Multiple Myeloma Morning Report July 21, 2009 Lindsay Kruska.
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Multiple MyelomaMultiple Myeloma
Morning ReportMorning Report
July 21, 2009July 21, 2009
Lindsay KruskaLindsay Kruska
Multiple MyelomaMultiple Myeloma
Neoplastic proliferation of single clone of Neoplastic proliferation of single clone of plasma cells producing monclonal plasma cells producing monclonal immunoglobulinimmunoglobulin
Cause unknownCause unknown Radiation and solvents ?associatedRadiation and solvents ?associated
1% malignancy, 10% hematologic 1% malignancy, 10% hematologic malignancy in USmalignancy in US Incidence 4-5/100,000Incidence 4-5/100,000
Median age presentation 60-66y, rare Median age presentation 60-66y, rare <40y (2%)<40y (2%)
Presentation: SymptomsPresentation: Symptoms
Suspected usually due to widely varied Suspected usually due to widely varied symptoms (>6mo in 40%)symptoms (>6mo in 40%) Bone pain (67%)Bone pain (67%) Weakness and Fatigue (30%)Weakness and Fatigue (30%) Weight loss (24%)Weight loss (24%) Asthenia (14%)Asthenia (14%) Dyspnea (4%)Dyspnea (4%) Fever (<1%)Fever (<1%) Sx of cord compressionSx of cord compression Repeated infections (pneumonia, pyelonephritis)Repeated infections (pneumonia, pyelonephritis)
Presentation: SignsPresentation: Signs
Physical ExamPhysical Exam PallorPallor Organomegaly, palpable LN rare (<5%)Organomegaly, palpable LN rare (<5%) Radiculopathy (cord compression) (5%)Radiculopathy (cord compression) (5%) Peripheral neuropathy uncommonPeripheral neuropathy uncommon
LabsLabs Hypercalcemia (36%)Hypercalcemia (36%) Increased serum total proteinIncreased serum total protein Anemia (34%)Anemia (34%) Acute renal failure (34%)Acute renal failure (34%) Low anion gapLow anion gap
Work upWork up CBC with diff, smearCBC with diff, smear
Anemia, rouleauxAnemia, rouleaux ChemistriesChemistries
Ca, Creatinine, Total proteinCa, Creatinine, Total protein SPEP with immunofixation (87% sensitive)SPEP with immunofixation (87% sensitive) UPEP (24h urine) (75% sensitive)UPEP (24h urine) (75% sensitive) Serum light chainsSerum light chains Beta-2 microglobulinBeta-2 microglobulin Bone marrow biopsyBone marrow biopsy Bone survey, occasionally advanced imagingBone survey, occasionally advanced imaging
Bone Marrow BiopsyBone Marrow Biopsy
Marrow Marrow plasmacytosis plasmacytosis (>10%)(>10%) CD138+, CD138+,
monoclonalmonoclonal Focal BM Focal BM
involvementinvolvement 10% require 10% require
multiple biopsiesmultiple biopsies
DiagnosisDiagnosis
Presence of serum and/or urine Presence of serum and/or urine monoclonal proteinmonoclonal protein IgG (53%), IgA (25%), IgD (1%)IgG (53%), IgA (25%), IgD (1%) Free light chains (20%)Free light chains (20%)
Clonal plasma cells or plasmacytomaClonal plasma cells or plasmacytoma End organ damageEnd organ damage
HyperHyperCCa, a, RRenal failure, enal failure, AAnemia, Lytic nemia, Lytic BBone lesionsone lesions
Main Differential Diagosis: Main Differential Diagosis: Elevated M proteinElevated M protein
MGUS (1%/year)MGUS (1%/year) Absence of symptomsAbsence of symptoms M protein <3g/L M protein <3g/L <10% plasma cells in marrow<10% plasma cells in marrow No anemia, renal failure, hyperCa, lytic lesionsNo anemia, renal failure, hyperCa, lytic lesions
Smoldering Multiple Myeloma (10%/year)Smoldering Multiple Myeloma (10%/year) Meets dx criteria for MM but no end organ Meets dx criteria for MM but no end organ
Primary amyloidosisPrimary amyloidosis Metastatic cancerMetastatic cancer
Differential Also IncludesDifferential Also Includes
Other lymphoid Other lymphoid neoplasms neoplasms CLLCLL B and T cell lymphomasB and T cell lymphomas
Non lymphoid Non lymphoid neoplasmsneoplasms Breast CaBreast Ca CMLCML Breast and colon cancerBreast and colon cancer
CirrhosisCirrhosis SarcoidosisSarcoidosis
Gaucher’s diseaseGaucher’s disease Pyoderma Pyoderma
gangrenosumgangrenosum Autoimmune Autoimmune
conditionsconditions Myasthenia gravisMyasthenia gravis Rheumatoid arthritisRheumatoid arthritis Cold agglutinin diseaseCold agglutinin disease
Several rare skin DOSeveral rare skin DO
StagingStaging
Durie-Salmon staging (Stages I-III, a/b), Durie-Salmon staging (Stages I-III, a/b), 19751975 M protein M protein Serum Calcium Serum Calcium Radiographic Bone InvolvementRadiographic Bone Involvement HemoglobinHemoglobin Renal failureRenal failure
International Staging System (Stages I-III), International Staging System (Stages I-III), 20052005 Serum beta2 microglobulinSerum beta2 microglobulin AlbuminAlbumin
TherapyTherapy Supportive careSupportive care
Hypercalcemia/bone involvement: usual therapies, Hypercalcemia/bone involvement: usual therapies, analgesia, occ XRTanalgesia, occ XRT
Renal involvement: adequate hydration, ?Renal involvement: adequate hydration, ?plasmapheresisplasmapheresis
Low threshold for infectious complications Low threshold for infectious complications Hyperviscosity: plasmapheresisHyperviscosity: plasmapheresis Neurologic compromise: palliative radiationNeurologic compromise: palliative radiation Anemia: transfusionAnemia: transfusion
Initation of specific therapyInitation of specific therapy ChemotherapyChemotherapy
ThalidomideThalidomide DexamethasoneDexamethasone Melphalan/prednisoneMelphalan/prednisone Bortezomib (proteasome inhibitor)Bortezomib (proteasome inhibitor)
HSCTHSCT
PrognosisPrognosis
Usually fatalUsually fatal 10-20% mortality within first 2 months10-20% mortality within first 2 months
Mean survival 4-5yMean survival 4-5y 5y survival 31%, 10y 10%, 15y 4%5y survival 31%, 10y 10%, 15y 4%
Delay in diagnosis associated with negative impact Delay in diagnosis associated with negative impact on disease courseon disease course Lead time biasLead time bias
Improved survival with therapy including HSCTImproved survival with therapy including HSCT Major causes of death: progressive myeloma, renal Major causes of death: progressive myeloma, renal
failure, sepsis, therapy related acute leukemia or failure, sepsis, therapy related acute leukemia or myelodisplasia; 25% die of age related illnessesmyelodisplasia; 25% die of age related illnesses
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