28.3. Guidelines for the diagnosis of AL amyloidosis

Chapter 28

28.3.1. International Myeloma Working Group (2009)

In the guidelines for sFLC analysis in MM and related disorders, the International Myeloma Working Group (IMWG) recommend a combination of sFLC analysis and immunofixation of serum and urine to screen for AL amyloidosis [167]. The guidelines also recommend that baseline sFLC measurements are obtained for all AL amyloidosis patients, as highly abnormal results have prognostic value (Section 28.4).

28.3.2. British Committee for Standards in Haematology (2015)

The British Committee for Standards in Haematology guidelines on the diagnosis and investigation of AL amyloidosis state that immunofixation of blood and urine and sFLCs should be measured in all patients with suspected AL amyloidosis [614]. They also recognise that Freelite sFLC assays are well established, whilst FLC assays from other manufacturers have not been validated (Chapter 8).

Clinical case history

AL amyloidosis identified by FLC analysis when electrophoretic tests were doubtful [615].

A 40-year-old woman, with spontaneous bruises, asthenia, abdominal pains and possible cardiomyopathy, was investigated for suspicion of AL amyloidosis. Abdominal fat biopsy showed Congo red positivity. SPE showed hypogammaglobulinaemia but no monoclonal proteins.

IFE showed a weak λ band without a corresponding intact immunoglobulin Figure 28.7. Quantitative immunoglobulin measurements were: IgG 4.9 g/L; IgA 1.02 g/L and IgM 0.32 g/L indicating hypogammaglobulinaemia. sFLC analysis showed: κ 7.8 mg/L; λ 210 mg/L and κ/λ ratio of 0.04. This supported a diagnosis of AL amyloidosis.

Nephelometric sFLC quantification was clearly abnormal and provided a measurable parameter for subsequent disease monitoring. In contrast, FLCs were barely detectable by conventional electrophoretic assays.

28.3.3 NCCN Clinical Practice Guidelines In Oncology (NCCN Guidelines®) for Systemic Light Chain Amyloidosis V.1.2019

The Guidelines developed by the National Comprehensive Cancer Network® (NCCN®) on the management of AL amyloidosis patients state that SPE, sIFE and uIFE should be performed in all patients with suspected AL amyloidosis [1211]. Additionally, they recognise sFLC analysis as a complementary test to the above mentioned methods.