptoms and isolated hematuria is exceedingly rare. Early diagnosis is important, as this condition carries a high mortality rate (13‒22%). We present a case of an 82-year-old man with isolated hematuria caused by a factor VIII inhibitor who was successfully treated with recombinant activated factor VII concentrate, as well as prednisone and cyclophosphamide.

scholarly journals Idiopathic Acquired Hemophilia A with Undetectable Factor VIII Inhibitor

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Nicholas B. Abt ◽  
Michael B. Streiff ◽  
Christian B. Gocke ◽  
Thomas S. Kickler ◽  
Sophie M. Lanzkron
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Term Therapy ◽  
Factor Viii Inhibitor ◽  
Factor Viii Activity ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Fviii Activity ◽  
Viii Inhibitor ◽  
Acquired Factor Viii Inhibitor

Objective. We present the case of a 73-year-old female, with no family or personal history of a bleeding disorder, who had a classic presentation for acquired hemophilia A. Factor VIII activity was low but detectable and a factor VIII inhibitor was undetectable.Methods. The patient’s plasma was comprehensively studied to determine the cause of the acquired coagulopathy. Using the Nijmegen modification of the Bethesda assay, no factor VIII autoantibody was measureable despite varying the incubation time from 1 to 3 hours.Results. The aPTT was prolonged at 46.8 seconds, which did not correct in the 4 : 1 mix but did with 1 : 1 mix. Using a one stage factor VIII activity assay, the FVIII activity was 16% and chromogenic FVIII activity was also 16%. The patient was treated with recombinant FVII and transfusion, significantly reducing bleeding. Long-term therapy was initiated with cyclophosphamide and prednisone with normalization of FVIII activity.Conclusions. Physicians can be presented with the challenging clinical picture of an acquired factor VIII inhibitor without a detectable inhibitor by the Bethesda assay. Standard therapy for an acquired hemophilia A should be considered.

Acquired Hemophilia A: A Single Institution’s Experience.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4103-4103
Author(s):  
Devinderpal Randhawa ◽  
Ibrahim Sidhom ◽  
Gunwant Guron ◽  
Trevor Layne
Keyword(s):  
Factor Viii ◽  
Immunosuppressive Therapy ◽  
Hemophilia A ◽  
Treatment Modalities ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Mortality And Morbidity ◽  
Life Threatening ◽  
Viii Inhibitor

Abstract Background: Acquired Hemophilia A (AH) due to factor VIII inhibitor is a rare life threatening disorder. If not diagnosed and treated urgently, significant mortality and morbidity results. AH can occur in setting of old age, autoimmune diseases, pregnancy, medication, malignancy, and lymphoproliferatve disorders. In majority of cases it is idiopathic. Objective: Review the treatment modalities and outcome of AH patients at our institution. Methods: A retrospective review of the data pertaining to patients who were diagnosed with AH at our institution between 1993–2004. Results: There were 5 patients diagnosed with AH, 3 female and 2 male. The median age was 67 years (range 30–84 years) the setting for development of AH in these patients was as follows: 1- postpartum, 1-HIV, 3 idiopathic. All patients presented with varying degree of spontaneous hemorrhage. The median Factor VIII inhibitor level was 16 Bethesda Unit (BU)(range 7. 2–31). Acute control of hemorrhage was achieved in all patients using either FEIBA (Factor eight inhibitor bypass activity) alone (1 patient), FEIBA and Novo seven (VIIa)(4 patients). Chronic immunosuppressive therapy was given as follows: Steroid alone (2 patients), Steroid and IVIG (1 patients), Steroid and Cyclophospamide (1 patient) and Steroid, Cyclophospamide and Rituximab (1 patient). Complete remission (CR) was obtained in 4 patients and with the final patient still receiving treatment. In one patient, the dose of Cyclophospamide was decreased due to Leucopenia. The median time to elimination of inhibitors was 5 month (range 1–10 month). There have been no mortalities. Conclusions: AH is a life threatening condition if not promptly diagnosed and treated, mortality remains significantly high. Treatment with factors replacement and immunosuppressive therapy was effective in all our patients

scholarly journals A Case of a Patient Who Is Diagnosed with Mild Acquired Hemophilia A after Tooth Extraction Died of Acute Subdural Hematoma due to Head Injury

