Abstract
The survivorship needs of patients with light-chain (AL) amyloidosis are complex, as is the diagnosis and treatment itself. Early diagnosis is critical in improving patient outcomes; however, given the nonspecific nature of the symptoms, most patients with AL amyloidosis require evaluation by multiple specialists, resulting in significant delays in diagnosis of up to 3 years. An early and accurate diagnosis can help reduce the psychological toll of the patient’s journey to diagnosis. Given the high symptom burden and complex process of diagnosis, it is not surprising that patients with AL amyloidosis report worse health-related quality of life than the general population. Organ dysfunction associated with AL amyloidosis also may make the treatment directed towards plasma cell clone difficult to tolerate, leading to morbidity and mortality. Furthermore, supportive care requires an integrated, multidimensional and patient-centered approach to improve survival and feelings of well-being, as organ responses lag behind hematologic responses. The impact of AL amyloidosis is often devastating for the patient and may last beyond the effects of treatment. Future research is needed to study and assess the needs of survivors of AL amyloidosis utilizing valid, reliable and standardized measures.
Highlights
New therapeutics for AL amyloidosis were investigated at an accelerated tempo after 2010, including bortezomib-based regimens, ixazomib an oral proteasome inhibitor, next-generation immunomodulatory drugs, and anti-CD38 monoclonal antibodies [16,17,18,19,20,21,22]
Patients describe the emotional impact of diagnosis, which often includes experiencing fear, anxiety, and depression [26]
A subset of patients have renal/cardiac progression with normal monoclonal free light chain levels or after achievement of a very good partial hematologic response. It is not clear if there is a small amount of circulating amyloidogenic light chain below the detection level or if the worsening of renal/cardiac disease represents the progression of the original, irreversible organ damage
Summary
Millions of adults who survive cancer are relieved when the treatment ends but report a daunting, heavy, and exhausting transition to a new way of life. Hemato 2022, 3 production of amyloidogenic light-chain precursor proteins were used without much consideration for the underlying organ dysfunction These treatments could be associated with significant adverse events, and patients often died before experiencing any benefit [8]. New therapeutics for AL amyloidosis were investigated at an accelerated tempo after 2010, including bortezomib-based regimens, ixazomib an oral proteasome inhibitor, next-generation immunomodulatory drugs, and anti-CD38 monoclonal antibodies [16,17,18,19,20,21,22] Many of these granted long-term disease control in the relapsed/refractory setting [23,24]. The long-term outcomes of survival, disease progression, functional outcomes, fertility, and QOL, and the long-term complications of secondary malignancies, are important to focus on during survivorship
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