Surgical removal of amyloid-laden lymph nodes: a possible therapeutic approach in a primary systemic AL amyloidosis patient with focal lymphadenopathy

Abstract

We report a patient with primary systemic AL amyloidosis who suffered from remarkable bilateral cervical lymphadenopathy. Intensive chemotherapies, including 2 cycles of high-dose melphalan with autologous peripheral blood stem cell transplantation, were insufficiently effective for both the lymphadenopathy and amyloidogenic IgGλ−type M-protein in serum, but the patient showed complete hematological remission after extensive surgical removal of enlarged lymph nodes that had massive depositions of λ-type immunoglobulin light chain-derived amyloid. Lymphadenectomy may be a possible therapeutic approach with regard to both cosmetic and hematological aspects in primary systemic AL amyloidosis patients with focal lymphadenopathy Lymphadenectomy in AL Matsuda M, et al.3

Clinical Manifestations at Diagnosis in Japanese Patients with Systemic AL Amyloidosis: A Retrospective Study of 202 Cases with a Special Attention to Uncommon Symptoms

“…4) showing remarkable uptake in one patient on positron emission tomography ( PET ) using 18 Ffluorodeoxyglucose (FDG) (20). Histopathology of the lymph nodes demonstrated massive deposition of amyloid with no obvious evidence suggestive of lymphoproliferative disorders (22). On immunohistochemistry, these amyloid deposits were positive for ALλ and ALκ in four and two patients, respectively.…”

Plasmacytoma and Amyloid Lymphadenopathy, FDG, and Fluorocholine

“…Recently, other PET radiopharmaceuticals, choline and methionine, have shown to be a valuable asset to evaluate plasma cell disorders 7,8 . Treatment options for amyloid lymphadenopathies may differ from the source of the systemic amyloid disease, which can include debulking surgery and also external radiation therapy apart from chemotherapy 9 . Differentiating the plasmacytoma and its complications like amyloid depositions at distant places may change the treatment plans.…”

“…7,8 Treatment options for amyloid lymphadenopathies may differ from the source of the systemic amyloid disease, which can include debulking surgery and also external radiation therapy apart from chemotherapy. 9 Differentiating the plasmacytoma and its complications like amyloid depositions at distant places may change the treatment plans. Here we present the importance of ability to point the lesions of interest via FDG and differentiating the natures of these lesions via FCH.…”