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Cutaneous light chain amyloidosis with multiple myeloma: A concise review.
Hematol Oncol Stem Cell Ther. 2019 Jun; 12(2):71-81.HO

Abstract

OBJECTIVE/BACKGROUND

Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes.

METHODS

An electronic search of the PubMed database was performed to obtain key literature in AL amyloidosis and MM, using the following search terms: multiple myeloma, immunoglobulin light chain amyloidosis, and cutaneous amyloidosis. The search results were narrowed by selecting studies in English. Results were confined to the following articles types: case reports, case series, and systematic reviews.

RESULTS

We identified 32 cases from the PubMed database search and examined their potential relevance. We found the following: (a) higher prevalence in women (two-thirds) and white population; (b) IgG and IgA were equally distributed with lambda (λ) light chain occurring in 53-66% of cases; (c) majority of cases (56%) presented as hemorrhagic bullous lesions, followed by purpura/ecchymosis in 25% of cases; and (d) majority (64%) died within 6 months since diagnosis.

CONCLUSIONS

We reviewed the constellation of the cutaneous manifestations of AL amyloidosis with concurrent MM. We found a female predominance, and more than half presented as hemorrhagic bullous lesions. There is a preponderance of λ light chains over kappa (κ) light chains, both as a free light chain (15% vs. 4%) and as an intact Ig (38% vs. 24%; absolute number of 14 vs. 7 patients, respectively). In the subgroup of patients with bullous skin lesions, λ light chain was present in eight cases and κ light chain in seven cases. All κ light chain subtypes presented with bullous lesions and no other cutaneous types of lesions. They carried very poor prognosis with majority of cases surviving only 6 months, much worse than overall patients with AL amyloidosis without myeloma or myeloma without amyloidosis.

Authors+Show Affiliations

Division of Hematology & Oncology, Saint Mary's Regional Medical Center, Lewiston, ME, USA.Division of Hematology & Oncology, Brookdale University Hospital Medical Center, Brooklyn, NY, USA. Electronic address: jcwang0005@gmail.com.

Pub Type(s)

Journal Article
Systematic Review

Language

eng

PubMed ID

30261180

Citation

Andrei, Mirela, and Jen Chin Wang. "Cutaneous Light Chain Amyloidosis With Multiple Myeloma: a Concise Review." Hematology/oncology and Stem Cell Therapy, vol. 12, no. 2, 2019, pp. 71-81.
Andrei M, Wang JC. Cutaneous light chain amyloidosis with multiple myeloma: A concise review. Hematol Oncol Stem Cell Ther. 2019;12(2):71-81.
Andrei, M., & Wang, J. C. (2019). Cutaneous light chain amyloidosis with multiple myeloma: A concise review. Hematology/oncology and Stem Cell Therapy, 12(2), 71-81. https://doi.org/10.1016/j.hemonc.2018.09.003
Andrei M, Wang JC. Cutaneous Light Chain Amyloidosis With Multiple Myeloma: a Concise Review. Hematol Oncol Stem Cell Ther. 2019;12(2):71-81. PubMed PMID: 30261180.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cutaneous light chain amyloidosis with multiple myeloma: A concise review. AU - Andrei,Mirela, AU - Wang,Jen Chin, Y1 - 2018/09/21/ PY - 2018/01/14/received PY - 2018/07/09/revised PY - 2018/09/03/accepted PY - 2018/9/28/pubmed PY - 2018/9/28/medline PY - 2018/9/28/entrez KW - Cutaneous amyloidosis KW - Immunoglobulin light-chain amyloidosis KW - Multiple myeloma SP - 71 EP - 81 JF - Hematology/oncology and stem cell therapy JO - Hematol Oncol Stem Cell Ther VL - 12 IS - 2 N2 - OBJECTIVE/BACKGROUND: Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes. METHODS: An electronic search of the PubMed database was performed to obtain key literature in AL amyloidosis and MM, using the following search terms: multiple myeloma, immunoglobulin light chain amyloidosis, and cutaneous amyloidosis. The search results were narrowed by selecting studies in English. Results were confined to the following articles types: case reports, case series, and systematic reviews. RESULTS: We identified 32 cases from the PubMed database search and examined their potential relevance. We found the following: (a) higher prevalence in women (two-thirds) and white population; (b) IgG and IgA were equally distributed with lambda (λ) light chain occurring in 53-66% of cases; (c) majority of cases (56%) presented as hemorrhagic bullous lesions, followed by purpura/ecchymosis in 25% of cases; and (d) majority (64%) died within 6 months since diagnosis. CONCLUSIONS: We reviewed the constellation of the cutaneous manifestations of AL amyloidosis with concurrent MM. We found a female predominance, and more than half presented as hemorrhagic bullous lesions. There is a preponderance of λ light chains over kappa (κ) light chains, both as a free light chain (15% vs. 4%) and as an intact Ig (38% vs. 24%; absolute number of 14 vs. 7 patients, respectively). In the subgroup of patients with bullous skin lesions, λ light chain was present in eight cases and κ light chain in seven cases. All κ light chain subtypes presented with bullous lesions and no other cutaneous types of lesions. They carried very poor prognosis with majority of cases surviving only 6 months, much worse than overall patients with AL amyloidosis without myeloma or myeloma without amyloidosis. SN - 1658-3876 UR - https://www.unboundmedicine.com/medline/citation/30261180/Cutaneous_light_chain_amyloidosis_with_multiple_myeloma:_A_concise_review_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1658-3876(18)30095-5 DB - PRIME DP - Unbound Medicine ER -