[Primary amyloidosis associated to severe factor X deficiency].
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.
Pérez Martínez
J
J
Servicio de Nefrología, Complejo Hospitalario Universitario de Albacete. drjuanpm@yahoo.es
Llamas
F
F
López Montes
A
A
Massó
P
P
Poblet
E
E
López Rubio
E
E
Gallego
E
E
Gómez Roldán
C
C
D000686:Amyloidosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005171:Factor X Deficiency / Q000150:complications* / Q000175:diagnosis; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D007668:Kidney / Q000473:pathology*; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
D018906:Antineoplastic Agents, Alkylating; D005938:Glucocorticoids; D007146:Immunoglobulin lambda-Chains; D008558:Melphalan; D011241:Prednisone