Resolution of Acquired Factor X Deficiency and Amyloidosis with Melphalan and Prednisone Therapy

Camoriano, John; Greipp, P. R.; Bayer, Gerald K.; Bowie, E. J. W.

doi:10.1056/nejm198704303161806

Cited by 41 publications

(15 citation statements)

References 13 publications

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“…All of the 4 patients achieving a CR were found to have increased levels of factor X after treatment and experienced no further clinical bleeding; 1 patient achieving a hematologic PR experienced a normalization of the factor X level, while the other 2 patients (1 PR, 1 NR) had no improvement in factor X ( Table 2). There are reports of isolated cases of AL amyloidosis in which resolution of acquired factor X deficiency occurs spontaneously, 12 after treatment with oral melphalan, 13,14 or after splenectomy. 15,16 This series is the first in which the salutary impact of high-dose chemotherapy and stem cell transplantation upon factor X levels has been reported.…”

Section: Resultsmentioning

confidence: 99%

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

Choufani

Sanchorawala

Ernst

et al. 2001

Blood

199

159

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Acquired deficiency of factor X occurs in patients with systemic amyloid lightchain (AL) amyloidosis, presumably due to adsorption of factor X to amyloid fibrils. Of 368 consecutive patients with systemic AL amyloidosis evaluated at Boston Medical Center, 32 patients (8.7%) had factor X levels below 50% of normal. Eighteen of these patients (56%) had bleeding complications, which were more frequent and severe in the 12 patients below 25% of normal; 2 episodes were fatal. Ten factor X-deficient patients received high-dose melphalan chemotherapy followed by autologous stem cell transplantation. Of 7 patients alive 1 year after treatment, 4 had a complete hematologic response, and all 4 experienced improvement in their factor X levels. One of 2 additional patients with partial hematologic responses had improvement in factor X. Thus, aggressive treatment of the underlying plasma cell dyscrasia in AL amyloidosis can lead to the amelioration of amyloid-related factor X deficiency. (Blood. 2001;97: 1885-1887)

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Section: Resultsmentioning

confidence: 99%

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

Choufani

Sanchorawala

Ernst

et al. 2001

Blood

199

159

View full text Add to dashboard Cite

show abstract

“…It has been proposed that clotting factors bind to amyloid fibrils and there are anecdotal reports of improvements in haemostasis after removal of heavily infiltrated spleens (Greipp et al, 1979), although a more recent study did not support this theory and attributed most abnormalities in coagulation to either impaired fibrin polymerization or a reduction in FX activity (Mumford et al, 2000). There are also reports of reversal of coagulopathy following cytotoxic chemotherapy (Camoriano et al, 1987). FX concentrate is available and anecdotal evidence suggests it is effective although it has a markedly shortened half-life and may need repeated dosing every 24 h (compared to every few days in other cases of FX deficiency).…”

Section: Bleeding/thrombosismentioning

confidence: 99%

Guidelines on the management of AL amyloidosis

Wechalekar¹,

Gillmore²,

Bird

et al. 2014

Br J Haematol

116

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“…[7] Resolution of hemostatic abnormality has been described by many investigators post splenectomy which helps by removing a considerable burden of amyloid. [8] Spontaneous remission with no specific active therapy has also been described in literature. [9] As a more aggressive approach, high-dose chemotherapy followed by autologous stem-cell transplantation usually have the best response rate of up to 60% as described by Chouffeni et al and thus offer advantages regarding long term outcome or survival.…”

Section: Discussionmentioning

confidence: 98%