Multidisciplinary supportive care in systemic light chain amyloidosis

Abstract

The immunoglobulin light-chain amyloidosis is a multisystemic disease which manifests by damage to the vital organs by light chain-derived amyloid fibril. Traditionally, the treatment has been directed to the underlying plasma cell clone with or without high dose chemotherapy followed by autologous stem cell transplantation using melphalan based conditioning. Now with the approval of highly tolerable anti-CD38 monoclonal antibody daratumumab based anti-plasma cell therapy in 2021, high rates of hematologic complete responses are possible even in patients who are otherwise deemed not a candidate for autologous stem cell transplantation. However, despite the progress, there remains a limitation in the strategies to improve symptoms particularly in patients with advanced cardiac involvement, those with nephrotic syndrome and autonomic dysfunction due to underlying systemic AL amyloidosis. The symptoms can be an ordeal for the patients and their caregivers and effective strategies are urgently needed to address them. The supportive care is aimed to counteract the symptoms of the disease and the effects of the treatment on involved organs' function and preserve patients' quality of life. Here we discuss multidisciplinary approach in a system-based fashion to address the symptom management in this dreadful disease. In addition to achieving excellent anti-plasma cell disease control, using treatment directed to remove amyloid from the vital organs can theoretically hasten recovery of the involved organs thereby improving symptoms at a faster pace. Ongoing phase III clinical trials of CAEL-101 and Birtamimab will address this question.

Keywords: Amyloidosis; Hematology; Light chain; Multidisciplinary approach; Supportive care.

Fig. 1

Perivascular amyloid deposits demonstrated as apple green birefringence under polarized light in this Congo Red stain specimen from a patient with systemic AL amyloidosis due to lymphoplasmacytic lymphoma (Bone marrow Congo Red, ×20).

Fig. 2

An electrocardiogram (EKG) showing pseudo-infarction pattern or Q-waves in chest Leads V1-V3 in this 62-year-old man presenting with AL cardiomyopathy from underlying clonal plasma cell neoplasms. Both limb and chest lead also demonstrate presence of low voltage.

Fig. 3

One of the cutaneous manifestations of systemic AL amyloidosis include amyloid purpura. Amyloid purpura is non-blanching purpuric relapsing and remitting skin lesions usually involving the peri-orbital skin, the upper trunk and neck. The purpura can break and cause oozing, skin breakdown and significant pain. A marked thinning of skin and skin breakdown needing local care (for oozing and bleeding) is seen in the picture.