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Tomohisa Kitamura ◽  
Tsuyoshi Sato ◽  
Eiji Ikami ◽  
Yosuke Fukushima ◽  
Tetsuya Yoda
Keyword(s):  
Factor Viii ◽  
Subdural Hematoma ◽  
Tooth Extraction ◽  
Hemophilia A ◽  
Coagulation Factor ◽  
Acute Subdural Hematoma ◽  
Factor Viii Inhibitor ◽  
Activity Levels ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia

Background. Acquired hemophilia A (AHA) is a rare disorder which results from the presence of autoantibodies against blood coagulation factor VIII. The initial diagnosis is based on the detection of an isolated prolongation of the activated partial thromboplastin time (aPTT) with negative personal and family history of bleeding disorder. Definitive diagnosis is the identification of reduced FVIII levels with evidence of FVIII neutralizing activity. Case report. We report a case of a 93-year-old female who was diagnosed as AHA after tooth extraction at her home clinic. Prolongation of aPTT and a reduction in factor VIII activity levels were observed with the presence of factor VIII inhibitor. AHA condition is mild. However, acute subdural hematoma of this patient occurred due to an unexpected accident in our hospital. Hematoma was gradually increased and the patient died 13 days after admission. Discussion. Although AHA is mild, intracranial bleeding is a life-threatening condition. We also should pay attention to the presence of AHA patients when we extract teeth.

A Case of Acquired Hemophilia Post Treatment with Recombinant Human Activated Protein C (rh APC).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4021-4021
Author(s):  
Yelena Patsiornik ◽  
Archana Maini
Keyword(s):  
Factor Viii ◽  
Factor Vii ◽  
Factor Viii Inhibitor ◽  
Factor Xii ◽  
Operating Table ◽  
Apache Score ◽  
Acquired Hemophilia ◽  
Drotrecogin Alfa Activated ◽  
Bleeding Episodes ◽  
Viii Inhibitor

Abstract A 87-year old male without a diagnosis of hemophilia presented to the ER with a large shoulder hematoma, developing after a minor fall. Activated partial thromboplastin time (aPTT) was 79.4 sec, not correcting on mixing studies. Factor assays showed a factor VIII of 11.9 %, factor IX of 74.7 %, factor XI of 82.2 % and factor XII of 65.5 %. Anticardiolipin IgG, IgM antibodies and lupus anticoagulant were negative. Factor VIII inhibitor was found and initially measured at 14 Bethesda Unit (BU). A diagnosis of Acquired Hemophilia was made and the patient was treated with recombinant factor VII (rVIIa) and factor VII concentrates, FFP, PRBC transfusions and steroids. The patient had 4 bleeding episodes during hospitalization. First episode was not treated because of the lack of correct diagnosis. However, rVIIa was administered for all bleeding episodes and prior to surgical procedures like tracheostomy. Interestingly, despite immunosupression with steroids, the inhibitor titer did not decrease, rather it displayed a greater variation in the BU values ranging from 14 to 67. The patient bled unpredictably at different titers of inhibitor, without any concordance between the bleeding or the aPTT or the BU value. Finally,he succumbed to uncontrolled bleeding from the tracheostomy site and expired on operating table on day 30 of his fateful admission. This is a unique case of Acquired Hemophilia as 8 months ago this patient had received rhAPC, also known as Drotrecogin Alfa Activated (DAA) for severe sepsis with APACHE score of 25. DAA has a newly established therapeutic role in the inhibitor of factors Va and VIIIa, limiting the thrombotic effect and thus the mortality of sepsis. However, cost-effectiveness and safety of DAA remain somewhat controversial. Despite its recent entry into the medical armamentarium of our fight against sepsis, there exist several references in the published literature about the need of a confirmatory prospective trial about its role even in patients with a high APACHE score. Although neutralizing antibody against APC have not been detected in any patient and there are no published reports about DAA induced antibodies to factor VIII, it remains a viable hypothesis nonetheless. Unknown mechanisms such as exposure of new epitopes of factor VIII degraded by rhAPC could precipitate factor VIII antibodies. Further, the unpredictable titer of factor VIII inhibitor may be secondary to the unique mode of antibody formation in this instance. Lack of Correlation between Factor VIII inhibitor Titers and Clinical Sequelae Dates PTT PT Hb/Ht F VIII inhibitor(BU) Bleeding Episodes 2/01/05 151.4 32 7.1/22.4 On admission: soft tissue hematoma 2/02/05 79.4 14.8 6.2/18.2 14 2/04/05 84.6 16.2 9.3/27.7 29.8 2/05/05 75.2 10 11/32.9 2/06/05 74.8 16.1 9.8/29.7 2/07/05 90.4 17.5 9.2/26.5 67 2/08/05 162.0 17.0 8.8/26.7 GI bleeding, hematuria 2/10/05 97.4 14.1 7.5/22 2/13/05 119 14.6 7.4/22.7 3/01/05 113.8 14.1 9.4/29.8 35 3/02/05 9.2/28.8 Hematuria, rectal bleeding 3/03/05 86.2 12 7/21.2 Bleeding from tracheostomy site

