title: Systemic immunoglobulin light-chain amyloidosis (AL) in Mexico: a single institution, 30-year experience.
abstract: METHODS
  In a 30-year period in a single institution, 23 cases of systemic immunoglobulin light chain amyloidosis (AL) were identified, within a group of 1,388 individuals with some form of a hematological malignancy.
  
  
  RESULTS
  AL is 14 times less frequent in Mexico than in Caucasians and it represents 15% of all monoclonal gammopathies. Median age was 57 years (range 39-98); there were 11 males and 12 females. The histologic diagnosis was done in the periumbilical fat in 39%, the bone marrow in 30%, the kidney in 13%, the gastrointestinal tract in 13% and in a lymph node in one case. The nephrotic syndrome was present in 61% of cases, heart failure in 35%, sensorimotor peripheral neuropathy in 26% and weight loss in 6%. Anemia was present in 14% of cases at diagnosis; median hemoglobin was 11 g/dL. An abnormal monoclonal spike in the peripheral blood was present in 70% of cases; it had a median of 1.2 g/dL (range 0.2-3.6); there were 7 cases of light-chain only disease and five in whom an abnormal paraproteinemia was not found. Six cases were associated with overt multiple myeloma. Seventeen individuals (74%) were followed for more than 3 months (range 90 to 5190 days, median 210); their overall survival (OS) was 71% at 173 months, whereas the median OS has not been reached, being above 173 months. Eight patients were treated with melphalan/predisone and five were given high dose chemotherapy and an autologous stem cell transplantation; the others were given other treatments.
  
  
  CONCLUSIONS
  AL is less frequent in Mexican mestizos and probably underrecognized; the clinical features of the disease are not significantly different from those informed from other populations.
authors.affiliation: Centro de Hematología y Medicina Interna de Puebla.
authors.forename: Jesús
authors.initials: J
authors.lastname: Hernández-Reyes
authors.forename: Evelyn
authors.initials: E
authors.lastname: Galo-Hooker
authors.forename: Guillermo J
authors.initials: GJ
authors.lastname: Ruiz-Delgado
authors.forename: Guillermo J
authors.initials: GJ
authors.lastname: Ruiz-Argüelles
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058227:Amyloidogenic Proteins / Q000032:analysis; D000686:Amyloidosis / Q000453:epidemiology* / Q000208:ethnology / Q000209:etiology / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003907:Dexamethasone / Q000008:administration & dosage; D005006:Ethnicity / Q000706:statistics & numerical data; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000706:statistics & numerical data; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D008800:Mexico / Q000453:epidemiology; D008875:Middle Aged; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000150:complications / Q000453:epidemiology; D009101:Multiple Myeloma / Q000150:complications / Q000401:mortality / Q000628:therapy; D009194:Myeloma Proteins / Q000032:analysis; D009928:Organ Specificity; D011241:Prednisone / Q000008:administration & dosage; D011446:Prospective Studies; D011719:Pyrazines / Q000008:administration & dosage; D013792:Thalidomide / Q000008:administration & dosage; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000150:complications / Q000453:epidemiology
chemical_list: D058227:Amyloidogenic Proteins; D001897:Boronic Acids; D007147:Immunoglobulin Light Chains; D009194:Myeloma Proteins; D011719:Pyrazines; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone; D008558:Melphalan; D011241:Prednisone