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Forms Revision: Myeloma Changes 1/31/2013 Disclosures Sharing knowledge. Sharing hope. Janet Brunner, PA-C • I have no relevant conflicts of interest to disclose. Angela Dispenzieri, MD Forms Revision: • I have no relevant conflicts of interest to disclose. Myeloma Changes J. Brunner, PA-C and A. Dispenzieri, MD February 2013 CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Objectives: 1) Understand why the Myeloma & Amyloid Forms were combined into one Plasma Cell Disorder (PCD)- Pre-HCT Data 2) Complete the revised Pre/Post-HCT Plasma Cell Disorder (PCD) forms accurately Form 2016 CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Subsequent HCT Plasma Cell Disorders (PCD) Additional options have been added: • Additional sub-types have been added • Same disease subtype, but without a prior disease • Osteosclerotic myeloma / POEMS syndrome insert completed (begin with Q1) • Light chain deposition disease (LCDD) • Same disease subtype, but there has been a • Question about preceding / concurrent PCD relapse or progression (begin with Q188- Was • Multiple myeloma (symptomatic) therapy given?) • Smoldering myeloma (asymptomatic) • Monoclonal gammopathy of unknown significance • Same disease (begin with Q233) (MGUS) CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT 1 1/31/2013 How to report Myeloma and Amyloidosis How to report Myeloma & Amyloidosis • Scenario 1 • Scenario 2 • Patient has smoldering myeloma (asymptomatic) & • Patient has symptomatic myeloma & amyloidosis amyloidosis. • Report symptomatic myeloma as the primary • Report amyloidosis as the primary diagnosis for HCT diagnosis for HCT (question 1) (question 1). • Report amyloidosis as a concurrent diagnosis • Report smoldering myeloma as a concurrent diagnosis (question 6) (question 6) CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Bone marrow Blood stream Antibodies Fight Immunoglobulins infection Harmful Use MyelomaPlasma cellscells a.k.a. Myeloma: Monoclonal antibody Poison kidneys M-protein Amyloid: Deposit in Eat away at bones M-spike various organs Crowd bone marrow Myeloma protein causing disease causing anemia Immunoglobulin LCDD Ig Similar to amyloid CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Asymptomatic vs. Symptomatic Myeloma Bone marrow Blood stream Criteria for Spectrum of MGUS MM Myeloma protein Oligosecretory Monoclonal Little protein M-spike No gammopathy secreted No ≥ 3 g/dL (MGUS) OR BMPC Non-IgM Inactive MM Light chain ≥ 10% ? Monoclonal End Organ Yes (smoldering secreted (no Most Damage?* Myeloma protein MM) cells heavy chain) myeloma cases Active or Yes symptomatic No protein MM secreted End organ damage without other explanation Non-secretory Bence Jones Or light chain Calcium Renal Anemia Bones Other myeloma CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Intern. Myeloma Working Group, Br J Haem 121, 749-57, 2003 2 1/31/2013 Light chain only myeloma vs. LCDD Kidney & Plasmaproliferative Disorders Glomerulus Glomerulopathies Tubulointerstitial Light chain only myeloma is merely a type Distal of MM (no heavy chain). It may cause tubule • AL amyloidosis • Cast nephropathy (myeloma kidney) light chain cast nephropathy (renal failure) • Light chain Proximal deposition disease tubule Light chain deposition disease (LCDD) • Type II cryo (diffuse • Acquired is actually very similar to amyloidosis MPGN) Fanconi’s except looks slightly different under • Fibrillary GN syndrome Light chains + microscope Tamm Horsfall Protein = light chain cast nephropathy CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Staging of Myeloma International Staging System • Durie-Salmon Staging Stage Criterion • Requires hemoglobin, serum calcium, monoclonal protein & bone survey findings Stage I 2M < 3.5 mg/L and alb 3.5 g/dL • International Staging System (ISS) Stage II Not Stage I or III • Requires β microglobulin & albumin 2 Stage III 2M 5.5 mg/L CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Greipp P R et al. JCO 2005;23:3412-3420 Durie Salmon Staging System International Durie Salmon Stage I Stage III Staging System Staging System All of the following One or more of the following Hemoglobin > 10.0 g/dL