AL Amyloidosis Combined with Acquired Factor V Deficiency

Gatel, A; Cacoub, Patrice; Piette, Jean Charles

doi:10.7326/0003-4819-128-7-199804010-00031

Cited by 20 publications

(9 citation statements)

References 2 publications

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“…Clearly, the frequency and severity of hemorrhage was worse in the patients with the lowest factor X levels. Conversely, in the nonfactor X-deficient patients, serious hemorrhagic complications were rare in this patient population, although factor X deficiency is not the only cause of hemorrhage in patients with AL amyloidosis, who also suffer from capillary fragility because of amyloid deposition in the vessel walls, dysfibrinogenemia, abnormal platelet aggregation, 1,6,7 and other factor deficiencies, including deficiencies of factors II, VII, IX, 8 and V. 9 Elevation of the thrombin time and Russell viper venom clotting time can be seen. 10,11 Nephrotic syndrome was present in 11 of the 32 patients with factor X below 50% (24-hour urinary protein excretion ranging from 3.8 to 16.8 g).…”

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

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“…This deficiency has been attributed to adsorption of factor X selectively by amyloid fibrils [4,10,11], and it can be reversed by reduction of the amyloid burden with splenectomy [12,13] or by following successful treatment with HDM/SCT [9]. Deficiency of factors II [14],V [15,16], IX [14], and XIII [17] have also been reported. Over a 4-year-period, four patients with AL amyloidosis presented to Boston Medical Center with hemorrhage and a prolonged activated partial thromboplastin time (aPTT) but no detectable clotting factor deficiency.…”

Section: Introductionmentioning

confidence: 99%

Association of acquired von Willebrand syndrome with AL amyloidosis

Kos¹,

Ward²,

Malek³

et al. 2007

American J Hematol

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Acquired loss of functional von Willebrand factor (VWF) has been termed the acquired von Willebrand syndrome (AVWS). AVWS is a rare adult-onset bleeding diathesis that is clinically similar to congenital von Willebrand disease (VWD), and occurs with a variety of autoimmune, lymphoproliferative, or myeloproliferative disorders. We have identified four patients with AVWS in association with immunoglobulin light chain (AL) amyloidosis. These patients, lacking any pre-existing or family history of abnormal bleeding, developed cutaneous, mucosal, or gastrointestinal bleeding in the course of their disease without deficiency of clotting factor X or other factors; the activated partial thromboplastin time (aPTT) was prolonged in three out of the four cases. Despite normal VWF antigen levels, VWF ristocetin cofactor activity (VWF:RCo) was low. Electrophoresis patterns of high molecular weight (HMW) VWF multimers were abnormal in two of the four cases. Two of the patients were treated with high-dose intravenous melphalan followed by autologous stem cell transplantation (HDM/SCT) and achieved hematologic remission. In these two patients, the bleeding diathesis improved and the coagulation parameters normalized, confirming a causal relationship between the plasma cell dyscrasia and the AVWS. AVWS should be considered in AL amyloidosis patients with hemorrhagic diatheses and normal clotting factor levels.

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“…Two of these 17 reported patients had Factor X deficiency. Various acquired deficiencies of coagulation factors have been reported in AL amyloidosis, Factor X deficiency being the most common [29,30]. We have reported that serum Factor X deficiency (<25%) was documented in 8.7% of patients with AL amyloidosis evaluated at our center [31].…”

Section: Discussionmentioning

confidence: 75%

Spontaneous rupture of the spleen in AL amyloidosis

et al. 2003

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The frequency of splenic involvement in AL amyloidosis is not precisely known. However, splenomegaly has been reported in 4-13% of patients. We report four cases of spontaneous splenic rupture in patients with AL amyloidosis. Splenic rupture was the initial manifestation of the disease in one of these patients. The other three experienced splenic rupture during or after high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM/SCT): one during stem cell mobilization with G-CSF prior to HDM/SCT and two after hematopoietic recovery following treatment. Two of the four patients had Factor X deficiency, the most common coagulation abnormality associated with AL amyloidosis. All four patients underwent splenectomy without significant postoperative complications. Splenic rupture in AL amyloidosis as a complication of aggressive treatment with HDM/SCT has not been reported previously. Am.

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AL Amyloidosis Combined with Acquired Factor V Deficiency

Cited by 20 publications

References 2 publications

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

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

Association of acquired von Willebrand syndrome with AL amyloidosis

Spontaneous rupture of the spleen in AL amyloidosis

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