scholarly journals Life-threatening hemorrhage from acquired hemophilia A as a presenting manifestation of prostate cancer

2016 ◽  
Vol 6 (4) ◽  
pp. 32461 ◽  
Author(s):  
Chirag Sheth ◽  
Amandeep Gill ◽  
Sumeet Sekhon
Keyword(s):  
Prostate Cancer ◽  
Hemophilia A ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Life Threatening

scholarly journals Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Quentin Binet ◽  
Catherine Lambert ◽  
Laurine Sacré ◽  
Stéphane Eeckhoudt ◽  
Cedric Hermans
Keyword(s):  
Bullous Pemphigoid ◽  
Neutralizing Antibodies ◽  
Hemophilia A ◽  
Rare Condition ◽  
Term Treatment ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Short Term Treatment ◽  
Life Threatening ◽  
Dose Levels

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening.Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled.Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.

Improved Prognosis of Acquired Hemophilia A: A 10-Year-Experience

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4348-4348 ◽  
Author(s):  
Rudiger E. Scharf ◽  
Barbara Bomke ◽  
Holger Seidel ◽  
Roya Gheisari ◽  
Marie Antonia Scharf ◽  
...  
Keyword(s):  
Immune Tolerance ◽  
Respiratory Diseases ◽  
Hemophilia A ◽  
Conflicts Of Interest ◽  
Treatment Algorithm ◽  
Factor Vii ◽  
Laboratory Evaluation ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Life Threatening

Abstract Abstract 4348 Background: Acquired hemophilia A (AHA) is a rare but significant hemostatic disorder caused by inhibitory autoantibodies against coagulation factor VIII (FVIII:C). The annual incidence of AHA is low with about 1 to 4 cases per million individuals. However, the mortality rate due to severe hemorrhages and comorbidity is high reaching 22% in several series. In the past, only a few patients were reported in whom an association of AHA with respiratory disorders was observed. Patients, Methods, and Study Protocol: We have performed a monocenter study on 35 consecutive patients with AHA A who were referred for diagnosis and treatment to the Düsseldorf Hemophilia Comprehensive Care Center between March 2001 and June 2011. The cohort included 24 males (age: 44–86 years) and 11 females (age: 20–83 years). For laboratory evaluation, a standardized staged protocol of APTT, FVIII:C activity and concentration, mixing studies with patient and normal plasma, and quantitation of inhibitor titers (Nijmegen modification of the Bethesda assay) was used. Diagnostic work-up for any underlying disease was performed according to a standardized protocol of clinical examinations and imaging procedures (including X-ray examination of thorax, sonography of abdomen, retroperitoneum and thyreoidea and, whenever indicated, computerized tomography of thorax, abdomen, or pelvis). Therapy was performed according to a treatment algorithm consisting of (a) acute antihemorrhagic therapy (irrespective of residual FVIII:C activity and inhibitor titer), (b) immediate immunosuppression (individually tailored to the patients’ risks with regard to age and comorbidity), and, if life-threatening bleedings persisted, (c) inhibitor elimination by immunoadsorption or plasmapheresis, and (d) concomitant immunotolerance regimens. Predefined clinical endpoints were control of bleeding, eradication of the inhibitor, complete or partial remission (CR, PR), relapse, or early death (< 30 days). CR was defined as no inhibitor detectable, FVIII:C activity > 80%, and withdrawal of immunosuppressive therapy. Results: In 21 (60%) of the 35 patients with AHA, an underlying disorder was identified, including 9 patients with respiratory diseases (26%), 8 patients with autoimmune disorders (23%), 3 with malignancies, and one with postpartum state, while in 14 patients (40%) AHA remained idiopathic. Upon admission, 16 of the 35 patients presented with life-threatening hemorrhages. In 13 of these 16 patients, control of bleeding was achieved by high doses of recombinant activated factor VII (rFVIIa; 90–120 μ g/kg every 2–3 h), while 3 patients required combined FVIII bypassing agents (rFVIIa plus bolus injections of activated prothrombin complex concentrates, aPCC; 100 IU/kg every 8–12 h). In the other 19 patients, bleeding also subsided in response to rFVIIa. Concurrent immunosuppression with prednisone alone (2 mg/kg/day) was performed in 11 patients, while 24 patients received cyclophosphamide (2 mg/kg/day) sequentially in combination with prednisone. In 5 patients in whom this first-line immunosuppression failed, 4 doses of rituximab (375 mg/m2) were administered as second-line therapy. Of the 35 patients, 13 required extracorporeal inhibitor elimination procedures due to persisting life-threatening bleeds. Exchange plasmapheresis was performed in 4, daily large-volume immunoadsorption (Ig-Therasorb) for up to 4 weeks in 9 patients. In 3 of them, immune tolerance was concomitantly induced by exogenous FVIII (100 IU/kg/day). Of the 35 patients in total, 28 individuals achieved CR (80%), 3 had PR, one relapsed, and 3 died within 30 days (one of acute myocardial infarction while on antihemorrhagic treatment, one of sepsis while on immunosuppression due to active AHA, one of lung bleeding in assocociation with pre-existing pulmonary sarcoidosis). Conclusions: This monocenter study demonstrates that control of life-threatening bleeding, eradication of the inhibitor, and induction of immune tolerance to FVIII have clearly improved the clinical outcome of AHA. Our data also suggest a shift in underlying disorders associated with AHA, whereby, in comparison to published studies, a relative increase in the proportion of patients with respiratory diseases is observed. Large controlled multicenter studies are required to confirm these findings. Disclosures: No relevant conflicts of interest to declare.