Hemoglobin < 8.5 g/dL Serum calcium < 12 mg/dL Serum calcium > 12 mg/dL On radiograph, normal bone structure or Advanced lytic bone lesions solitary bone plasmacytoma only IIA 909/1759 58 (54,61) Low M-component production rates High M-component rates IgG < 5 g/dL, IgA < 3 g/dL, or urine IgG > 7 g/dL, IgA > 5 g/L, or urine light M spike on electrophoresis < 4 g/24 chain M spike > 12 g/24 hrs hrs Stage II Fitting neither stage I or III A: Serum creatinine < 2 mg/dL B: Serum creatinine 2 mg/dL CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Greipp P R et al. JCO 2005;23:3412-3420 3 1/31/2013 Cytogenetics IFM Tandem Transplant 513 Pts Risk by FISH and beta-2 microglobulin B2M FISH • FISH LT4 Nml LT4 13 • Conventional GT4 Nml GT4 13 LT4 4;14; 17p GT4 4;14, 17p CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Avet-Loiseau, H. et al. Blood 2007;109:3489-3495 Copyright ©2007 American Society of Hematology. Copyright restrictions may apply. Metaphase Cytogenetics FISH for t(11;14) Genetic Risk in Multiple Myeloma Standard Risk* Intermediate High Risk t(11;14) del17p FISH t(6;14) t(4;14) t(14;16) Hyperdiploid t(14;20) Cytogenetic deletion 13 Other All others Or hypodiploidy GEP High Risk Signature Or PCLI>3% CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Dispenzieri et al. Mayo Clin Proc 2007;82:323-341; Kumar et al. Mayo Clin Proc 2009 84:1095-1110 http://msmart.org/ v7 Revised and updated: Jan 2011 Laboratory Studies This section has been expanded to include: • Gene Expression Profile CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT 4 1/31/2013 Gene Expression Profiling (GEP) Gene expression patterns can distinguish risk groups in training cohort. • Measures activity (the expression) of thousands of genes at once, to create a global picture of cellular function. • GEP can distinguish between cells that are actively dividing, or show how the cells react to a particular treatment. Shaughnessy J D et al. Blood 2007;109:2276-2284 CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT ©2007 by American Society of Hematology Amyloidosis Section Amyloidosis • Cardiac biomarkers (revised form) • Cardiac biomarkers (current) • Brain natriuretic peptide (BNP) • Brain natriuretic peptide (BNP) and/or N-terminal • N-terminal prohormone brain natriuretic peptide (NT- prohormone brain natriuretic peptide (NT-proBNP) proBNP) • Troponin • Troponin I • Troponin T • High sensitivity troponin T CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT Cardiac Biomarkers Significance of Cardiac Biomarkers in AL Troponin T < 0.035 ug/L* (N=127) • BNP- secreted by the ventricles of the heart in (N=182) NT-proBNP < 332 ng/L* response to excessive stretching of heart muscle (N=174) cells (cardiomyocytes) Stage 1: Neither high • Troponin- integral to muscle contraction in skeletal Stage 2: Either high & cardiac muscle. It is the most specific & sensitive Stage 3: Both high laboratory markers of myocardial cell injury CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT CENTER FOR INTERNATIONAL BLOOD & MARROW RESEARCH TRAINING & DEVELOPMENT *Dispenzieri et al J Clin Oncol. 2004 ;22(18):3751-7 Kumar et al, Mayo Clinic Proc, 2011 86(1):12-8. 5 1/31/2013 Validation of the criteria of response to treatment in AL Pre-HCT Therapy amyloidosis Palladini G, Dispenzieri A, Gertz MA, et al. New Criteria for Response to Treatment in Immunoglobulin Light Chain Amyloidosis Based on Free Light Chain Measurement and Cardiac Biomarkers: Impact on Survival Outcomes. Journal of clinical oncology. Added common regimens: 2012;30(36):4541-4549. 1.0 1.0 • VCD (Bortezomab, cyclophosphamide, 0.9 0.9 p=0.01 dexamethasone) 0.8 0.8 p<0.001 0.7 p<0.001 0.7 • RVD/VRD (Bortezomab, lenilidomide, 0.6 0.6 dexamethasone) 0.5 0.5 p<0.001 p<0.001 0.4 0.4 • DVD/VDD (Bortezomab, liposomal doxirubicin, Proportion survivingProportion Proportion surviving 0.3 0.3 CR (97 patients, 3.6 deaths/100 py) dexamethasone) 0.2 VGPR (233 patients, 9.6 deaths/100 py) 0.2 NT-proBNP progression (at least 300 ng/L and 30% increase), 169 patients 0.1 PR (140 patients, 23.7 deaths/100 py) 0.1 NT-proBNP stable, 108 patients NR (179 patients, 47.2 deaths/100 py) NT-proBNP
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