Treatment of acquired factor X inhibitor by plasma exchange with concomitant intravenous immunoglobulin and corticosteroids

Hsia, Cyrus C.; Keeney, Mike; Bosco, Annmarie; Xenocostas, Anargyros

doi:10.1002/ajh.21105

Cited by 10 publications

(18 citation statements)

References 15 publications

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“…As expected, preventing the formation of FXa severely impairs the function of the prothrombinase complex to generate thrombin. This observation may explain the discrepancy reported in the effectiveness of rFVIIa [21,27], as cases in which FXa generation or FXa activity is inhibited would not be expected, a priori , to respond to rFVIIa. This provides an added rationale for determining the mechanism of action for each acquired FX inhibitor.…”

Section: Discussionmentioning

confidence: 99%

Acquired, non‐amyloid related factor X deficiency: review of the literature

Lee¹,

Duan-Porter

Metjian³

2012

Haemophilia

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Summary. Factor X (FX) deficiency is a rare coagulopathy due to congenital deficiency (Stuart–Prower disease) or in association with primary amyloidosis. Acquired and isolated FX deficiency occurring in the absence of a plasma cell dyscrasia has only been infrequently described. After recently diagnosing and treating a case of acquired, isolated FX deficiency, we embarked upon a review of the literature to help guide clinicians who may face this clinical situation. The literature was reviewed to identify cases of isolated, acquired FX deficiency unrelated to congenital deficiency, use of vitamin K antagonists, or amyloidosis. There were 34 cases of acquired FX deficiency identified, occurring in association with malignancy, drug exposure and infection. The majority of described cases (38%) were preceded by a non‐specific respiratory viral illness. The initial presentation was variable, ranging from no bleeding to life‐threatening haemorrhage. Twenty per cent of patients had musculoskeletal bleeding resembling patients with haemophilia. Both the prothrombin time and the activated partial thromboplastin time were markedly prolonged in nearly all patients. In 26% of patients, a specific FX inhibitor was identified. Numerous therapies have been utilized in patients with acquired FX deficiency including high‐dose glucocorticoids, plasma exchange with fresh frozen plasma and intravenous immunoglobulin. In 18% of patients, the coagulopathy resolved spontaneously. All patients achieved a complete recovery. Acquired factor X deficiency is a rare disorder, commonly associated with a preceding viral illness and a circulating FX inhibitor. Although multiple treatment modalities have been described with variable success, in many cases, it is a self‐limited condition.

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Section: Discussionmentioning

confidence: 99%

Acquired, non‐amyloid related factor X deficiency: review of the literature

Lee¹,

Duan-Porter

Metjian³

2012

Haemophilia

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“…Support with rFVIIa and suppression with rituximab are both potentially effective modalities, but in light of the few cases where these modalities have been used, and because of their expense and questionable efficacy, their utility cannot be advocated without more data. rFVIIa has been used in one other case of factor X inhibitor, albeit unsuccessfully [9]. It has, however, been reported to provide some efficacy in patients with amyloid-mediated factor X deficiency [13].…”

Section: Discussionmentioning

confidence: 99%

“…Severe bleeding generally occurs with factor X levels less than 20%, probably because of the central role factor X plays in both intrinsic and extrinsic pathways. Most factor X inhibitors have been reported as occurring after acute respiratory illness, exposure to macrolide antibiotics or in association with a malignancy (table 1) [4,5,6,7,8,9,10]. Acquired factor X deficiencies have also been described after similar prodromes; this entity is also rare and may represent a different presentation of the same rare syndrome.…”

Section: Introductionmentioning

confidence: 99%

Factor X Inhibitor: A Fulminant Presentation and Fatal Course of a Rare Syndrome in a 59-Year-Old Male

Gollard¹,

Rahman²,

Ratnasabapathy³

2012

Acta Haematol

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Factor X inhibitors are rare. The few cases documented in the literature have occurred after viral prodromes, in association with cancer, or after exposure to antibiotics. Acquired factor X deficiencies are also rare and their etiology is largely unknown. We report a new case of a factor X inhibitor and review prior cases of both factor X inhibitors and non-amyloidosis-related acquired factor X deficiencies.

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“…Unlike AHA, there is no consensus on the optimal treatment of acquired FXI inhibitors [1] and most data are derived from treatment of patients with congenital FXI deficiency, with or without secondary inhibitors [19]. Proposed treatment includes (a) antifibrinolytic agents [10, 19], aPCC [20], or rFVIIa [15] for arresting the bleeding, (b) corticosteroids [4, 5, 7–9, 11, 12], azathioprine [6, 8, 11], intravenous immunoglobulins [6, 21], plasma-exchange [21], or rituximab [8] for inhibitor eradication, and (c) specific treatment of the underlying immunologic disorder [5, 8]. In the present report, no hemostatic therapy was administered because of unavailability of coagulation tests, severe disability, and rapid clinical deterioration and death.…”

Section: Discussionmentioning

confidence: 99%

Acquired Factor XI Inhibitor Presenting as Spontaneous Bilateral Subdural Hematoma in an Elderly Patient

Vazzana

Scarti

Beltrame

et al. 2014

Case Reports in Hematology

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Development of autoantibodies against coagulation factors is an uncommon bleeding disorder associated with cancer, autoimmune conditions, pregnancy, or no apparent disease. Spontaneous FVIII inhibitors are the most frequently encountered; those against FXI have been only anecdotally reported. We report a case of acquired FXI inhibitor presenting as fatal intracranial spontaneous bleeding in an elderly patient with history of cancer and previous transfusions. Few cases of acquired FXI inhibitor have been reported in association with connective tissue disease, cancer, or surgery. Bleeding includes mucocutaneous bleeding, postsurgical hemorrhage, or life-threatening events. Treatment consists of arresting the bleeding and inhibitor eradication. High degree of suspicion is essential to promptly diagnose and treat this uncommon condition.

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Treatment of acquired factor X inhibitor by plasma exchange with concomitant intravenous immunoglobulin and corticosteroids

Cited by 10 publications

References 15 publications

Acquired, non‐amyloid related factor X deficiency: review of the literature

Acquired, non‐amyloid related factor X deficiency: review of the literature

Factor X Inhibitor: A Fulminant Presentation and Fatal Course of a Rare Syndrome in a 59-Year-Old Male

Acquired Factor XI Inhibitor Presenting as Spontaneous Bilateral Subdural Hematoma in an Elderly Patient

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