Localized immunoglobulin light chain amyloidosis: Novel insights including prognostic factors for local progression

Autor: Michael Kreuter, Tobias Dittrich, Carsten Müller-Tidow, Marco Basset, Christoph Kimmich, Kaya Veelken, Stefan Schönland, Simone Brandelik, Ute Hegenbart, Christoph Röcken, Nikolaus Bosch, Jessica C. Hassel, Kamal Hummedah, Christiane Stuhlmann-Laeisz, Norbert Blank
Rok vydání: 2020
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Zdroj: American journal of hematologyREFERENCES.
ISSN: 1096-8652
Popis: In localized light chain amyloidosis (locAL), amyloidogenic light chains (aLC) are produced and deposited locally by a B-cell clone. We present 293 patients with immunohistochemically confirmed locAL. Lung (nodular pulmonary) with 63 patients was the most involved organ. The aLC was λ in 217 cases (κ:λ ratio 1:3). A local B-cell clone was identified in 30% of cases. Sixty-one (21%) had a concomitant autoimmune disorder (cAD). A monoclonal component (MC) were present in 101 (34%) patients and were more frequent in subjects with cAD (51% vs 34%; P = .03). Cigarette smoking was more prevalent in lung locAL (54% vs 37%; P = .018). After a median follow-up of 44 months, 16 patients died and 5- and 10-years locAL progression-free survival (PFS) were 62% and 44%. Interestingly, locAL-PFS was shorter among patients with an identified clonal infiltrate at amyloid deposition site (40 vs 109 months; P = .02) and multinuclear giant cells and/or an inflammatory infiltrate resulted in longer locAL-PFS in lung involvement (65 vs 42 months; P = .01). However, no differences in locAL PFS were observed in patients with cAD, a MC and involved organ site. Treatment was administered in 163 (54%) patients and was surgical in 135 (46%). Median locAL-PFS after first treatment was 56 months. Responders had longer locAL-PFS (78 vs 17 months; P
Databáze: OpenAIRE
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