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Reactions 1463, p14 - 3 Aug 2013 S Ceftriaxone Fatal acquired haemophilia? in an elderly patient: case report A 66-year-old woman developed acquired haemophilia due to anti-factor XIII inhibitors (AH13) after treatment with ceftriaxone for neurosyphilis. She subsequently died. The woman had neurosyphilis in 2010 and received a ceftriaxone infusion [dosage not stated]. She presented with a haematoma in her right hand in February 2011. Ceftriaxone had been administered approximately 6 months prior to bleeding onset. She underwent incision of her haematoma and the wound continuously oozed. She developed gingival bleeding while brushing her teeth and epistaxis. She consulted a haematology department and tests revealed a slightly prolonged activated partial thromboplastin time. Debridement of necrotic tissue of her right hand was performed and she had repeated postoperative bleedings. She developed an intramuscular haematoma in her right thigh and went into shock. Factor XIII (FXIII) deficiency was suspected. An ammonia release assay showed FXIII activity of 52%. The woman received prednisolone. She developed a diffuse subcutaneous haematoma from her waist to her thigh and an intramuscular haematoma in her left abdominal rectus. She was transferred to an ICU because of shock and a deterioration of consciousness. Her abdominal haemorrhage expanded into her frontal intrapelvic cavity and she developed intraperitoneal haemorrhage. She underwent haemodiafiltration and plasma exchange but eventually died due to haemorrhagic shock which resulted from her intraperitoneal bleeding. Her death occurred within 2 months after her first bleeding episode. Results of FXIII activity available after her death revealed extremely low levels of activity. Tests also showed a strongly positive result for an anti-FXIII inhibitor and assays showed anti-FXIII-A antibodies. Author comment: "[D]rug-induced disorders cannot be completely excluded from the underlying cause of this AH13 case, because she had received [ceftriaxone] for neurosyphilis. . . This may be not a clear case of drug-induced antibodies because [ceftriaxone] was administered approximately 6 months before the bleeding onset, and antibodies to [ceftriaxone] are not of autoantibody type but only of the immune-complex type." Sugiyama H, et al. Aggressive fatal case of autoimmune hemorrhaphilia resulting from anti-Factor XIII antibodies. Blood Coagulation and Fibrinolysis 24: 85-9, No. 1, Jan 2013. Available from: URL: http://dx.doi.org/10.1097/ MBC.0b013e328358e8e7 - Japan 803090682 1 Reactions 3 Aug 2013 No. 1463 0114-9954/13/1463-0001/$14.95 Adis © 2013 Springer International Publishing AG. All rights reserved

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Reactions 1463, p14 - 3 Aug 2013

SCeftriaxone

Fatal acquired haemophilia? in an elderly patient:case report

A 66-year-old woman developed acquired haemophilia dueto anti-factor XIII inhibitors (AH13) after treatment withceftriaxone for neurosyphilis. She subsequently died.

The woman had neurosyphilis in 2010 and received aceftriaxone infusion [dosage not stated]. She presented with ahaematoma in her right hand in February 2011. Ceftriaxonehad been administered approximately 6 months prior tobleeding onset. She underwent incision of her haematoma andthe wound continuously oozed. She developed gingivalbleeding while brushing her teeth and epistaxis. She consulteda haematology department and tests revealed a slightlyprolonged activated partial thromboplastin time. Debridementof necrotic tissue of her right hand was performed and she hadrepeated postoperative bleedings. She developed anintramuscular haematoma in her right thigh and went intoshock. Factor XIII (FXIII) deficiency was suspected. Anammonia release assay showed FXIII activity of 52%.

The woman received prednisolone. She developed a diffusesubcutaneous haematoma from her waist to her thigh and anintramuscular haematoma in her left abdominal rectus. Shewas transferred to an ICU because of shock and a deteriorationof consciousness. Her abdominal haemorrhage expanded intoher frontal intrapelvic cavity and she developed intraperitonealhaemorrhage. She underwent haemodiafiltration and plasmaexchange but eventually died due to haemorrhagic shockwhich resulted from her intraperitoneal bleeding. Her deathoccurred within 2 months after her first bleeding episode.Results of FXIII activity available after her death revealedextremely low levels of activity. Tests also showed a stronglypositive result for an anti-FXIII inhibitor and assays showedanti-FXIII-A antibodies.

Author comment: "[D]rug-induced disorders cannot becompletely excluded from the underlying cause of thisAH13 case, because she had received [ceftriaxone] forneurosyphilis. . . This may be not a clear case of drug-inducedantibodies because [ceftriaxone] was administeredapproximately 6 months before the bleeding onset, andantibodies to [ceftriaxone] are not of autoantibody type butonly of the immune-complex type."Sugiyama H, et al. Aggressive fatal case of autoimmune hemorrhaphilia resultingfrom anti-Factor XIII antibodies. Blood Coagulation and Fibrinolysis 24: 85-9, No.1, Jan 2013. Available from: URL: http://dx.doi.org/10.1097/MBC.0b013e328358e8e7 - Japan 803090682

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Reactions 3 Aug 2013 No. 14630114-9954/13/1463-0001/$14.95 Adis © 2013 Springer International Publishing AG. All rights reserved