Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy

Choufani, Élie; Sanchorawala, Vaishali; Ernst, T J; Quillen, Karen; Skinner, Martha; Wright, Daniel G.; Seldin, David C.

doi:10.1182/blood.v97.6.1885

Cited by 199 publications

(177 citation statements)

References 15 publications

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“…Inherited factor X deficiency has been reported to have an incidence of 1:1,000,000 [1,2]. Acquired factor X deficiency is even rarer and is associated with amyloidosis in the majority of cases [3]. Only a few cases of factor X deficiency attributed to an inhibitor in adults have been described in the literature [4][5][6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2007

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A patient with spontaneous hemorrhage from multiple body sites was found to have markedly prolonged international normalized ratio (INR) and activated partial thromboplastin times (aPTT) with incomplete correction of aPTT on mixing studies using normal plasma. The cause of this severe hemorrhage was due to a specific factor X inhibitor. No underlying or associated diseases were found. Initial treatment with fresh frozen plasma, vitamin K, and recombinant activated factor VII (rFVIIa) was unsuccessful. However, therapy utilizing plasma exchange with concomitant intravenous immunoglobulin and corticosteroids resulted in a rapid and sustained normalization of factor X levels with a clinical hemostatic response. Am. J. Hematol. 83:318-320, 2008. V

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Section: Introductionmentioning

confidence: 99%

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

et al. 2007

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“…Six to fourteen percent of AL amyloidosis patients develop an acquired Factor X (FX) deficiency 3, 4, 5. The direct binding/adsorption of FX onto amyloid fibrils has been thought to be a major mechanism for FX deficiency in amyloidosis.…”

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confidence: 99%

Direct Factor X sequestration by systemic amyloid light‐chain amyloidosis

Tashiro

Shirasaki

Watanabe

et al. 2018

Clinical Case Reports

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“…Acquired factor X deficiency is the most common coagulation factor deficiency that has been identified in patients with AL amyloidosis [27]. It has been reported to occur in 6.3-14% of these patients [24,[27][28][29].…”

Section: Primary Amyloidosismentioning

confidence: 99%

“…It has been reported to occur in 6.3-14% of these patients [24,[27][28][29]. Factor X deficiency has long been thought to result from adsorption or the in vivo binding of the factor to the amyloid fibrils [30,31].…”

Section: Primary Amyloidosismentioning

confidence: 99%

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Splenectomy in plasma cell dyscrasias: A review of the clinical practice

et al. 2006

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Veterans Healthcare System, Little Rock, ArkansasPlasma cell dyscrasias are a group of clinically and biochemically diverse disorders of unknown etiology, characterized by the disproportionate proliferation of one or more clones of B cells, and the presence of a structurally and electrophoretically homogeneous (monoclonal) immunoglobulin or polypeptide subunit in serum or urine. The role of splenectomy in the management of plasma cell dyscrasias has not been well defined. Using MEDLINE, the authors searched the English-language published literature from the year 1970 through September 2005 to determine the indications for splenectomy in plasma cell dyscrasias.A review of the literature in humans and animals supported the idea that the spleen provides a special microenvironment favorable for homing or differentiation of IgM producing B cells, and splenectomy can, at times, lead to remission in Waldenströ m's macroglobulinemia. The other reported reasons for splenectomy in plasma cell dyscrasias are hypersplenismrelated pancytopenia, control of splenic plasmacytomas, and management of a splenic abscess. Splenic infiltration in primary amyloidosis can be an indication for splenectomy, where removal of a large spleen can also reverse an acquired factor X deficiency. Thus, the spleen can be considered a potential target organ for management of plasma cell dyscrasias, and therapeutic success has been achieved with removal of this organ.However, splenectomy can be a potentially morbid procedure in patients with plasma cell dyscrasias, and major postoperative complications include infection, hemorrhage, and thrombosis. Am. J. Hematol. 81:946-954, 2006. V V C 2006 Wiley-Liss, Inc.

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Acquired factor X deficiency in patients with amyloid light-chain amyloidosis: incidence, bleeding manifestations, and response to high-dose chemotherapy

Cited by 199 publications

References 15 publications

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

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

Direct Factor X sequestration by systemic amyloid light‐chain amyloidosis

Splenectomy in plasma cell dyscrasias: A review of the clinical practice

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