A new case of acquired haemophilia B in the context of liver autoimmune disease

Acquired haemophilia A (AHA) is a rare and severe haemorrhagic autoimmune disease caused by autoantibodies directed against factor VIII (FVIII). Treatment is based on two principles, including haemostatic control to compensate FVIII inhibition and eradication of inhibiting antibodies using immunosuppressive therapy. Rapid recognition and proper management are essential to avoid excess morbidity and mortality. Effective and safe treatments can be challenging, given that AHA patients are often elderly, with multiple comorbidities. Emicizumab, a bispecific antibody that mimics the action of FVIII, has proven effective in managing patients with congenital haemophilia, with or without inhibitors. Likewise, its mode of action suggests theoretical efficacy in AHA patients. We herein describe two AHA cases with comorbidities that were treated effectively using emicizumab combined with immunosuppressive therapy. We have also reviewed the current literature regarding the promising use of emicizumab in this indication.

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ACQUIRED HAEMOPHILIA B

The Lancet ◽

10.1016/s0140-6736(81)91596-8 ◽

1981 ◽

Vol 317 (8221) ◽

pp. 672

Author(s):

Sinas Ozsoy

Keyword(s):

Haemophilia B ◽

Acquired Haemophilia

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Acquired haemophilia A as a secondary autoimmune disease after alemtuzumab treatment in multiple sclerosis: A case report

Multiple Sclerosis and Related Disorders ◽

10.1016/j.msard.2018.11.029 ◽

2019 ◽

Vol 27 ◽

pp. 403-405 ◽

Cited By ~ 10

Author(s):

M. Pisa ◽

P. Della Valle ◽

A. Coluccia ◽

V. Martinelli ◽

G. Comi ◽

...

Keyword(s):

Multiple Sclerosis ◽

Case Report ◽

Autoimmune Disease ◽

Haemophilia A ◽

Acquired Haemophilia

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Acquired haemophilia B: a case report and literature review

Annales de biologie clinique ◽

10.1684/abc.2011.0638 ◽

2011 ◽

Vol 69 (6) ◽

pp. 685-688 ◽

Cited By ~ 2

Author(s):

Inès Jedidi ◽

Sondes Hdiji ◽

Naourez Ajmi ◽

Faiza Makni ◽

Sayda Masmoudi ◽

...

Keyword(s):

Case Report ◽

Literature Review ◽

Haemophilia B ◽

Acquired Haemophilia

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Acquired haemophilia in association with organ-specific autoimmune disease

Haemophilia ◽

10.1046/j.1365-2516.2001.00519.x ◽

2001 ◽

Vol 7 (5) ◽

pp. 523-525 ◽

Cited By ~ 32

Author(s):

D. J. Meiklejohn ◽

H. G. Watson

Keyword(s):

Autoimmune Disease ◽

Acquired Haemophilia ◽

Organ Specific

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Acquired Haemophilia A Associated with Subsequent Hepatocellular Carcinoma

Hämostaseologie ◽

10.1055/s-0038-1668570 ◽

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.

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Laryngeal Manifestations of Autoimmune Diseases

Perspectives of the ASHA Special Interest Groups ◽

10.1044/2020_persp-19-00168 ◽

2020 ◽

Vol 5 (2) ◽

pp. 439-456

Author(s):

Jenny L. Pierce

Keyword(s):

Autoimmune Disease ◽

Autoimmune Diseases ◽

Patient Outcomes ◽

Correct Diagnosis ◽

Upper Airway ◽

Clinical Implications ◽

Laryngeal Function ◽

The Past ◽

Referral Practices ◽

Timely Referral

Purpose This review article provides an overview of autoimmune diseases and their effects on voice and laryngeal function. Method A literature review was conducted in PubMed. Combinations of the following keywords were used: “autoimmune disease and upper airway,” “larynx,” “cough,” “voice,” “dysphonia,” and “dyspnea.” Precedence was given to articles published in the past 10 years due to recent advances in this area and to review articles. Ultimately, 115 articles were included for review. Results Approximately 81 autoimmune diseases exist, with 18 of those highlighted in the literature as having laryngeal involvement. The general and laryngeal manifestations of these 18 are discussed in detail, in addition to the clinical implications for a laryngeal expert. Conclusions Voice, breathing, and cough symptoms may be an indication of underlying autoimmune disease. However, these symptoms are often similar to those in the general population. Appropriate differential diagnosis and timely referral practices maximize patient outcomes. Guidelines are provided to facilitate correct diagnosis when an autoimmune disease is suspected.

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