AL amyloidosis with a localized B cell neoplasia

Autor: Ute Hegenbart, Ilske Oschlies, Sandra Krüger, Julius-Valentin Baumgart, Christoph Röcken, Christiane Stuhlmann-Laeisz, Stefan Schönland
Rok vydání: 2018
Předmět:
0301 basic medicine
Adult
Male
Pathology
medicine.medical_specialty
Lymphoma
B-Cell
Population
DNA Mutational Analysis
Plasma Cells
Gene Rearrangement
B-Lymphocyte
Heavy Chain
Biology
Plasma cell
Immunoglobulin light chain
Pathology and Forensic Medicine
03 medical and health sciences
Immunoglobulin kappa-Chains
0302 clinical medicine
Lymphoplasmacytic Infiltrate
Immunoglobulin lambda-Chains
medicine
AL amyloidosis
Biomarkers
Tumor
Gene Rearrangement
B-Lymphocyte
Light Chain
Humans
Immunoglobulin Light-chain Amyloidosis
Prospective Studies
education
Molecular Biology
B cell
Aged
Aged
80 and over
education.field_of_study
B-Lymphocytes
Amyloidosis
Cell Biology
General Medicine
Middle Aged
medicine.disease
Immunohistochemistry
030104 developmental biology
medicine.anatomical_structure
Phenotype
030220 oncology & carcinogenesis
Mutation
Myeloid Differentiation Factor 88
Female
Waldenstrom Macroglobulinemia
IGHV@
Immunoglobulin Heavy Chains
Zdroj: Virchows Archiv : an international journal of pathology. 474(3)
ISSN: 1432-2307
Popis: Immunoglobulin light chain-derived (AL) amyloidosis may occur as a systemic disease usually with dismal prognosis and a localized variant with favorable outcome. We report 29 patients with AL amyloidosis and associated lymphoplasmacytic infiltrate spatially related to amyloid deposits. In 17 cases, the amyloid deposits were classified as ALλ and 12 as ALκ Histopathology in all cases showed relatively sparse plasma cells and B cells without tumor or sheet formation by the lymphoplasmacytic infiltrate. The B cells predominantly showed an immunophenotype of the marginal zone. In situ, hybridization revealed 17 cases with λ− and 10 with κ light chain restricted plasma cells, which was concordant with the AL subtype in each case. Clonal immunoglobulin heavy variable gene (IGHV) or κ light chain rearrangement was found in 23/29 interpretable cases. A single case harbored a MYD88L265P-mutation. Taken together, we detected 27 (93%) cases of AL amyloidosis with an associated light chain restricted and predominantly molecularly clonal plasma cell population. Clinical data were available in 18 patients. Five patients suffered from systemic lymphoma and two from systemic AL amyloidosis. The remaining cases were classified as localized with regard to both, the AL amyloidosis and the light chain restricted plasma cell population. To the best of our knowledge, we herein present the largest cohort of AL amyloidosis associated with a light chain restricted and predominantly molecularly clonal plasma cell population, which we designate as a distinct disease entity: “AL amyloidosis with a localized B cell neoplasia of undetermined significance”.
Databáze: OpenAIRE
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