Acquired hemophilia A: three different presentations of the same disease

Campo, Raquel; Bargay, J.; Cladera, Antonia; Balaguer, H; Requena, María J.; Mascaró, Martin

doi:10.1097/mbc.0b013e328335d012

Cited by 4 publications

(4 citation statements)

References 12 publications

(18 reference statements)

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“…Furthermore, in approximately 50% of reported cases, AH is associated with primary, underlying conditions involving autoimmune disease, drugs (eg penicillin, chloramphenicol, phenytoin, INF‐α), cancer, graft‐vs‐host disease after allogeneic bone marrow transplantation. Therefore, the increased mortality observed could also be related to underlying disorders, an older age at disease onset, or infectious complications associated with immunosuppressive or cytotoxic treatments …”

Section: Introductionmentioning

confidence: 99%

Acquired haemophilia in cancer: A systematic and critical literature review

et al. 2017

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Aim:There is a paucity of data on the clinical presentation and management of cancer patients with acquired haemophilia (AH), we here report a systematic literature review on acquired haemophilia in the context of cancer.Methods: Treatment outcomes of AH were defined as complete response (CR), partial response (PR) or no response (NR), based on inhibitor eradication, coagulation factor VIII levels and bleeding control. Reported deaths were either related to cancer or bleeding.Results: Overall, 105 cases were collected and analyzed according to classification of cancer and efficacy of treatments for inhibitor and malignancy. The mean age was 68 years for both males (range 37-86 years) and females (range 43-89 years), 39 patients were female subjects and 66 were males. A solid cancer was diagnosed in 60 subjects, while 45 patients suffered a haematological malignancy. Solid cancers affected mainly males; however, the incidence of solid tumours vs haematological malignancies was not statistically significant (P = .09). Not all patients were treated for their underlying cancer, bleeding and/or inhibitor, in two cases outcome is unavailable. CR was reported in 62.1% (64/103) cases, PR in 9.7% (10/103) cases, NR with or without death was reported in 28.1% (29/103) cases. Conclusion:CR was best achieved when successful and complete elimination of autoantibodies occurred contemporaneously with the successful treatment of the underlying malignancy. In some cases, recurrent autoantibodies were harbingers of relapsed cancer. Type of cancer, inhibitor titer, treatments administered for bleeding control and inhibitor eradication did not significantly affect clinical outcome of analyzed cases. K E Y W O R D Sacquired haemophila, bleeding, cancer, diagnosis, haematological malignancy, inhibitor, treatment

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Section: Introductionmentioning

confidence: 99%

Acquired haemophilia in cancer: A systematic and critical literature review

et al. 2017

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“…Other authors reported a hemorrhage into the pleural cavity in one male in a group of 15 acquired hemophilia patients [ 17 ]. Such patients may also experience other, less dangerous intramuscular and subcutaneous bleeding, nosebleeds, or bleeding after intravenous punctures and intramuscular injections [ 1 – 5 , 12 , 13 , 18 , 19 ]. According to Forsyth, bleeding into the skin appeared in 53% of patients [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

“…Hematomas on both legs appeared in another 68-year-old patient treated with warfarin and suffering from bronchial asthma, atrial fibrillation, and a complex heart-valve disorder. The third patient, a 66-year-old man with acute bronchial asthma, suffered from spontaneously appearing hematomas on both lower extremities [ 18 ]. Other authors described a case of an 82-year-old man with post-traumatic bleeding into the retroperitoneal space.…”

Section: Discussionmentioning

confidence: 99%

Acquired hemophilia A as a cause of recurrent bleeding into the pleural cavity – and literature review

Wojtyś¹,

Zuk²,

Alchimowicz³

et al. 2014

kitp

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Acquired hemophilia A is a coagulation disorder caused by autoantibodies against blood coagulation factor VIII. The first sign of this disease is often massive bleeding, which can affect patients after routine procedures. The parameter which indicates the presence of this condition is isolated prolonged activated partial thromboplastin time (APTT). The present article describes a case of a 32-year-old man with acute interstitial pneumonia and pleural effusion, in whom a massive hemothorax appeared after thoracocentesis; active bleeding was observed after the introduction of a chest tube. The patient was operated on, and no pinpoint bleeding was discovered during the procedure. Active bleeding was still taking place postoperatively. The patient underwent another operation after 6 days. Once more, no pinpoint bleeding was found. Prolonged APTT was observed. The activity of blood coagulation factor VIII was 3.04%. The presence of antibodies against factor VIII was confirmed, and acquired hemophilia was diagnosed. The article also includes an analysis of the literature on acquired hemophilia.

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“…The development of factor VIII inhibitors is often idiopathic, although it has been identified in numerous conditions including malignancy, the peripartum period, autoimmune and cutaneous disorders, graftversus-host disease following bone marrow transplant, and with the use of medications such as penicillin and phenytoin [1]. It has also been described in the literature to develop in association with asthma, although the incidence of this is poorly defined [1][2][3][4]. We were unable to identify a direct relationship between the medication list of our patient and acquired hemophilia at the time of this publication.…”

Section: Discussionmentioning

confidence: 99%