Pemphigus foliaceus and acquired haemophilia: a rare but important association with life-threatening consequences

2018 ◽  
Vol 43 (7) ◽  
pp. 825-828 ◽  
Author(s):  
A. G. Wernham ◽  
C. Peng ◽  
B. Bailiff ◽  
A. Ilchyshyn
Keyword(s):  
Pemphigus Foliaceus ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Life Threatening

Acquired haemophilia A: successful treatment of a patient using upfront immunosuppressive therapy and haemostatic agents

2021 ◽  
Vol 14 (6) ◽  
pp. e242876
Author(s):  
Su Yun Chung ◽  
Janice Gloria Shen ◽  
Kristin Lynn Sticco
Keyword(s):  
Immunosuppressive Therapy ◽  
Successful Treatment ◽  
Treatment Protocol ◽  
Autoimmune Disorder ◽  
Immunosuppressive Regimen ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Underlying Malignancy ◽  
Haemostatic Agents ◽  
Life Threatening

Acquired haemophilia A (AHA) is a rare and possibly fatal autoimmune disorder that is challenging to treat. Although a majority of cases are idiopathic, AHA can also be associated with an underlying malignancy, autoimmune disorder, pregnancy, infection or certain medications. The diagnosis and treatment of AHA require a specialist with both clinical and laboratory expertise. The goal of treatment is aimed at achieving haemostasis as well as eradicating factor inhibitors. We present a patient with AHA and life-threatening haemorrhage who was successfully treated with a combination of haemostatic agents and a triple-drug immunosuppressive regimen. In reviewing recent studies and published guidelines, we advocate that a newer agent, emicizumab, can potentially be incorporated into the treatment protocol for AHA given its promising performance in the realm of congenital haemophilia.

scholarly journals Acquired haemophilia syndrome: Pathophysiology and therapy

2010 ◽  
Vol 138 (suppl. 1) ◽  
pp. 64-68 ◽  
Author(s):  
Ivo Elezovic
Keyword(s):  
Soft Tissues ◽  
Coagulation Factor ◽  
Prior History ◽  
Haemophilia A ◽  
Acute Bleeding ◽  
Fviii Inhibitor ◽  
Acquired Haemophilia ◽  
Life Threatening ◽  
Bleeding Episodes ◽  
Inhibitor Titer

Acquired inhibitors against coagulation factor VIII (FVIII), also termed acquired haemophilia A, neutralize its procoagulant function and result in severe or often life-threatening bleeding. The antibodies arise in individuals with no prior history of clinical bleeding. Acquired haemophilia occurs rarely with the incidence of approximately 1 to 4 per million/ year, with severe bleeds in up to 90% of affected patients, and high mortality between 8-22%. About 50% of diagnosed patients were previously healthy, while the remaining cases may be associated with postpartum period, autoimmune diseases, malignancy, infections, or medications. Most patients have spontaneous haemorrhages into the skin, muscles or soft tissues, and mucous membranes, or after trauma and surgery, whereas haemarthroses are uncommon. The diagnosis of acquired haemophilia A based on the prolongation of activated partial thromboplastin time which does not normalize after the addition of normal plasma, reduced FVIII, with evidence of FVIII inhibitor measured by the Bethesda assay (Nijmegen modification). The treatment of acute bleeding episodes and the long-term eradication of the autoantibodies in acquired haemophilia are the main therapeutic strategy. Two options are currently available for acute bleeding control: the use rFVIIa or FEIBA in patients with higher inhibitor titer (>5 BU), or to raise the level of FVIII by administration of DDAVP or concentrates of FVIII in patients with low level of inhibitors (<5 BU). Treatment with FEIBA (50-100 IU/ kg every 8-12 hours) has shown good haemostatic response in 76-89% of the bleeding episodes. Patients treated with rFVIIa (90 ?g/kg every 2-6 hours) have achieved good response in 95-100% as a first-line, and 75-80% as a salvage therapy. Patients with low inhibitor titer and lower response can be treated with concentrate of FVIII in the recommended dose of 40 IU/kg plus 20 IU/kg for each BU of inhibitor. The treatment of non-life-threatening haemorrhages with desmopressin (DDAVP 0.3 ?g/kg) may increase both FVIII and vWF. Sometimes inhibitors disappear spontaneously, but longterm management is necessary for eradication of inhibitors by immunosuppression (prednisone 1 mg/kg 3 weeks alone or in combination cyclophosphamide 2 mg/kg), immunomodulation, intravenous immunoglobulin (HD IgG 2g/kg 2 or 5 d), physical removal of antibodies (plasmapheresis or immunoadsorption), or various combinations. Recently, a therapy with rituximab, an anti-CD20 monoclonal antibody, has shown to be effective in acquired haemophilia.

