Surgery-associated acquired hemophilia A

Theodossiades, G.; Tsevrenis, V.; Nomikou, E.; Dadiotis, L; Kontopoulou-Griva, I.

doi:10.1007/s002770100367

Cited by 17 publications

(8 citation statements)

References 10 publications

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“…Of note, while previous cases have shown acquired hemophilia developing immediately following surgery [ 33 , 34 ], our patient did not develop AHA until 24 days post-op. This lag seems to indicate the development of IgG autoantibodies.…”

Section: Discussioncontrasting

confidence: 59%

“…However, documentation of trauma or surgery as etiologies themselves is rare. Alumkal et al (1999) [ 33 ] and Theodossaides et al (2001) [ 34 ] both published cases of patients with no previous histories developing acquired hemophilia immediately following surgery. Green and Lechner (1981) [ 35 ] also found an association with trauma and/or surgery after reviewing 215 patients.…”

Section: Discussionmentioning

confidence: 99%

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A Hematological Menace: Multiple Venous Thrombosis Complicated by Acquired Factor VIII Deficiency

et al. 2016

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Patient: Male, 21Final Diagnosis: Acquired Factor VIII DeficiencySymptoms: Abdominal hematoma • DVT • life threatening bleedingMedication: —Clinical Procedure: Life saving medical therapySpecialty: HematologyObjective:Rare diseaseBackground:Acquired hemophilia A (AHA) classically presents with spontaneous bleeding of mucosal sites, GI tract, and sub-cutaneous tissues, often leading to large hematomas and ecchymosis. Among documented cases, 50% are idiopathic and few have been associated with trauma or surgery. We present a case of life-threatening bleeding caused by AHA, following trauma and complicated by multiple venous thrombi.Case Report:A 21-year-old man presented with multiple injuries secondary to trauma leading to extensive life-saving surgery. Two weeks post-operatively, he developed multiple deep venous thrombi and was started on anticoagulation. Twenty-four days post-operatively, he started bleeding from multiple mucosal sites and developed an abdominal hematoma. Anticoagulation was stopped, with administration of fresh frozen plasma and vitamin K. Diagnosis of AHA was made based on low factor VIII level and presence of factor VIII inhibitors after an appropriate battery of tests ruled out other possible diagnoses. He was started on steroids and recombinant factor VIIa, leading to immediate improvement. Once stable, Rituximab infusions resulted in decreasing factor VIII inhibitor levels, with gradual normalization of PTT.Conclusions:AHA remains a diagnostic challenge because of its rarity, leading to delay in diagnosis and causing significant morbidity and mortality. Elevated PTT relative to PT/INR is a strong clue which should be followed by mixing studies. Very few cases have been associated with surgery or trauma and relatively few large, controlled trials have compared different treatment modalities for AHA. Growing evidence supports anti-CD20 (Rituximab) as an effective treatment option, as in this case.

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Section: Discussioncontrasting

confidence: 59%

Section: Discussionmentioning

confidence: 99%

A Hematological Menace: Multiple Venous Thrombosis Complicated by Acquired Factor VIII Deficiency

et al. 2016

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“…AHA is diagnosed in patients without a personal or family history of bleeding, who have prolonged APTT, reduced FVIII levels, and detectable specific AAFVIIIs. Surgery is known to be a precipitating cause of acquired hemophilia, which is something to be considered with unusual postoperative bleeding [ 10 , 11 ]. In our case, due to the intervening period of approximately 2 months, we did not consider surgery to be directly associated with the inhibitor, although it might have played a role in its development.…”

Section: Discussionmentioning

confidence: 99%

Myelofibrosis and acquired hemophilia A: a case report

et al. 2016

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BackgroundMyelofibrosis and acquired hemophilia A is a rare association. To the best of our knowledge only one case of myelofibrosis and acquired hemophilia A has been previously described.Case presentationA 66-year-old Caucasian man diagnosed with myelofibrosis evolving in acute myeloid leukemia was referred to us for postoperative bleeding. Hemostatic studies showed prolonged activated partial thromboplastin time, decreased factor VIII coagulation, and a high factor VIII inhibitor titer; these findings led to a diagnosis of acquired hemophilia A for which he was treated with methylprednisolone and recombinant activated factor VII on admission. Due to a lack of response he was subsequently treated with rituximab combined with activated prothrombin complex concentrates. Furthermore, he received azacytidine to treat the underlying hematological malignancies. Immunosuppressive rituximab therapy resolved acquired hemophilia A with marked efficacy.ConclusionsRapid and accurate diagnosis, effective hemostatic therapy, and timely treatment for underlying disease are important in the management of acquired hemophilia A secondary to hematological malignancy.

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“…The incidence of AHA is extremely rare, with approximately 0.2–1.48 cases per million per year [ 1 , 4 , 7 , 8 ]. Acquired FVIII inhibitors are classically associated with chronic inflammatory disorders, autoimmune disorders, pregnancy, malignancies, multiple transfusions, and drugs [ 1 – 5 , 7 , 8 ]. Approximately, 10% of patients with AHA have an underlying malignancy [ 9 ], and many have undergone surgery.…”

Section: Introductionmentioning

confidence: 99%

Acquired Hemophilia A Presenting as Massive Postoperative Bleeding in a Patient with Oral Squamous Cell Carcinoma

Oba

Nakahira

Kogashiwa

et al. 2020

Case Reports in Otolaryngology

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Acquired hemophilia A (AHA) is an extremely rare and serious bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). Approximately, 10% of patients with AHA have an underlying malignancy. We report on a 46-year-old man with AHA and advanced oral cancer who presented with massive bleeding after surgery. Preoperative blood coagulation tests showed no abnormalities. He underwent radical tumor resection followed by reconstruction using a free rectus abdominal musculocutaneous flap. Massive subcutaneous hemorrhage developed in his neck and abdomen on the first postoperative day. The hemorrhage remained uncontrolled, despite embolization of the responsible vessels. Subsequent laboratory data showed prolonged activated partial thromboplastin time and decreased FVIII levels. On the basis of his clinical course and the presence of the FVIII inhibitor, we speculated that the patient suffered from AHA. We administered recombinant activated factor VII and prednisolone, after which the spontaneous bleeding stopped and the subcutaneous hemorrhage resolved. A review of the literature identified only three previous documented cases of AHA associated with head and neck cancer. This case indicates that AHA should not be ruled out in patients with uncontrolled postoperative bleeding, while attempting to ensure bleeding control and preventing potentially catastrophic fatal consequences.

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Surgery-associated acquired hemophilia A

Cited by 17 publications

References 10 publications

A Hematological Menace: Multiple Venous Thrombosis Complicated by Acquired Factor VIII Deficiency

A Hematological Menace: Multiple Venous Thrombosis Complicated by Acquired Factor VIII Deficiency

Myelofibrosis and acquired hemophilia A: a case report

Acquired Hemophilia A Presenting as Massive Postoperative Bleeding in a Patient with Oral Squamous Cell Carcinoma

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