Hemorrhagic disorder due to an isoniazid-associated acquired factor XIII inhibitor in a patient with waldenström's macroglobulinemia

Krumdieck, Richard; Shaw, Denise R.; Huang, Shu T.; Poon, Man‐Chiu; Rustagi, Pradip K.

doi:10.1016/0002-9343(91)90643-c

Cited by 9 publications

(14 citation statements)

References 36 publications

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“…Finally, it is important to note that the AH13 cases examined often showed mixed characteristics of the three types of anti‐FXIII autoantibody. This is very likely because their autoantibodies are oligoclonal rather than monoclonal , as suggested by the fact that most of our patients had anti‐FXIII autoantibodies of more than one IgG subclass (Table S2). In addition, multiple clones may share the same subclass.…”

Section: Discussionmentioning

confidence: 92%

Anti‐factor XIII A subunit (FXIII‐A) autoantibodies block FXIII‐A2B2 assembly and steal FXIII‐A from native FXIII‐A2B2

Souri

Osaki

Ichinose

2015

Journal of Thrombosis and Haemostasis

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Summary. Background: Autoimmune hemophilia-like disease (hemorrha-philia or hemorrhagic disorder) caused by anti-factor XIII antibodies (termed AH13) or 'autoimmune FXIII deficiency' is a life-threatening bleeding disorder. AH13 was thought to be rare worldwide. Objectives: Because the number of diagnosed AH13 cases has recently been increasing, at least in Japan, we conducted a nationwide survey supported by the Japanese Ministry of Health, Labor, and Welfare, and explored the pathologic mechanism(s) of AH13. Methods: We diagnosed AH13 cases during the last 11 years according to the presence of anti-FXIII autoantibodies confirmed by a dot blot assay and ELISA, and characterized 33 of these both immunologically and biochemically. Results: The AH13 cases were immunologically classified into three types, Aa, Ab, and B. Type Aa autoantibodies, observed in 27 cases, were directed against the native FXIII A subunit (FXIII-A), and blocked FXIII activation. The autoantibodies not only prevented assembly of new FXIII-A 2 B 2 heterotetramers, but also removed FXIII-A from native FXIII-A 2 B 2 heterotetramers by forming an FXIII-A-IgG complex. Type Ab autoantibodies, detected in three cases, preferentially bound to activated FXIII-A and inhibited its activity. Type Aa and Ab autoantibodies were 'neutralizing ' FXIII antibodies (or FXIII inhibitors), and thus could be screened with functional assays. Type B antibodies, detected in two cases, were non-neutralizing anti-FXIII B subunit (FXIII-B) autoantibodies that possibly accelerated the clearance of FXIII, and thus could be diagnosed exclusively with immunologic methods. Conclusion: There are three major types of anti-FXIII autoantibody, with distinct targets and mechanisms that cause AH13.

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Section: Discussionmentioning

confidence: 92%

Anti‐factor XIII A subunit (FXIII‐A) autoantibodies block FXIII‐A2B2 assembly and steal FXIII‐A from native FXIII‐A2B2

Souri

Osaki

Ichinose

2015

Journal of Thrombosis and Haemostasis

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“…Acquired FXIII deficiency is even rarer. Thirty‐four English language publications could be accessed to review and are summarized in Table . There is a female predominance of acquired FXIII deficiency in these cases.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

“…Diseases associated with acquired FXIII deficiency include malignancies and autoimmune diseases such as leukaemia [17], monoclonal gammopathy of unknown significance [25], SLE [28] and immune thrombocytopenic purpura [8,28,42] and a prolonged upper respiratory tract infection [45]. Medications reported to precipitate acquired deficiency include isoniazid [13,15,18,33], practolol [22], procainamide [38,39], amiodarone [31], penicillin [30], ciprofloxacin [23] and tocilizumab [44]. The severity of bleeding is variable, as is the clinical course.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

