the CD138+, CD38+, CD19−, CD4+/- phenotype) may be a more sensitive method to enumerate circulating clonal plasma cells and diagnose plasma cell leukemia.There are two forms of plasma cell leukemia: Primary plasma cell leukemia in which patients without a history of multiple myeloma present with diagnostically high levels of circulating plasma cells and Secondary plasma cell leukemia in which patients with multiple myeloma suffer their dyscrasias progression by the expansion of large numbers of their malignant plasma cells into the circulation and distant tissues. Historically, primary plasma cell leukemia was more common than the secondary form but with the increased survival of multiple myeloma patients due to new treatment regiments, more cases of secondary plasma cell leukemia are occurring; currently, the two forms occur in approximately equal numbers. Patients with primary plasma cell leukemia present with clinical findings that are less commonly found in multiple myeloma, e.g. they often have hepatomegaly, splenomegaly, lymphadenopathy, nerve and central nervous system defects, bleeding tendencies secondary to thrombocytopenia, and pleural effusions. They are less likely than multiple myeloma patients to have lytic bone lesions. In several studies of patients with either form of plasma cell leukemia, the disease was associated with clonal IgG in 28% to 56% of cases, IgA in 4% to 7% of cases, and a light chain in 23% to 44% of cases; 0-12% of patients had no myeloma protein. Medium survival for primary and secondary plasma cell dyscrasias have been 7–13 months and 2–7 months, respectively, but appear to be improving with new treatment regimens.
In a small study of 16 patients with renal amyloidosis, the 5 patients with AH amyloidosis and the 11 patients with AHL amyloidosis had less frequent concurrent cardiac involvement and better overall survival than 202 patients with renal AL amyloidosis. The hematological response to chemotherapy of the AH and AHL renal amyloidosis patients was comparable to those with renal AL amyloidosis. POEMS syndrome
POEMS syndrome (also known as Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare and complex medical syndrome that involves a combination of syndrome-defining signs and symptoms due to the dysfunction of multiple organs. The syndrome is associated with a plasma cell dyscrasia in almost 100% of cases, pathological overexpression of certain cytokines in >95% of cases, and the lymphoproliferative disorder termed Castlemans disease in ~15% of cases. (Rare cases of POEMS have been associated with polyclonal rather than clonal plasma cells; these cases are not plasma cell dyscrasias but rather appear to be caused by the over-activity of non-malignant immune cell responses in chronic infections or autoimmune diseases.) POEMS is an acronym standing for the characteristic signs or symptoms of the syndrome: Polyneuropathy, Organomegaly, Endocrinopathy, Plasma cell disorder (typically, the plasma cell burden is low in POEMS patients), and Skin changes (e.g. hemangioma, hyperpigmentation).