Cerebellar hemorrhage as a first presentation of acquired Hemophilia A

Micić, Dejan; Williams, Eliot C.; Medow, Joshua E.

doi:10.1007/s12028-010-9489-0

Cited by 11 publications

(1 citation statement)

References 10 publications

(27 reference statements)

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“…A subgroup with a lower inhibitor titer (<20 Bethesda units/mL) and a higher baseline FVIII activity (≥1 IU/dL) was found to have >50% chance of achieving PR with steroids alone by day 21 [ 12 ]. In one case, recurrence of spontaneous cerebellar and cerebral hemorrhage with FVIII activity at 13% and FVIII inhibitor titer 1.7 Bethesda units/mL was expired [ 13 ]. In the cases of AHA with intracranial hemorrhage, the treatment might be more difficult than other typical cases.…”

Section: Discussionmentioning

confidence: 99%

Acquired Hemophilia A with a Rare Presentation of Acute Subdural Hematoma

Sehara¹,

Hayashi

Mimuro³

2015

Case Reports in Neurological Medicine

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An 80-year-old man was admitted for acute subdural hematoma caused by a mild brain injury. His coagulation test showed an isolated prolongation of activated partial thromboplastin time (aPTT). Though the subdural hematoma did not progress, oozing bleed from the wound of tracheostomy continued. Failure of correction on aPTT mixing test supported the presence of an inhibitor to a coagulation factor. Once the diagnosis of acquired hemophilia A (AHA) was made, steroid therapy was performed, which leads him to complete remission of AHA. Isolated prolongation of aPTT can be the key to diagnose a rare coagulopathy, such as AHA.

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Section: Discussionmentioning

confidence: 99%

Acquired Hemophilia A with a Rare Presentation of Acute Subdural Hematoma

Sehara¹,

Hayashi

Mimuro³

2015

Case Reports in Neurological Medicine

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Acquired hemophilia A as a disease of the elderly: A comprehensive review of epidemiology, pathogenesis, and novel therapy

Lehoczki,

Fekete,

Mikala

et al. 2024

GeroScience

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Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder characterized by the development of neutralizing autoantibodies (inhibitors) against coagulation factor VIII (FVIII). This review provides an in-depth exploration of AHA, covering its epidemiology, pathogenesis, clinical presentation, diagnosis, complications, and treatment strategies, focusing on recent advancements. AHA can manifest in both men and women with no prior bleeding history. The annual incidence is estimated to be approximately 1 case per million individuals in the general population. The incidence increases significantly with age: the incidence among individuals aged 60 years or older is approximately 3 to 4 cases per million individuals per year. Typically, patients present with an acquired bleeding disorder that is characterized by an isolated, prolonged activated partial thromboplastin time stemming from FVIII deficiency. Diagnosis relies on the detection of neutralizing antibodies using the Nijmegen-modified Bethesda assay. Hemostatic control strategies involve bypassing agents like recombinant activated factor VII, activated prothrombin complex concentrate, and recombinant porcine FVIII for bleeding patients. Emicizumab, a novel bypassing agent, exhibits several potential advantages. In the realm of immunosuppressive treatment for inhibitor eradication, the CyDRi regimen emerged as a remarkable advancement, significantly enhancing the outlook for the management of AHA even in the elderly frail population.

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A case of acquired hemophilia A diagnosed after percutaneous endoscopic gastrostomy

Okamura¹,

Komatsu²,

Ito³

et al. 2015

Clin J Gastroenterol

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A 65-year-old male with no personal or familial history of bleeding disorders underwent percutaneous endoscopic gastrostomy (PEG) for neurogenic dysphagia due to subarachnoid hemorrhage. On postoperative day 6, continuous oozing of venous blood was observed at the stoma. Prothrombin time was within normal range, but activated partial thromboplastin time was prolonged. Cross-mixing test results indicated the existence of an inhibitor, and laboratory findings revealed decreased factor VIII activity and high levels of factor VIII inhibitor. The patient was diagnosed as having acquired hemophilia A, for which steroid monotherapy was effective. Acquired hemophilia A is a rare but potentially fatal disease. Clinicians should be aware of this condition in patients presenting with sudden hemorrhage after PEG or other endoscopic treatments, even in those with no apparent history of bleeding.

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Cerebellar hemorrhage as a first presentation of acquired Hemophilia A

Cited by 11 publications

References 10 publications

Acquired Hemophilia A with a Rare Presentation of Acute Subdural Hematoma

Acquired Hemophilia A with a Rare Presentation of Acute Subdural Hematoma

Acquired hemophilia A as a disease of the elderly: A comprehensive review of epidemiology, pathogenesis, and novel therapy

A case of acquired hemophilia A diagnosed after percutaneous endoscopic gastrostomy

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