[Primary amyloidosis associated to severe factor X deficiency]. / Amiloidosis primaria (AL) asociada a déficit severo de factor X.
Nefrologia
; 24(5): 493-8, 2004.
Article
em Es
| MEDLINE
| ID: mdl-15648909
ABSTRACT
Amyloidosis is a systemic disease characterized by generalized deposition of beta-organized proteic fibrillar material with green birefringence under polarized light, in different tissues and organs, the most frequent kidney, liver and heart, with important clinical repercussion. Primary or AL amyloidosis is the most common subtype of amyloidosis (1), confirmed by biopsy-proved amyloid deposition in abdominal fat pad, rectum, kidney or liver, if necessary, in which fragments of monoclonal light chains are deposited. Cases with factor X (Stuart factor) of coagulation deficiency associated are described, due to adsorption of this factor to amyloid fibrills. Normally, evolution is fatal, with only few months of survival. We report a case of primary amyloidosis with nephrotic syndrome, severe factor X deficiency (without bleeding complications), possible heart affection and short-term good response to chemotherapic treatment.
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Base de dados:
MEDLINE
Assunto principal:
Deficiência do Fator X
/
Amiloidose
/
Rim
/
Síndrome Nefrótica
Tipo de estudo:
Diagnostic_studies
/
Risk_factors_studies
Limite:
Humans
/
Male
/
Middle aged
Idioma:
Es
Ano de publicação:
2004
Tipo de documento:
Article