scholarly journals Acquired hemophilia A developing cerebral infarction 36 days after the frequent administration of bypass hemostatic agents

2018 ◽  
Vol 10 (2) ◽  
Author(s):  
Makoto Saito ◽  
Hajime Senjo ◽  
Minoru Kanaya ◽  
Koh Izumiyama ◽  
Akio Mori ◽  
...  
Keyword(s):  
Cerebral Infarction ◽  
Factor Viii ◽  
Hemophilia A ◽  
Activity Level ◽  
High Dose ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Hemostatic Agents ◽  
Frequent Administration

A 74-years-old male who was a smoker and received treatment for hypertension, dyslipidemia, peripheral arterial disease and idiopathic interstitial pneumonia complained of subcutaneous hemorrhage of the right lower thigh. Marked anemia (hemoglobin 5.5 g/dL) and prolonged activated partial thromboplastin time (≥130 seconds) were noted. The factor VIII activity level was reduced to 1.2 %, and the factor VIII inhibitor titer was 285.3 BU/mL, a diagnosis of acquired hemophilia A (AHA) was made. Then, hematomas of 5 intra-muscles were recurred. Hemostasis became difficult despite frequent and high-dose administration of recombinant human coagulation factor VIIa (total: 18 days, 305 mg). Hemostasis was achieved by switching to activated prothrombin complex concentrate (for 3 days, 18,000 units), however, cerebral infarction occurred after 36 days. After the frequent administration of bypass hemostatic agents on elderly AHA patients with several risk factors for ischemic stroke, the risk of subsequent thrombotic events may persist for 1 month.

How we treat a hemophilia A patient with a factor VIII inhibitor

Blood ◽  
2009 ◽  
Vol 113 (1) ◽  
pp. 11-17 ◽  
Author(s):  
Christine L. Kempton ◽  
Gilbert C. White
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Clotting Factor ◽  
Patient Specific ◽  
Factor Viii Inhibitor ◽  
Factor Viii Activity ◽  
Related Factors ◽  
Acute Bleeding ◽  
Viii Inhibitor

