Acquired factor X deficiency. Altered plasma antithrombin activity and association with amyloidosis

Galbraith, P.A.

doi:10.1001/jama.230.12.1658

Cited by 15 publications

(13 citation statements)

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“…This condition is generally a feature of amyloidosis [16][17][18], and it is believed to be the consequence of rapid clearance of fac tor X from the circulation by adherence to amyloid fibrils [19] in the spleen [20] and elsewhere.…”

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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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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“…DISCUSSION While acquired selective factor X deficiency has occurred frequently in association with systemic amyloidosis (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12), the patients reported here demonstrated unique severe combined deficiencies of both factors IX and X. DISCUSSION While acquired selective factor X deficiency has occurred frequently in association with systemic amyloidosis (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12), the patients reported here demonstrated unique severe combined deficiencies of both factors IX and X.…”

Section: Resultsmentioning

confidence: 99%

Coagulopathy in amyloidosis: Combined deficiency of factors IX and X

et al. 1977

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Combined severe deficiencies of blood clotting factors IX and X were observed in 2 patients who suffered from systemic amyloidosis. This unique deficiency state was marked by refractoriness to Vitamin K as well as to transfusion therapy. Increased antithrombin activity was present in both individuals and corresponded in time to the emergence of a monoclonal IgG kappa light chain paraprotein in 1. Both patients demonstrated profound bleeding disorders. It is hypothesized that the Vitamin K dependent factors have special affinity for amyloid deposits due to an unusual amino acid (gamma-carboxyglutamic acid) present in these factors.

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“…Although TT and RT prolongations are peculiar features of AL Amyloidosis, they do not predict bleeding manifestations. Other coagulation abnormalities such as factor X deficiency, enhanced fibrinolysis, and amyloid angiopathy seem to correlate better with clinical symptoms (15). Deficiencies in specific coagulation factors in have long been recognized and along with factor X, acquired deficiencies in factor IX, factor II and factor VII have also been described (16).…”

Section: Clotting Alterationsmentioning

confidence: 99%