A child with acquired factor XIII deficiency: case report and literature review

Kessel

Shore‐Lesserson

et al. 2013

Haemophilia

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Factor XIII (FXIII) deficiency is a rare bleeding disorder, which can result in life threatening hemorrhage. Rarer still is acquired FXIII deficiency, in which the disorder is due to autoantibodies that inhibit the factor. To describe one of the youngest reported patients with this condition. To discuss the challenges we encountered in monitoring response with the available assays. To review the literature and provide a review of all acquired FXIII cases. We present the case of our patient, a 9-year-old girl with acquired FXIII deficiency. We present a comprehensive review of all acquired FXIII deficiency cases reported globally in English, with focus on clinical presentation, diagnostic assays, treatment and prognosis. There is no current standard for therapy and measuring response to therapy can be complicated by limitations of assays in the presence of inhibitors. Clinicians should be aware of acquired FXIII deficiency as a potentially life threatening bleeding disorder even in young children. The case presented illustrates a young patient with acquired FXIII deficiency with a good clinical response to cryoprecipitate and difficulty in hemostasis monitoring utilizing clinically available assays.

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“…This may be not a clear case of drug-induced antibodies because CTRX was administered approximately 6 months before the bleeding onset, and antibodies to CTRX are not of autoantibody type but only of the immune-complex type [11]. In the case of AH13, anti-FXIII inhibitors had developed while patients were taking isoniazid for pulmonary tuberculosis or ciprofloxacin for medial otitis [12,13]. The neurosyphilis per se and HCV infection may have also been related to the development of the patient's antibodies.…”

Section: Resultsmentioning

confidence: 92%

Aggressive fatal case of autoimmune hemorrhaphilia resulting from anti-Factor XIII antibodies

Sugiyama¹,

Uesugi²,

Suzuki³

et al. 2013

Blood Coagulation &Amp; Fibrinolysis

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Factor XIII (FXIII) is a fibrin-stabilizing factor consisting of catalytic A subunits (FXIII-A) and carrier B subunits (FXIII-B). Congenital FXIII deficiency is a rare bleeding disorder. Acquired FXIII deficiency resulting from FXIII hypo-synthesis and/or hyperconsumption is a relatively common disorder in which patients seldom bleed. On the contrary, 'autoimmune/acquired hemorrhaphilia XIII/13 due to anti-FXIII antibodies (AH13)' is a rare but life-threatening bleeding disorder. Through a nationwide survey of AH13, we diagnosed aggressive AH13 in a 66-year-old woman. She consulted our department because of a spontaneous hematoma in her hand. After 1.5 months, she also developed an intramuscular hematoma but retained approximately half (52%) of the normal FXIII activities. The patient's bleeding symptoms were aggravated to catastrophic massive bleedings in the large abdominal muscles and intrapelvic and intraperitoneal spaces. Two months after the bleeding onset, she died despite undergoing plasma exchange, which was performed because we were deeply suspicious of the presence of an anti-FXIII inhibitor. Seven days after her death, extremely low FXIII activity (6%) and positive data on anti-FXIII inhibitor were reported by a commercial laboratory. Our dot blot assay detected anti-FXIII-A autoantibodies, afterwards. Thus, the diagnosis of aggressive AH13 as early as possible is necessary to save patients' lives.

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Hemorrhagic disorder due to an isoniazid-associated acquired factor XIII inhibitor in a patient with waldenström's macroglobulinemia

Cited by 9 publications

References 36 publications

Anti‐factor XIII A subunit (FXIII‐A) autoantibodies block FXIII‐A2B2 assembly and steal FXIII‐A from native FXIII‐A2B2

Anti‐factor XIII A subunit (FXIII‐A) autoantibodies block FXIII‐A2B2 assembly and steal FXIII‐A from native FXIII‐A2B2

A child with acquired factor XIII deficiency: case report and literature review

Aggressive fatal case of autoimmune hemorrhaphilia resulting from anti-Factor XIII antibodies

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