Acquired factor X deficiency with associated defects in platelet aggregation

Edgin, Richard A.; Metz, Earl N.; Fromkes, John J.; Beman, Floyd M.

doi:10.1016/0002-9343(80)90512-4

Cited by 15 publications

(21 citation statements)

References 13 publications

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“…Other laboratory abnormalities in patients with acquired FX deficiency have been described. In two patients, a prolonged bleeding time was found [24,29]. Abnormal platelet aggregation testing was reported in one of these patients, with an initial undetectable aggregation response seen with epinephrine and collagen administration.…”

Section: Resultsmentioning

confidence: 99%

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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: Resultsmentioning

confidence: 99%

“…Abnormal platelet aggregation testing was reported in one of these patients, with an initial undetectable aggregation response seen with epinephrine and collagen administration. This later normalized when FX levels increased [24]. A lupus anticoagulant was found in two other patients [27].…”

Section: Resultsmentioning

confidence: 99%

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

Lee¹,

Duan-Porter

Metjian³

2012

Haemophilia

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“…The best characterized and most common syndrome of acquired factor X deficiency occurs in amyloidosis; amyloid fibrils are actually thought to bind to and precipitate factor X from circulation [13,14,15,16]. Non-amyloid-associated-acquired factor X deficiencies have been documented; these could be a different facet of a continuum of immune-mediated factor X deficiencies rarely seen and reported (table 3) [17,18,19,20,21,22,23,24,25]. The possibility that both are in fact immune-mediated, perhaps from an epitope found both on a pathogen and factor X, is possible.…”

Section: Discussionmentioning

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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“…Stefanini and Wiggishoff[!6\ reported a case associated with adrenocortical carcinoma with remission of the deficiency after tumor removal. Edgin et al [27] reported a case associated with a viral syndrome that remitted after corticosteroid therapy. Our own case had no abnormal bleeding, and the deficiency apparently developed and resolved over a period of at most 3 weeks.…”

Section: Discussionmentioning

confidence: 99%

Progressive Systemic Sclerosis with the Nephrotic Syndrome and Acquired Factor X Deficiency

Eiser¹,

Zilversmit²,

Neff³

et al. 1986

Am J Nephrol

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A patient with progressive systemic sclerosis (PSS) involving skin, digit, esophagus, and lung developed the nephrotic syndrome and rapidly progressive renal insufficiency. Renal pathology revealed capillary collapse, cellular crescents, arteriolar hyaline deposition, and mesangial proliferation. On immunofluorescence IgM, C3, and fibrinogen were present in mesangium and capillary walls. Prebiopsy coagulation screening revealed a factor X deficiency which caused substantial prolongation of the partial thromboplastin time without an overt bleeding diathesis. The acquired factor X deficiency resolved after fresh frozen plasma and vitamin K administration, although some spontaneous improvement was noted. Nephrotic syndrome may occasionally be seen in the acute fulminant form of PSS and should not deter diagnosis of PSS.

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Acquired factor X deficiency with associated defects in platelet aggregation

Cited by 15 publications

References 13 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

Progressive Systemic Sclerosis with the Nephrotic Syndrome and Acquired Factor X Deficiency

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