Multipe Myeloma and Related Disorders Flashcards
What are the 5 different amylodoses?
- AL (immunoglobulin light-chain) amyloidosis
- Hereditary amyloidosis
- AA (secondary) amyloidosis
- Age-related (senile) amyloidosis
- Dialysis-related anyloidosis
AL amyloidosis
What is the amyloid protein?
Monoclonal free λ or κ chains
Hereditary amyloidosis
What is the amyloid protein?
Mutated thransthyretin (TTR), fibrinogen alpha chain
AA amyloidosis
What are the diseases associated with?
RA, IBD, familial mediterrancea fever, chronic infection
AA amyloidosis
What is the amyloid protein?
Seru amyloid A protein
Dialysis-related amyloidosis
What is the amyloid protein?
Beta-2 microglobulin
AL amyloidosis
What diseases are associated with?
MGUS
MM
Waldenstrom macroglobulinemia (rare)
Waldenstrom Macroglobulinemia
Where does it originate from?
B-cell NHL, production of an IgM λ or κ M protein
Waldenstrom Macroglobulinemia
Dx
- Neoplastic infiltrate consisting of clonal lymphocytes, plasmacytoid lymphocytes, plasma cells, and immunoblasts comprising 10% or more of the bone marrow cellularity;
- M protein >=3g/dL
- Symptomatic
Smoldering Waldenstrom Macroglobulinemia
Dx
- Neoplastic infiltrate consisting of clonal lymphocytes, plasmacytoid lymphocytes, plasma cells, and immunoblasts comprising 10% or more of the bone marrow cellularity;
OR 2. M protein >=3g/dL - Asymptomatic
Waldenstrom Macroglobulinemia
Sx:
“B symptoms”
Bleeding diathesis;
Hyperviscosity: HA, blurry vision, dizziness, tinnitus, hearing loss;
Sensorimotor polyneuropathy
Waldenstrom Macroglobulinemia
Why bleeding diathesis?
- Hyperviscosity;
- Plt dysfunction
- Hyperfibrinogenemia
Waldenstrom Macroglobulinemia
What the fundoscopy findings?
Retinal vein dilation;
Papilledema;
Flame hemorrhage
Waldenstrom Macroglobulinemia
What test to assess for prognosis?
Beta-2 microblogulin
Albumin
LDH
Waldenstrom Macroglobulinemia
Rx for hyperviscosity
If hyperviscosity: immediate plasmapheresis
Waldenstrom Macroglobulinemia
Rx
Inital: rituximab monotherapy or with chemo (either cyclophosphamide, bendamustine) or a purine analog (fludarabine, cladribine) with/w/o steroids
HSCT
Waldenstrom Macroglobulinemia
Why sensorimotor polyneuropathy?
Antimyelin-associated glycoprotein acticity of the M protein
Cryoglobulinemia
What are the 3 types?
Type I:
Type II and III:mixed
Cryoglobulinemia
What are the disease associated with type I cryoglobulineia?
Underlying plasma cell dyscrasias or lymphoproliferative disorder (B cell NHL)
Cryoglobulinemia
What are the disease associated with type II and III cryoglobulinemia?
Chronic infection
Autoimmunedisease
Underlying plasma cell dyscrasias or lymphoproliferative disorder (B cell NHL)
Cryoglobulinemia
What is the most common disease associated with type II and III cryoglobulinemia?
Hepatitis C
Cryoglobulinemia
Type I characteristics:
Monoclonal immunoglobulin (lgG, lgM, or lgA) with no RF activity
Cryoglobulinemia
Type II characteristics:
Polyclonal immunoglobulins + monoclonal immunoglobulin (lgM, lgA) with RF activity
Cryoglobulinemia
Type III characteristics:
Polyclonal immunoglobulins with RF activity