Successful management of concurrent acquired hemophilia A and a lupus anticoagulant in a pediatric hematopoietic stem cell transplant patient

Jones, LaQuita M; Dandoy, Christopher E.; Jodele, Sonata; Myers, Kasiani C.; Luchtman‐Jones, Lori; Quinn, Charles T.; Mullins, Eric S.; El-Bietar, Javier

doi:10.1038/s41409-017-0041-0

Cited by 1 publication

(3 citation statements)

References 13 publications

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“…46 – 52 Symptoms described at diagnosis were muscle haematomas, 46 , 47 , 52 ankle haemarthrosis, 46 severe bleeding after tooth extraction. 49 Associated conditions were: previous HSCT and concomitant staphylococcus aureus infection, 50 streptococcal infection and amoxicillin treatment, 51 previous course of amoxicillin. 52 …”

Section: Specific Aha Populationsmentioning

confidence: 99%

“…Other cases have been described regarding 7 children (4 males, 3 females) diagnosed with AHA at a median age of 10 years (range 5-14). [46][47][48][49][50][51][52] Symptoms described at diagnosis were muscle haematomas, 46,47,52 ankle haemarthrosis, 46 severe bleeding after tooth extraction. 49 Associated conditions were: previous HSCT and concomitant staphylococcus aureus infection, 50 streptococcal infection and amoxicillin treatment, 51 previous course of amoxicillin.…”

mentioning

confidence: 99%

“…[46][47][48][49][50][51][52] Symptoms described at diagnosis were muscle haematomas, 46,47,52 ankle haemarthrosis, 46 severe bleeding after tooth extraction. 49 Associated conditions were: previous HSCT and concomitant staphylococcus aureus infection, 50 streptococcal infection and amoxicillin treatment, 51 previous course of amoxicillin. 52 Two papers have recently regarded AHA in the elderly: one review from 80 studies, including 159 cases 53 and a cases report concerning a small number of patients.…”

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confidence: 99%

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Acquired Haemophilia A: An Intriguing Disease

Mazzucconi¹,

Baldacci

Ferretti

et al. 2020

Mediterr J Hematol Infect Dis

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Acquired Haemophilia A is a rare acquired bleeding disorder caused by autoantibodies directed against Factor VIII, which neutralize FVIII activity. These inhibitors differ from alloantibodies against FVIII which can occur in congenital Haemophilia A after repeated exposures to plasma-derived or recombinant FVIII products. In most cases the disease occurs suddenly in subjects without personal or familiar history of bleedings, with symptoms that may be mild, moderate or severe. However, only laboratory alterations are present in ̴ 30% of patients. The incidence varies from 1 to 4 cases per million/year; more than 80% of patients are elderly, males and females are similarly affected. There is a small peak of incidence related to pregnancy in young women aged 20–40 years. The disease may be underdiagnosed in the elderly. The diagnostic algorithm is based on an isolated prolonged activated partial thromboplastin time, normal thrombin time, absence of Lupus Anticoagulant and a mixing test that reveals the presence of an inhibitor: the finding of reduced FVIII activity and the detection of neutralising autoantibodies against FVIII lead to diagnosis. The disease is idiopathic in 44%-63% of cases, while in the others etiological factors are present. Bleeding prevention and treatment are based on therapeutic tools as bypassing agents, recombinant porcine FVIII concentrate or, in a limited number of cases, FVIII concentrates and desmopressin. As soon as the diagnosis has been made, immunosuppressive therapy must be started to eradicate the inhibitor. Better knowledge of the disease, optimal management of bleeding and eradication of the inhibitor have significantly reduced morbidity and mortality in most patients. Keywords: autoantibodies against FVIII, bleeding symptoms, bleeding treatment, eradication therapy.

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