Abstract The most significant complication of treatment in patients with hemophilia A is the development of alloantibodies that inhibit factor VIII activity. In the presence of inhibitory antibodies, replacement of the missing clotting factor by infusion of factor VIII becomes less effective. Once replacement therapy is ineffective, acute management of bleeding requires agents that bypass factor VIII activity. Long-term management consists of eradicating the inhibitor through immune tolerance. Despite success in the treatment of acute bleeding and inhibitor eradication, there remains an inability to predict or prevent inhibitor formation. Ideally, prediction and ultimately prevention will come with an improved understanding of how patient-specific and treatment-related factors work together to influence anti–factor VIII antibody production.

scholarly journals ACQUIRED HEMOPHILIA A IN DIABETIC WOMAN, Case Report

2018 ◽  
Vol 3 (2) ◽  
pp. 107
Author(s):  
Wiwiek Probowati
Keyword(s):  
Diabetes Mellitus ◽  
Factor Viii ◽  
Hemophilia A ◽  
World Federation ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
History Of ◽  
Viii Inhibitor ◽  
Faktor Viii ◽  
Hemofilia A

Background Acquired hemophilia is a rare condition in which autoantibodies, usually IgG class are produced against factor VIII or IX. Age distribution is bimodal. Although hemophilia is a hereditary disease, approximately 20-30% of patients have no family history of clotting disorders. Case report A 55-year-old lady with major complaints of gums bleeding, bruises on the both of leg and thighs that are varying colour (multiple spontaneus haematomas. No previous history of bleeding and no history of blood clotting disorders in the family. She has diabetes mellitus for 8 years was treated metformin three times a day, and )controlled level of blood glucose. Laboratory findings showed decreased haemoglobin (5,7 mg/dl) with prolonged aPTT 129 s(28 s control) but still got the normal PPT that is 17.2 s (14.4 s control) and normal INR 1,3, factor VIII activity got decrease with result of 1,5% (control 91,1%) but factor IX activity still normal 118,7% (control 108%). The Factor VIII inhibitor was 19.52 Bethesda Unit. After exclusion of other possible pathological condition and on the basis of lab criteria we diagnosed the case as acquired hemophilia A. Discussion The diagnosis of Acqured hemophlia A (AHA) shoud be considered in patient who present with bleeding and prolonged aPPT. The pattern of bleeding in AHA differs from that in congenital hemophilia A. Bleeding tends to occur in soft tissue, muscle, retroperitoneal space, iatrogenic bleeding is also common. The diagnosis is then confirmed by a Bethesda positive assay for F VIII inhibitor titre. Diagnostic test in AHA are clotting factor measurement (isolated low FVIII level) and quantification of the inhibitor titer (presence of inhibitor). Most often the cause is idiopathic in 50% of patient or it can be associated with autoimun disorders, hepatitis and diabetes. Chronic disease or diabetes mellitus makes misrecognition tends to self antigen.1,3 Conclusion A 55-years-old lady with diabetes got symptoms gum bleeding, multiple spontaneuos hematomes and very low in factor VIII activity and presence of factor VIII inhibitor. It is concluded that this patient is diagnosed of acquired hemophilia A. Keywords: Acquired Haemophilia A, activated partial thromboplastin time (APTT), Factor VIII. Multiple haematomes in acquired haemphilia References 1. Ma AD, Carrizosa D. Acquired Factor VIII Inhibitors: Pathophysiology and Treatment. American Society of Hematology. 2006. 