scholarly journals Anticoagulation for Stroke Prevention after Restoration of Haemostasis with Emicizumab in Acquired Haemophilia A

2021 ◽  
Author(s):  
Kadhim Al-Banaa ◽  
Nicolas Gallastegui-Crestani ◽  
Annette von Drygalski
Keyword(s):  
Atrial Fibrillation ◽  
Factor Viii ◽  
Stroke Prevention ◽  
The Elderly ◽  
High Titre ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Inhibiting Factor ◽  
Life Threatening

Acquired haemophilia A (AHA) is a rare haemorrhagic disorder caused by the development of autoantibodies inhibiting factor VIII function. It predominantly affects the elderly, who are often burdened with a considerable number of comorbidities, and can result in life-threatening bleeding. The management of AHA consists of two aspects: inhibitor eradication with an immunomodulator and bleed control with a bypassing agent. Here we present a case of AHA with a high titre inhibitor in a patient with extensive comorbidities and atrial fibrillation in whom inhibitor eradication could not be achieved within a few weeks using corticosteroids alone. Due to coronavirus disease (COVID)-19 restrictions and complications of care, emicizumab offered an effective and convenient therapy, not only sparing the need for continued and intensified inhibitor eradication, but also allowing anticoagulation for stroke prophylaxis.

Acquired Haemophilia A Associated with Subsequent Hepatocellular Carcinoma

2018 ◽  
Vol 39 (01) ◽  
pp. 095-099
Author(s):  
Stanisława Bazan-Socha ◽  
Joanna Zdziarska ◽  
Teresa Iwaniec ◽  
Jerzy Walocha ◽  
Jacek Musiał ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Autoimmune Disease ◽  
Factor Viia ◽  
Clotting Factor ◽  
Haemophilia A ◽  
Acquired Haemophilia ◽  
Threatening Condition ◽  
Life Threatening ◽  
Diagnosis Of Cancer ◽  
Oncologic Diseases

AbstractAcquired haemophilia A (AHA) is a rare autoimmune disease caused by antibodies directed against clotting factor VIII. About half of cases are idiopathic, but AHA may also be secondary to autoimmune, dermatologic, or oncologic diseases. In approximately 10% of non-idiopathic cases, the disease occurs after or with the diagnosis of cancer as an extremely rare paraneoplastic syndrome. We describe the case of a 73-year-old male patient diagnosed with AHA and successfully treated with recombinant human activated factor VIIa and immunosuppression. Two and a half years later, however, the disease relapsed and a routine ultrasound revealed a liver tumour that was then diagnosed as hepatocellular carcinoma. We present this case to increase awareness that this life-threatening condition may develop years prior to the diagnosis of cancer.

​Acute airway compromise and coagulopathy: a rare presentation of acquired haemophilia A

2020 ◽  
Vol 13 (5) ◽  
pp. e233345
Author(s):  
William Byron Howden ◽  
Jonathan Kam ◽  
Nicholas Leith ◽  
Shashinder Singh
Keyword(s):  
Mortality Rate ◽  
Current Understanding ◽  
Haemophilia A ◽  
Airway Compromise ◽  
Rare Presentation ◽  
Airway Control ◽  
Acquired Haemophilia ◽  
Life Threatening

Acquired haemophilia A is a rare but important diagnosis, carrying a mortality rate of 22%. Life-threatening sequalae of this diagnosis includes airway compromise, which can rapidly lead to demise of the patient if left untreated. Our case examines an 80-year-old man presenting with a supraglottic haematoma resulting from acquired haemophilia A causing airway compromise and necessitating definitive airway control. A review of current understanding and management of the disease is also ddiscussed.

scholarly journals Acquired haemophilia – A diagnosis not to be missed

2008 ◽  
Vol 7 (2) ◽  
pp. 70-72
Author(s):  
S J Kitson ◽  
◽  
N F Grigoropoulos ◽  
Keyword(s):  
Autoimmune Diseases ◽  
Factor Viii ◽  
Coagulation Factor ◽  
Clinical History ◽  
Haemophilia A ◽  
Haematological Malignancies ◽  
Acquired Haemophilia ◽  
Wide Range ◽  
Life Threatening ◽  
History Of

Acquired haemophilia is a rare, life threatening bleeding disorder characterised by the development of auto-antibodies to coagulation factor VIII. Diagnosis is based upon the clinical history of mucocutaneous haemorrhages combined with a selective prolongation of the APTT. The condition is associated with a wide range of conditions, such as autoimmune diseases , solid and haematological malignancies. Treatment involves controlling the bleeding manifestations and eliminating the inhibitor antibodies. Three cases from our recent practice are used to highlight the variable severity of this condition.

