10.        Myeloma and Related Conditions

10.2              primary systemic amyloidosis

  General considerations

Primary systemic amyloidosis is a rare plasma cell dyscrasia in which insoluble immunoglobulin light chain fragments are produced, They polymerize into fibrils that deposite extracellularly and cause dysfunction of visceral organs and death.

Primary systemic amyloidosis has many characteristics in common with multiple myeloma and, therefore, is treated similarly. Conventional-dose melphalan can prolong the median duration of survival , but clinical responses with improvement of impaired organ functions are rare and the response develops slowly, VAD (vincristine, doxorubicin, dexamethasone), or high-dose dexamethasone, or interferon alpha are also possible.

For selected patients (single organ involvement, age < 55 years, absence of renal insufficiency, no symptomatic cardiac dysfunction) myeloablative chemotherapy with autologous stem cell rescue may result in substanially better response rates than low-dose treatment . Transplantation-related morbidity and mortality, however , are clearly higher than in patients with MM.

Literature:

            COMENZO and GERTZ, Blood 99 (2002): 4276 – 4282 (review of

            autotransplantation)

DEMBER et al, Ann, Intern. Med. 134 (2001): 746- 753 (high-dose melphalan with stem cell rescue)

GERTZ and RAJKUMAR, Curr, Treat. Options Oncol. 3 (2002): 261 – 271 (review)

PALLADINI et al, Br. J. haematol. 113 (2001): 1044 – 1046 (high-dose dexamethasone)

SANCHORAWALA et al, Br. J. Haematol. 117 (2002): 886-889 (low-dose continuous oral melphalan)