AL Management

Targeted treatments

AL amyloidosis is a clonal plasma cell disorder that is characterized by multi-organ dysfunction as a result of deposition of immunoglobulin monoclonal light chain–derived amyloid fibrils, despite a low burden of plasma cells.

The three principles of treatment of AL amyloidosis are:

  • to cause rapid, deep and sustained reduction of amyloidogenic light chains;
  • to individualize therapy on the basis of extent and degree of organ involvement;
  • to provide optimal organ-specific supportive care in order to minimize treatment-related morbidity and mortality and maximize quality of life.

Because of the complexity, multidisciplinary nature and rarity of this disease, referral of patients to centres that are highly experienced in the management of AL amyloidosis is strongly recommended. The list of AAN centers can be found here. Complex case discussions and teleconferences with the referring physician are an integral part of these multidisciplinary services. We welcome your participation at the monthly AAN Grand Rounds (insert link). Patient treatment can be conducted either in, or in close collaboration with, one of the AAN centers.

The therapeutic landscape of AL amyloidosis is rapidly expanding and we advocate for enrolment of patients in many clinical trials that are currently open in Australia. AAN centers can facilitate discussions around the enrolment process and ensure timely assessment of patients. Many clinical trials provide travel and accommodation support for regional patients.

For the comprehensive diagnosis, staging and management of AL amyloidosis in the Australian context, please refer to the Myeloma Scientific Advisory Group (MSAG) clinical practice guideline.

Treatment algorithm for some patients with systemic AL amyloidosis may involve high dose chemotherapy and stem cell transplantation. Expert guidelines from the International Society of Amyloidosis (ISA) and European Haematology Association (EHA) are available here.

Survival in AL amyloidosis depends upon rapid reduction of the pathological immunoglobulin free light chain and stabilization or recovery of organ function, especially cardiac function. Improvements in organ function can take many months to several years to occur.

Recommendations for haematological and organ response assessment can be found in the MSAG clinical practice guideline. The following articles can also be of interest.

Careful medical management of amyloidosis-related complications is essential for the improvement of patient quality of life and the achievement of organ response and is best undertaken within a multidisciplinary model incorporating multiple medical specialties, allied health and specialized nursing staff.

Further information on the supportive care recommendations can be found here.

HEALTH PROFESSIONALS LANDING PAGE

Overview  |  Amyloidosis for GPs  |  ATTR Management  |  Supportive Care