Systemic Light Chain Amyloidosis Clinical Practice Guidelines (NCCN, 2023)

The guideline update on primary systemic light chain amyloidosis (SLCA) was released in January 2023 by the National Comprehensive Cancer Network.^[1]

Diagnosis

Essential studies for diagnosis of SLCA are as follows:

• Serum immunofixation electrophoresis

• 24-hour urine immunofixation electrophoresis

• Serum free light chain (FLC) ratio analysis

After identification of FLCs in serum or urine, aspiration of abdominal subcutaneous fat and/or biopsy of the involved organs is necessary for pathologic evaluation to confirm amyloid in the tissue; confirmation is typically done with Congo red staining. Immunohistochemical methods, electron microscopy, or mass spectrometry are then used to confirm that the amyloid deposits are composed of light chains.

Subsequent workup is geared toward identifying the organs involved and the severity of organ involvement and assessment of the feasibility and safety of different treatment approaches.

Treatment

For primary therapy of SLCA, the preferred regimen is daratumumab and hyaluronidase in combination with bortezomib/cyclophosphamide/dexamethasone. Other recommended regimens are as follows:

• Bortezomib/cyclophosphamide/dexamethasone

• Bortezomib with or without dexamethasone

• Bortezomib/melphalan/dexamethasone (for patients ineligible for hematopoietic cell transplantation [HCT])

• Bortezomib/lenalidomide/dexamethasone

Oral melphalan/dexamethasone is an option for patients with SLCA who are not candidates for HCT.

For previously treated SLCA, treatment would depend on prior therapy received, patient preferences, and toxicity profile; repeating the initial therapy may be considered, especially if the patient has not had relapse of disease for several years. Regimens for use in relapsed SLCA include the following:

• Bortezomib with or without dexamethasone

• Bortezomib/cyclophosphamide/dexamethasone

• Bortezomib/melphalan/dexamethasone

• Daratumumab

• Ixazomib/dexamethasone

• Ixazomib/lenalidomide/dexamethasone

• Lenalidomide/cyclophosphamide/dexamethasone

• Lenalidomide/dexamethasone

• High-dose melphalan followed by HCT

• Melphalan/dexamethasone

• Pomalidomide/dexamethasone

The following regimens are useful for previously treated SLCA, in certain circumstances:

• Bendamustine/dexamethasone – For patients who have received multiple prior regimens

• Carfilzomib with or without dexamethasone – For non-cardiac SLCA

• Venetoclax with or without dexamethasone – For patients with t(11;14) SLCA

For more information, see Primary Systemic Amyloidosis. For more Clinical Practice Guidelines, please go to Guidelines.

Medscape © 2023 WebMD, LLC

Any views expressed above are the author's own and do not necessarily reflect the views of WebMD or Medscape.

Cite this: Systemic Light Chain Amyloidosis Clinical Practice Guidelines (NCCN, 2023) - Medscape - Mar 02, 2023.