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Majolica's Message Board Messages

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Hi Steve,

  I posted way back with you.  I just wanted to say.  I am too  still going strong with mgus and acquired von willebrand for over 13 years now.  Some bone pain now and then.  Low sodium at times.  But still kicking.

RE: MGUS referral advice.

by Majolica - October 17, 2017

Hello,

  I suggust you enter the Dana Faber Center for Prevention of Progression of Blood Cancers.  They mail a kit that you take to you regular hematology visit and get 3 extra vials of blood to overnight at there expense.  I have been doing it for 2 years.


Hello,

  Here is some info that can help you.

Plasma Cell Myeloma

Definition
  • Multifocal clonal proliferation of plasma cells based in the bone marrow
    • May be designated as either asymptomatic/smoldering or symptomatic, see below
Alternate/Historical Names
  • Multiple myeloma
  • Myelomatosis
  • Medullary plasmacytoma
  • Kahler’s disease
Diagnostic Criteria
  • Asymptomatic / smoldering vs. symptomatic myeloma are separated based on the presence of complications
    • Asymptomatic / smoldering myeloma requires defined levels of evidence of clonality but lacks complications
      • Elevated M protein (>3g/dL) AND/OR
      • >10% clonal plasma cells in marrow
      • Must not have characteristic clinical complications or end organ/tissue damage
    • Symptomatic myeloma requires any level of evidence of clonality plus characteristic complications
      • Any level of clonal bone marrow plasma cells (usually >10%) OR
      • Any level of M protein in serum or urine
        • Usually >3 g/dL IgG or >2.5 g/dL IgA or
        • >1 g/24 hr urine light chain
      • Must have characteristic clinical complications including:
        • Skeletal destruction with osteolytic lesions, pathological fractures, bone pain
        • Hypercalcemia, anemia, hyperviscosity
        • Renal insufficiency
        • Amyloidosis
    • (MGUScomprises those cases exhibiting both lower levels of M protein and marrow involvement and lacking complications)
  • Variants:
    • Non-secretory myeloma(<5% of cases)
      • Same as above but absence of M protein by immunofixation electrophoresis; detectable by serum free light chain analysis in 2/3 cases
    • Plasma cell leukemia
      • Symptomatic plasma cell myeloma WITH
      • ≥2K/uL or ≥20% circulating clonal plasma cells (frequently lymphocytoid)
      • Frequent extramedullary involvement
  • Morphologic characteristics
    • Distribution: interstitial (clusters not associated with blood vessels), or sheets
    • Plasma cell characteristics:
      • Eccentric nuclei
      • Moderate to abundant basophilic cytoplasm
      • Pale perinuclear “hof” representing the golgi zone.  
    • Variable morphology may be seen:
      • Large cells with prominent nucleoli to small cells with “lymphocytoid” appearance
      • Monotonous to pleomorphic
    • Cytologic atypia:
      • While suggestive, is not diagnostic of plasma cell dyscrasia
      • Nuclear pleomorphism, blastic chromatin most specific
      • Plasmablastic morphology is associated with poorer prognosis
        • Large central nucleolus, dispersed chromatin
        • High N:C ratio, large nucleus
        • Loss of nuclear eccentricity and perinuclear hof

Dita Gratzinger MD PhD ditag@stanford.edu

Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Initial posting and updates:: 9/1/07, 2/23/08, 4/14/10

Supplemental studies

Immunohistology and Flow
  • Plasma cell quantitation is performed by differential count on the bone marrow aspirate or by immunohistochemistry for CD138 on the bone marrow biopsy
    • Flow cytometry may significantly underestimate plasma cell percentage
      • It does establish clonality and can differentiate residual clonal plasma cells from admixed polytypic plasma cells

Hematolymphoid markers

  • CD45 absent or dim in >99%

B cell and plasma cell markers

  • CD138 >99%
  • CD38 >99%
  • CD79a, most
  • CD20 variable, often absent
  • CD19 variable, often absent
  • CD56 up to 80% -- aberrant antigen
  • Cytoplasmic kappa or lambda light chain by flow cytometry, in situ hybridization, or immunohistochemistry

