Cutaneous light chain amyloidosis with multiple myeloma: A concise review

Andrei, Mirela; Wang, Jen C.

doi:10.1016/j.hemonc.2018.09.003

Cited by 26 publications

(35 citation statements)

References 48 publications

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“…Patients with lung locAL are usually older, 12 and skin and ST locAL are more common in women. 16 A cAD and a MC and/or abFLCR are particularly common in these patients. In general, prognosis of locAL is good, however, a worse survival is observed in patients with lung locAL.…”

Section: Discussionmentioning

confidence: 96%

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

et al. 2020

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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 < .001). Three patients with lung locAL and a MC were diagnosed as systemic AL amyloidosis at follow‐up. In summary, locAL pathogenesis seems to be heterogeneous and the clonal infiltrate leads local progression.

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Section: Discussionmentioning

confidence: 96%

“…In another patient, amyloid deposits were found in nasopharynx (respiratory involvement) and in a lateral cervical lymph node (lymphatic tissue involvement). 1 141 146 (86) / 1 (16) 2 (29) 100 / 74…”

Section: Overall Survival From Diagnosismentioning

confidence: 99%

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

et al. 2020

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“…Organs most commonly affected include kidney, heart, liver, nerves, and gastrointestinal tract, but any organ or tissue can be affected, including the skin. The condition, alone or in conjunction with underlying plasma cell dyscrasias, including MM, is commonly diagnosed when patients are in their 60s 5 …”

Section: Discussionmentioning

confidence: 99%

“…Skin findings secondary to myeloma-associated AL amyloidosis are variable in presentation, with bullae (56%) being the most commonly reported lesion, followed by purpura/ecchymoses (25%) and nodules/papules (16%); a few cases of scleroderma, macroglossia, alopecia, nail dystrophy, and condyloma have also been reported 5 . Nodular and papular lesions, seen in our patient, are caused by direct dermal infiltration of amyloid; they classically appear yellow, shiny, smooth, waxy, and often hemorrhagic and are found on flexor surfaces, face, neck, or buccal mucosa 4 .…”

Section: Discussionmentioning

confidence: 99%

“…Although there has been progress made in the treatment of patients with MM, management approaches for AL amyloidosis associated with MM are generally not well defined. Prognosis for patients of myeloma-associated AL amyloidosis is poor, with reported median survival as short as 4 months to 5 years 5 . Prognosis of patients with skin involvement is even worse, potentially reflecting extensive systemic involvement 5 …”

Section: Discussionmentioning

confidence: 99%

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