Tags

Type your tag names separated by a space and hit enter

Immunoglobulin light chain amyloidosis: 2013 update on diagnosis, prognosis, and treatment.
Am J Hematol. 2013 May; 88(5):416-25.AJ

Abstract

DISEASE OVERVIEW

Immunoglobulin (Ig) light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of Ig light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/autonomic neuropathy, and atypical multiple myeloma.

DIAGNOSIS

Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for diagnosis. Invasive organ biopsy is not required because amyloid deposits can be found in bone marrow biopsy or subcutaneous fat aspirate in 85% of patients. Verification that amyloid is of immunoglobulin origin is mandatory.

PROGNOSIS

N-terminal pro-brain natriuretic peptide (NT-proBNP), serum troponin T, and immunoglobulin free light chain values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months.

THERAPY

All patients with a visceral amyloid syndrome require therapy to prevent deposition of amyloid in other viscera and prevent progressive organ failure of involved sites. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include NT-proBNP <5,000 ng/mL, troponin T < 0.06 ng/mL, age <70 years, <3 organs involved, and serum creatinine ≤1.7 mg/dL. Nontransplant candidates can be offered melphalan-dexamethasone. Pomalidomide appears to have activity, as do other combinations of chemotherapy with agents such as cyclophosphamide-thalidomide (or lenalidomide or bortezomib)-dexamethasone, bortezomib-dexamethasone, and melphalan-prednisone-lenalidomide.

FUTURE CHALLENGES

Late diagnosis remains a major obstacle to initiating effective therapy when organ dysfunction is still recoverable. Recognizing the presenting syndromes is necessary for improving survival.

Authors+Show Affiliations

Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA. gertz.morie@mayo.edu

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

23605846

Citation

Gertz, Morie A.. "Immunoglobulin Light Chain Amyloidosis: 2013 Update On Diagnosis, Prognosis, and Treatment." American Journal of Hematology, vol. 88, no. 5, 2013, pp. 416-25.
Gertz MA. Immunoglobulin light chain amyloidosis: 2013 update on diagnosis, prognosis, and treatment. Am J Hematol. 2013;88(5):416-25.
Gertz, M. A. (2013). Immunoglobulin light chain amyloidosis: 2013 update on diagnosis, prognosis, and treatment. American Journal of Hematology, 88(5), 416-25. https://doi.org/10.1002/ajh.23400
Gertz MA. Immunoglobulin Light Chain Amyloidosis: 2013 Update On Diagnosis, Prognosis, and Treatment. Am J Hematol. 2013;88(5):416-25. PubMed PMID: 23605846.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Immunoglobulin light chain amyloidosis: 2013 update on diagnosis, prognosis, and treatment. A1 - Gertz,Morie A, PY - 2013/01/14/received PY - 2013/01/16/accepted PY - 2013/4/23/entrez PY - 2013/4/23/pubmed PY - 2013/6/20/medline SP - 416 EP - 25 JF - American journal of hematology JO - Am J Hematol VL - 88 IS - 5 N2 - DISEASE OVERVIEW: Immunoglobulin (Ig) light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of Ig light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/autonomic neuropathy, and atypical multiple myeloma. DIAGNOSIS: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for diagnosis. Invasive organ biopsy is not required because amyloid deposits can be found in bone marrow biopsy or subcutaneous fat aspirate in 85% of patients. Verification that amyloid is of immunoglobulin origin is mandatory. PROGNOSIS: N-terminal pro-brain natriuretic peptide (NT-proBNP), serum troponin T, and immunoglobulin free light chain values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months. THERAPY: All patients with a visceral amyloid syndrome require therapy to prevent deposition of amyloid in other viscera and prevent progressive organ failure of involved sites. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include NT-proBNP <5,000 ng/mL, troponin T < 0.06 ng/mL, age <70 years, <3 organs involved, and serum creatinine ≤1.7 mg/dL. Nontransplant candidates can be offered melphalan-dexamethasone. Pomalidomide appears to have activity, as do other combinations of chemotherapy with agents such as cyclophosphamide-thalidomide (or lenalidomide or bortezomib)-dexamethasone, bortezomib-dexamethasone, and melphalan-prednisone-lenalidomide. FUTURE CHALLENGES: Late diagnosis remains a major obstacle to initiating effective therapy when organ dysfunction is still recoverable. Recognizing the presenting syndromes is necessary for improving survival. SN - 1096-8652 UR - https://www.unboundmedicine.com/medline/citation/23605846/Immunoglobulin_light_chain_amyloidosis:_2013_update_on_diagnosis_prognosis_and_treatment_ L2 - https://doi.org/10.1002/ajh.23400 DB - PRIME DP - Unbound Medicine ER -