Other

  • Cyclin D1 (Bcl1) positive plasma cell myelomas often lymphoplasmacytic, CD20+, have better prognosis
Cytogenetics
  • Loss of 13q14
    • Most common abnormality
    • Associated with poor prognosis
  • t(11:14)(q13:q32)
    • Associated with:
      • Cyclin D1 overexpression
      • Lymphoplasmacytic or small lymphocytic morphology
    • Better prognosis
Useful Laboratory Tests
  • Serum or urine protein electrophoresis, immunofixation, light chain quantification
  • Quantitation and typing of monoclonal immunoglobulin / light chain
  • Serum free light chain analysis may be required to demonstrate clonal light chains
  • These studies may be used to
    • Establish presence of a monoclonal plasma cell population
    • Quantitation helps subtype the plasma cell dyscrasia (i.e. >3g/dL serum monoclonal protein is a major criterion for myeloma)
    • Track disease burden over time
Plasma cell leukemia
  • Frequently CD20+, CD56-,  t(11;14)+
  • Frequently unusual M component (non-secretory, IgD, IgE, light chain only)
Differential Diagnosis

  • Reactive plasmacytosis
  • Plasmacytoma
  • Monoclonal gammopathy of uncertain significance
  • Lymphoplasmacytic lymphoma
  • Plasmablastic lymphoma

Plasmacytoma/MyelomaReactive Plasmacytosis
Light chain restriction by flow cytometry, immunohistochemistry or in situ hybridization No light chain restriction

PlasmacytomaMyeloma
Single osseous or extraosseus lesion Lytic bony lesions
No increase away from lesion Usually increased plasma cells on random bone marrow biopsy
No end-organ damage May have end-organ damage

Symptomatic Plasma Cell MyelomaAsymptomatic / Smoldering MyelomaMGUS
Usually but not always >10% clonal plasma cells in bone marrow or plasmacytoma ** ≥10% clonal plasma cells in bone marrow AND/OR <10% clonal plasma cells in bone marrow
M protein in serum or urine or free light chain in urine Serum M-protein >3g/dL Serum M-protein <3g/dL
** Lytic bony lesions or  end-organ damage (hypercalcemia/ anemia/ renal insufficiency etc) No lytic lesions or end-organ damage No lytic lesions or end-organ damage

** Critical points: bone marrow plasma cells or M-protein above threshholds separates asymptomatic myeloma from MGUS and bone or other end organ damage separates symptomatic from asymptomatic myeloma

Plasmacytoma/MyelomaLymphoplasmacytic LymphomaandNodal,ExtranodalandSplenicMarginal Zone Lymphomas
Plasma cells may be pleomorphic or plasmablastic Plasma cell component usually not markedly atypical
Uniform plasma cell morphology Plasma cells mixed with small lymphocytes
IgG or IgA M component most common IgM or IgG M component most common
CD20 often negative CD20 80%
Uniformly CD138 positive Scattered CD138 positive cells
Often CD56+, CD19-, CD45- CD56-, CD19+, CD45+

Plasmablastic LymphomaAnaplastic (Plasmablastic)Plasmacytoma/Myeloma
Plasmablastic morphology Subset plasmablastic
Often HIV+ or otherwise immunosuppressed Usually not immunosuppressed
Often in oral cavity or mucosal areas of head Extraosseous sites overlap
~60% EBV+ usually EBV negative

Plasmacytoma and plasmablastic lymphoma have the same immunophenotype, other than EBV and are generally separated based on clinical grounds

Clinical

  • 15% of all hematologic malignancies
  • Median age 69 years
  • Median survival 3 years
    • May present with / cause bone pain, pathologic fractures, renal failure, hypercalcemia, recurrent infections due to hypogammaglobulinemia
  • Non-secretory variant (1%)
    • No M protein
    • Less renal damage
  • Asymptomatic/smoldering myeloma
    • Minority of patients
    • Initially 10 % annual progression to myeloma
  • Plasma cell leukemia
    • >20% circulating plasma cells
    • Poor survival
Grading / Staging / Report