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 Localised prostate cancer and hemophilia A (AHA): Case report and management of the disease

2014 ◽  
Vol 86 (3) ◽  
pp. 227 ◽  
Author(s):  
Francesco Celestino ◽  
Cristian Verri ◽  
Francesco De Carlo ◽  
Savino Mauro Di Stasi
Keyword(s):  
Prostate Cancer ◽  
Prostatic Carcinoma ◽  
Hemophilia A ◽  
Lymphoproliferative Disorders ◽  
The Other ◽  
Bleeding Diathesis ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Retropubic Radical Prostatectomy ◽  
Inflammatory Bowel

Acquired Hemophilia A (AHA) is a rare bleeding diathesis characterized by the development of autoantibodies against factor VIII (FVIII). About half of the cases are idiopathic and the other half are associated with autoimmune diseases, postpartum problems, infections, inflammatory bowel disease, drugs, lymphoproliferative disorders or solid tumors . AHA is associated with malignancies in 7-15% of cases. We report a case of AHA in a 65 year old patient with prostatic carcinoma, who underwent retropubic radical prostatectomy (RP).

scholarly journals Tinjauan Terkini Hemofilia A yang Didapat : Aspek Diagnosis dan Manajemen

2020 ◽  
Vol 3 (2) ◽  
pp. 79
Author(s):  
Ibnu Purwanto
Keyword(s):  
Partial Thromboplastin Time ◽  
Hemophilia A ◽  
Underlying Disease ◽  
Activated Partial Thromboplastin Time ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Fviii Activity ◽  
Life Threatening ◽  
Hemostatic Therapy ◽  
Hemofilia A

<p>Hemofilia A yang didapat adalah penyakit yang jarang terdiagnosis dan seringkali salah terdiagnosis namun berpotensi menyebabkan perdarahan yang mengancam nyawa. Penyakit autoimun akibat pembentukan autoantibodi (inhibitor) terhadap FVIII ini hampir setengahnya memiliki gangguan lain yang mendasari. Pemanjangan activated partial thromboplastin time, mixing test yang tidak terkoreksi, rendahnya aktivitas FVIII, dan bukti inhibitor FVIII mendukung penegakan diagnosis Hemofilia A yang didapat. Rintangan dalam manajemen pasien dimulai dari penegakan diagnosis hingga penentuan terapi, baik terapi hemostatik, imunosupresi, serta pengobatan penyakit penyerta. Pemilihan terapi serta pengendalian terhadap efek samping dari pengobatan memerlukan perhatian khusus agar tercapai hemostasis dan remisi yang bertahan lama.</p><p>Acquired Hemophilia A can potentially cause life-threatening conditions due to profuse bleeding, but this autoimmune disease is mostly underdiagnosed. Hemophilia A occurs due to the development of an antibody against FVIII, moreover up to half of these cases have underlying conditions. Prolonged activated partial thromboplastin time, uncorrected mixing test, low FVIII activity, and detection of FVIII inhibitors support the diagnosis of acquired Hemophilia A. However, several challenges lay within patients’ management strategy, such as diagnosis workup and therapeutical choices. Treatment for acquired hemophilia A encompasses hemostatic therapy, immunosuppression, and treatment of underlying disease. Moreover, therapeutical choice and side effects control require special consideration to achieve hemostasis and durable remission.</p>

scholarly journals Acquired hemophilia A in a patient with advanced prostate cancer

2015 ◽  
Vol 5 (2) ◽  
pp. 45-48 ◽  
Author(s):  
Daniel da Motta Girardi ◽  
Douglas Rafael Almeida Silva ◽  
Paula Ribeiro Villaça ◽  
Ciro Eduardo Souza ◽  
Leonardo Gomes da Fonseca ◽  
...  
Keyword(s):  
Prostate Cancer ◽  
Advanced Prostate Cancer ◽  
Hemophilia A ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia

Simultaneous Deep Venous Thrombosis and Acquired Factor VIII Inhibitor

2002 ◽  
Vol 8 (4) ◽  
pp. 375-379 ◽  
Author(s):  
Steven R. Deitcher ◽  
Teresa L. Carman ◽  
Kandice Kottke-Marchant
Keyword(s):  
Venous Thrombosis ◽  
Factor Viii ◽  
Deep Venous Thrombosis ◽  
Hemophilia A ◽  
Duplex Ultrasound ◽  
Factor Viii Inhibitor ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Prolonged Aptt ◽  
Life Threatening

Acquired hemophilia A is a life-threatening immune-mediated hemorrhagic disorder that is most often found in individuals older than 50 who present with an unexplained activated partial thromboplastin time (aPTT) prolongation and clinically significant bleeding. The prolonged aPTT associated with acquired hemophilia A reflects factor VIII activity deficiency due to neutralizing or clearing autoantibodies. Deep venous thrombosis, in contrast, is a veno-occlusive disorder associated with several distinct hypercoagulable states that can result in significant morbidity and mortality due to pulmonary embolism, thrombus extension, and the post-thrombotic syndrome. A prolonged a PYI in the setting of thrombosis may reflect the presence of a lupus anticoagulant. In the absence of accurate diagnosis and the immediate institution of specific therapy, both disorders can be fatal. Three cases of acquired factor VIII inhibitors that included a prolonged aPTT, bleeding, and duplex ultrasound evidence of deep venous thrombosis are presented. The diagnostic and therapeutic challenges posed by these cases as well as a proposed mechanism by which pathologic thrombosis can develop in a patient with a life-threatening bleeding disorder are discussed.

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