Heme - Plasma Cell Dyscrasias - Multiple Myeloma, Mgus, Waldenstroms

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Types of plasma cell proliferation

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Small and brief

Small to modest but stable

Uncontrolled

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Most common heavy chains

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g then a then m

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Waldenstroms Macroglobulinemia

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Acts like an indolent lymphoma - It is of IgM origin.

Identical to lymphoplasmacytic lymphoma

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Plasma cell characteristics of a myeloma

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Post-germinal center

Immunoglobulin class switching and somatic hypermutation

Expressed CD38 and CD138

Gets cytogenetic changes along the way.

IL-6 is an important growth factor

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Progressive events in multiple myeloma

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Normal plasmablast --> MGUS --> Intramedullary myeloma --> extramedullary myeloma

Each transition needs a mutation

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Electrophoresis

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Albumin travels furthest

Ig doesnt go far at all. If very sharp high peak, there is a monoclonal type present.
If broad and large, it is chronic inflammation

If it was sharp, do electrophor to determine the heavy (G,M,A) and light (Kappa or lambda) type.

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Plasma cell proliferation can cause..

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Marrow replacement and bone lesions.

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Standard sx of MGUS

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CRAB

HyperCalcemia, renal failure, anemia, bone lesions.

(is this right?)

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MGUS

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Monoclonal gammopathy of uncertain significance.

This disease doesn't really cause much trouble but can progress to myeloma (at a rate of about 1 more % per year you have it)

Dx is when BM plasma cells are less than 10%.

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MGUS and myeloma more prevalent in...

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men.

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IgG subtype of MGUS

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less likely to progress to myeloma.

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Prognostic factors for MGUS going to myeloma.

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Abnormal FLC ratio

High serum M protein size?

IgA or IgM (bad)

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FLC ratio

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Kappa/lambda ratio

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Standard sx of myeloma

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CRAB

Low residual immunoglobulins

Infections

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Cells in myeloma (BM aspirate)

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Nucleus on one end, elongated oval cells.

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Bence-Jones proteins

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When kappa or lambda is seen in urine and low in serum. Because the light chains are small and they easily pass into kidneys.

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Durie-Salmon staging

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For multiple myeloma

Stages 1-3 - based on myeloma cell mass, hemoglobin, serum calcium, bone lesions, M-component production rate.

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M-component protein

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Just know that all Ig heavy classes are in the gamma or beta region of the electrophoresis.

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International staging system for myeloma

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1-3

Looks are serum beta-2 microglobulin and serum albumin.

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Dx criteria for multiple myeloma

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Presence of serum/urinary monoclonal protein

Presence of clonal plasma cells in BM or plasmacytoma.

Presence of end organ damage related to the dyscrasia or secondary to it (e.g. high calcium, bone lesions, anemia, renal failure)

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Progression of multiple myeloma

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It goes in remissions, but those gradually are less dramatic and more frequent.

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Tx of multiple myeloma - New active agents

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Thalidomide, bortezomid, lenalidomide

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Tx of multiple myeloma - Less toxic agent

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Liposomal doxorubicin

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Another tx scheme for multiple myeloma that seems to be helpful

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autologous bone marrow transplantation

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A group of chemo agents helpful if autologous BM transplantation is not possible

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MPT - Melphalan, Thalomid, Prednisone.

(not sure where i got that from)

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General tx of multiple myeloma

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Gentle tx may be good (less tx complications, greater survival)

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68 yo M w/ wt loss, night sweats, cough
Elevated gammaglobulins, anemia,
CXR RUL lung mass (lung CA)

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Polyclonal gammaopathy

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72 yo F w/ RA x many years
0.4 gm/DL IgG kappa, nml labs
Only pain in joints

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MGUS

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72 yo M w/ 3 months increasing L hip pain
Hgb 9.8, Ca 11.6, Cr 1.8
Xrays show lytic Lesion L hip
IgG Lambda 3.5 gm/DL in serum
Lambda light chains in urine

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Stage III Myeloma.

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Lt chains can cause...

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Amyloid deposition and renal damage

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IgMs can cause...

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Neuropathy (anti-myelin associated glycoproteins can cause this too) and hyperviscosity...because they are pentamers.

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Amyloid AL

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Light chains deposited in tissues

With or without MGUS

The more critical the organ in which amyloid deposits in, the worse the px.

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Hyperviscosity can cause...

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Neuro issues - Blurred vision, HA, vertigo, confusion, stroke, ataxia...

Funduscopic - Dilated segmented and tortuous retinal veins (sausage-link appearance)

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How to tx too much IgM

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Pheresis - removing serum.

works bc serum is the only place IgM deposits.

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Tx of Waldenstrom's

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Like a low grade lymphoma

(rituxan, bortezomib, fludarabine, cyclophosphamide, steroids)

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Smoldering Multiple myeloma

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this is normal multiple myeloma without CRAB sx.