  • Grading is not applicable
Durie-Salmon Staging System
  • Stage I
    • Hemoglobin >10 g/dL
    • Serum IgG <5 g/dL
    • Serum IgA <3 g/dL
    • Normal serum calcium
    • Urine monoclonal protein excretion <4 g/day
    • No generalized lytic bone lesions
  • Stage II
    • Intermediate between stages I and III
  • Stage III One or more of the following:
    • Hemoglobin <8.5 g/dL
    • Serum IgG >7 g/dL
    • Serum IgA >5 g/dL
    • Serum calcium >12 g/dL
    • Urine monoclonal protein excretion >12 g/day
    • Advanced lytic bone lesions
The pathology report should contain the following information:
  • Diagnosis in the World Health Organization (WHO) classification
    • Equivalent diagnosis in other classifications used by relevant clinicians
  • Results of supplementary studies if performed
  • Relationship to other specimens from the same patient
  • Information relevant to staging if available

 

ListsPlasma cell neoplasms / Immunosecretory disorders (WHO 2008)
  • Monoclonal gammopathy of undetermined significance (MGUS)
  • Plasma cell myeloma
    • Variants:
      • Non-secretory
      • Smoldering
      • Plasma cell leukemia
  • Plasmacytoma
    • Solitary plasmacytoma of bone
    • Extraosseus (extramedullary) plasmacytoma
  • Monoclonal immunoglobulin deposition diseases
    • Primary amyloidosis
    • Systemic light chain and heavy chain deposition diseases
  • Osteosclerotic myeloma(POEMS syndrome)
  • Heavy chain diseases (variants of lymphoma rather than plasma cell neoplasms)
    • Gamma heavy chain disease (seelymphoplasmacytic lymphoma)
    • Mu heavy chain disease (seechronic lymphocytic leukemia)
    • Alpha heavy chain disease /immunoproliferative small intestinal disease(variant of extranodal marginal zone lymphoma)

Hello,

  I am sorry to hear about your mgus diagnoses.  I have had IgG Lambda mgus for almost 12 years.  Been to several doctors looking for answers over the last 12 years.  Most hematologists are told that mgus does not have symptoms.  They are finding that mgus in deed can and does have various symptoms that can impact your health.  But no treatments are usually introduced unless causing you issues.

Fatigue is one of my biggest issues as well.  I take a good vitamin that helps.  Get exercise to combat the fatigue too.

About what does your future holds?  We would all love to know that.  The fact is that your chance of getting myeloma is only about 1% a year.  So that is good news. 

Info for new Mgus patients

by Majolica - September 13, 2017

Hello,

   I have had mgus for almost 12 years.  Besides getting my labs done every 6 months at hematologist.  I also entered the mgus clinical trail at dana Faber cancer institute.  They are doing a study on mgus to find out who progresses and who does not progress to myeloma.  They send you a kit that you take to your 6 month visits and have them draw 3 extra viles of blood that is flown out to them. 

I feel these studies are very important and hope it can shed some light for future mgus & myeloma patients. 

Center for Prevention of Progression of Blood Cancers (CPOP)

Hello,

   I have not posted in a few years now.  I have had mgus and acquired von willebrand for 12 years this January. I get my blood work done every 6 months.

The only changes in my blood work over the 12 years is that I now have 2 m-spikes and now have immunopareis..low immunglobulins.  Some neuropathy, bone pain from time to time and fatigue.

Don't let any doctors tell you mgus does not have symptoms.  There are many people with symptoms.  My acquired bleeding from the mgus is like being a hemophiliac.

Best thing I did was to eat food without antibiotics and take a good multi vit and wash your hands a lot.

Good luck.

I have had mgus for almost 10 years.  YES! There are symptoms!  Many of which you all decibed.  Exercise is the best way to combat muscle & bone pain. 

Hello,

  I have had mgus for almost 10 years now.  Here are the best sites to get your infor on your labs, as well any questions answered.  Good Luck to you.

http://www.myelomabeacon.com/resources/

https://myeloma.org/Main.action

Hope this helps!

RE: Time for a transplant

by Majolica - June 11, 2015

Hi Steve,

  Sorry to hear that you have to start treatments.  I would check out Myeloma Beacon or IMF Myeloma.  There is plenty of info on ASCT there.  I wish you the very best.

http://www.myelomabeacon.com/

https://myeloma.org/Main.action

Hello,

  Can anyone try and describe what a enlarged spleen feels like.  I have have discomfort there off and on for a while.  My last labs where not that good and was suppose to return to repeat them again.  I have confused doctors for years.  I have monoclonal gammopathy and AVWS.  I have been having blasts in my blood smears with elevated wbc/ monocytes, etc.

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Multiple Myeloma

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