2. Antonela Tufano, Antonio Copola, Anna Guida, Acquired Hemophilia in Elderly, Current Gerontology and Geriatric Research volume 2010 3. Giangrande P. Acquired Hemophilia. World Federation of Hemophilia. 2012. 4. Sakurai Y, Takeda T. Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder. Journal of Immunology Research. 2014. 5. Srivastava A, et al. Guidelines for the Management of Hemophilia 2nd edition. World Federation of Hemophilia. 2012 6. Rotty LWA. Hemofilia A dan B dalam Buku Ajar Ilmu Penyakit Dalam. Interna Publishing. Jakarta. 2014. Laporan Kasus ACQUIRED HEMOPHILIA A PADA WANITA PENDERITA DIABETES Wiwiek Probowati1, Mardiah Suci Hardanti2 1 Bagian Penyakit Dalam, Rumah Sakit Bethesda, Yogyakarta 2 Bagian Haematologi Onkologi Medik, Bagian Penyakit Dalam Universitas Gadjah Mada, Rumah Sakit Umum Pusat Sardjito Yogyakarta Latar Belakang Acquired Hemofilia adalah kondisi sangat jarang di mana autoantibodi, IgG diproduksi terhadap faktor VIII atau IX. Distribusi usia adalah bimodal. Meskipun hemofilia adalah penyakit keturunan, sekitar 20-30% pasien tidak memiliki riwayat keluarga gangguan pembekuan. Laporan kasus Seorang wanita berusia 55 tahun dengan keluhan utama perdarahan gusi, memar di kedua kaki dan paha yang warnanya bervariasi (multiple spontaneus hematoma). Tidak ada riwayat perdarahan sebelumnya dan tidak ada riwayat gangguan pembekuan darah dalam keluarga. Ia menderita diabetes mellitus selama 8 tahun diterapi dengan metformin dan kadar glukosa darah terkontrol. Hasil laboratorium menunjukkan penurunan hemoglobin (5,7 mg / dl) dengan PT 129 (kontrol 28), PPT normal yaitu 17,2 s (kontrol 14,4 s) dan INR normal 1,3, aktivitas faktor VIII mengalami penurunan dengan hasil 1,5% (kontrol 91,1%) tetapi aktivitas faktor IX masih normal 118,7% (kontrol 108%). Inhibitor Faktor VIII adalah 19,52 Unit Bethesda. Berdasarkan kriteria laboratorium, diagnosis kasus ini adalah acquired hemofilia A. Diskusi Diagnosis Acqured hemophlia A (AHA) harus dipertimbangkan pada pasien yang datang dengan perdarahan dan pemanjangan PPT. Pola perdarahan pada AHA berbeda dari pada hemofilia kongenital A. Perdarahan cenderung terjadi pada jaringan lunak, otot, ruang retroperitoneal, perdarahan iatrogenik juga sering terjadi. Tes Bethesda positif untuk titer inhibitor F VIII. Tes diagnostik dalam AHA adalah dengan mengukur faktor pembekuan (tingkat FVIII rendah terisolasi) dan kuantifikasi titer inhibitor (inhibitor). Penyebab tersering pasien dikaitkan dengan gangguan autoimun, hepatitis dan diabetes. Penyakit kronis atau diabetes mellitus menimbulkan misrecognition terhadap antigen penderita.1,3 Kesimpulan Seorang wanita 55 tahun dengan diabetes mengalami gejala perdarahan gusi, hematom spontan di paha dan perut dengan aktivitas faktor VIII yang sangat rendah dan munculnya faktor VIII inhibitor. Pasien ini didiagnosis menderita Acquired hemofilia A. Kata kunci: Acquired Haemophilia A, activated partial thromboplastin time (APTT), Factor VIII. Hematom multipel pada acquired hemofilia Daftar Pustaka 1. Ma AD, Carrizosa D. Acquired Factor VIII Inhibitors: Pathophysiology and Treatment. American Society of Hematology. 2006. 2. Antonela Tufano, Antonio Copola, Anna Guida, Acquired Hemophilia in Elderly, Current Gerontology and Geriatric Research volume 2010 3. Giangrande P. Acquired Hemophilia. World Federation of Hemophilia. 2012. 4. Sakurai Y, Takeda T. Acquired Hemophilia A: A Frequently Overlooked Autoimmune Hemorrhagic Disorder. Journal of Immunology Research. 2014. 5. Srivastava A, et al. Guidelines for the Management of Hemophilia 2nd edition. World Federation of Hemophilia. 2012 6. Rotty LWA. Hemofilia A dan B dalam Buku Ajar Ilmu Penyakit Dalam. Interna Publishing. Jakarta. 2014.

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