Recognition of the unique bleeding pattern and laboratory findings in acquired haemophilia A facilitates prompt treatment of a life-threatening disorder

2021 ◽  
Vol 14 (8) ◽  
pp. e244238
Author(s):  
Hunter Cameron ◽  
Juliana Perez Botero
Keyword(s):  
Soft Tissue ◽  
Disease Process ◽  
Clotting Factor ◽  
Definitive Treatment ◽  
Severe Bleeding ◽  
Haemophilia A ◽  
Older Individuals ◽  
Significant Treatment ◽  
Acquired Haemophilia ◽  
Life Threatening

Acquired haemophilia A (AHA) is an uncommon but severe acquired bleeding disorder caused by the development of antibodies against clotting factor VIII, impairing secondary haemostasis. It is more common in older individuals and characteristically presents with spontaneous soft tissue bleeding that can rapidly become life-threatening. Definitive treatment requires immunosuppression to eradicate anti-FVIII antibodies, while providing haemostatic support to manage bleeding. Transfusions of fresh frozen plasma or cryoprecipitate, typically used to treat severe bleeding, are ineffective in patients with AHA. Instead, highly specialised clotting factor concentrates are required. While the appearance and extent of the soft tissue bleeding and the markedly prolonged activated partial thromboplastin time are characteristic, lack of familiarity with this disease process can lead to significant treatment delays. We report the clinical course and management of a 65-year-old woman who presented with severe anaemia of unclear aetiology with unrecognised soft tissue bleeding who was subsequently diagnosed with AHA.

Clinical features and outcome of acquired haemophilia A

2010 ◽  
Vol 30 (03) ◽  
pp. 156-161 ◽  
Author(s):  
R. Gheisari ◽  
B. Bomke ◽  
T. Hoffmann ◽  
R. E. Scharf
Keyword(s):  
Respiratory Diseases ◽  
Care Center ◽  
Treatment Algorithm ◽  
Early Death ◽  
Underlying Disease ◽  
Pulmonary Sarcoidosis ◽  
Relative Increase ◽  
Laboratory Evaluation ◽  
Haemophilia A ◽  
Acquired Haemophilia

SummaryWe have performed a monocenter study on 29 consecutive patients with acquired haemophilia A who were referred for diagnosis and treatment to the Düsseldorf Haemophilia Comprehensive Care Center between March 2001 and February 2010. Patients, methods: 18 men (age: 44–86 years) and 11 women (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 quantification of inhibitor titers (Bethesda assay) was used. Diagnostic work-up included elaborate examinations for any underlying disease. Results: In 18 (62%) of the 29 patients with acquired haemophilia A, an underlying disorder was identified, including 9 patients with respiratory diseases (31%), 7 patients with autoimmune disorders (24%), one with malignancy, and one with postpartum state, while in 11 patients (38%) acquired haemophilia A remained idiopathic. Haemotherapy of bleeding, suppression or elimination of the inhibitor, and induction of immunotolerance to endogenous FVIII:C were performed according to a treatment algorithm. Predefined clinical endpoints were control of bleeding, eradication of the inhibitor, complete or partial remission (CR, PR), relapse, or early death (≤30 days). Of the 29 patients in total, 22 individuals achieved CR (76%), three had PR, one relapsed, and three died within 30 days (one of acute myocardial infarction while on anti-haemorrhagic treatment, one of sepsis while on immunosuppression due to active acquired haemophilia A, one of lung bleeding in association with pre-existing pulmonary sarcoidosis). Conclusion: This monocenter study demonstrates that control of life-threatening bleeding, eradication of the inhibitor, and induction of tolerance to endogenous FVIII have significantly improved the clinical outcome of acquired haemophilia A. Our data also suggest a shift in underlying disorders associated with acquired haemophilia A, whereby, in comparison to published studies, a relative increase in the proportion of patients with respiratory diseases is present.

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