Diagnosis and management of AL amyloidosis due to B-cell non-Hodgkin lymphoma

Abstract

Immunoglobulin light chain (AL) amyloidosis may be caused by a B-cell non-Hodgkin lymphoma (NHL) rather than a plasma cell neoplasm in rare cases, which presents unique diagnostic and management considerations. NHL associated with AL will often have an IgM paraprotein; thus, this disease is termed IgM-related AL amyloidosis (IgM AL). The clinical presentation of IgM AL is more likely to involve the lungs, peripheral nerves, and soft tissue; cardiac involvement is less common. Patients with IgM AL amyloidosis should undergo a lymphoma-directed work-up including evaluation for nodal and extranodal disease. Additionally, patients with an IgM paraproteinemia should be screened for AL amyloidosis through history and physical examination. Treatment regimens active against underlying lymphoma, rather than plasma cell-directed regimens, are recommended. Historical response rates in IgM AL have been poor; prospective studies of novel antineoplastic regimens may improve treatment outcomes.

Amyloid Light-Chain (AL) Amyloidosis of the Trachea Associated With an Indolent B-cell Neoplasm

“…In our case, the tracheal amyloidosis was associated with a CD5-positive, undifferentiated, B-cell neoplasm. Non-Hodgkin lymphoma-associated AL can have an IgM paraprotein, but this was not observed in our patient [ 11 ].…”

“…Non-IgM-related AL with lymphoma is commonly associated with marginal zone/MALT lymphoma [ 4 , 7 , 8 ] but has also been associated with CLL/SLL [ 8 , 9 ], aggressive lymphomas such as diffuse large B-cell lymphoma [ 3 , 8 ] and localized B-cell neoplasia [ 10 ]. Non-IgM-associated AL may present with localized amyloid deposits at the lacrimal gland, breast, lung, stomach, and lymph nodes [ 4 , 11 ].…”

Sudden unexpected death after initial infusion of rituximab for Waldenström macroglobulinemia/lymphoplasmacytic lymphoma: an autopsy case

“…Approximately 10% of WM/LPL cases are accompanied by amyloid immunoglobulin (Ig) light chain (AL) amyloidosis [ 3 ]. When these conditions coexist, overall survival is significantly worse than that for WM/LPL alone [ 4 , 5 ]. Cardiac amyloidosis is a risk factor for sudden death [ 6 ], and, because AL amyloidosis frequently affects the heart [ 7 ], it is thought to be associated with unexpected sudden death among patients with WM/LPL.…”