0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:17
1034 Myeloma and Gammopathies■Primary amyloidosis (AL): fibrils consisting of variable region of
immunoglobulin light chain in heart, tongue, gastrointestinal tract,
and skin; in 20% cases associated with myeloma
■Waldenstrom’s macroglobulinemia: elevated serum IgM, excess
bone marrow lymphoplasmacytoid cells, visceral but rare bone
involvement
■Non-Hodgkin’s lymphoma: lymph node, visceral, and/or bone mar-
row infiltration with monoclonal B cells, occasionally associated with
serum monoclonal protein
■Other lymphoproliferative syndromes: distinct histopathologymanagement
What to Do First
■Assess severity and pace of disease, complications, and candidacy
for treatment
General Measures
■Vigorous hydration
■Avoid nephrotoxic medications
specific therapy
Observation
■Systemic, indolent or smoldering myeloma, Stage I myeloma (low
level monoclonal protein and bone marrow plasmacytosis, no bone
disease, asymptomatic)
Conventional Chemotherapy and Supportive Care
■All other stages of myeloma
Conventional Therapy
■Melphalan/prednisone; dexamethasone; vincristine/doxorubicin/
dexamethasone; thalidomide/dexamethasone; other combination
chemotherapy
■All cytotoxic; equivalent response rates but responses more rapid to
combination chemotherapy
■Limit exposure to myelotoxic agents (including alkylating agents and
nitrosoureas) to avoid compromising stem cell reserve prior to stem
cell harvest in candidates for transplantHigh-Dose Therapy
■Autologous stem cell transplant for patients with adequate cardiac,
pulmonary, renal, and hepatic function; modest prolongation of sur-
vival, but no cures to date.
■Allogeneic transplantation in clinical trial