Hematology and Oncology Book

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Multiple Myeloma

Aka: Multiple Myeloma, Myeloma, Plasmacytoma
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  1. Epidemiology
    1. Elderly (mean age 68 years)
    2. Prevalence: 50,000 persons in United States
    3. Incidence: 16,000 new cases per year in United States
    4. Twice as common in black persons
    5. More common in men
    6. Family History confers 2-4 fold increased risk (autosomal dominant trait)
    7. Associated conditions
      1. Obesity
      2. Rheumatoid Arthritis
      3. Monoclonal Gammopathy of Undetermined Significance (MGUS)
    8. Associated with certain occupational exposures
      1. Farming Pesticides
      2. Petroleum workers
      3. Woodworkers
      4. Leather workers
      5. Ionizing radiation
  2. Pathophysiology
    1. Malignant proliferation of Plasma Cells
      1. Overproduce M Protein
    2. Plasmacytoma may also form solitary plasma cell tumor
    3. Origin
      1. Spontaneous (de novo) onset in 80% of cases
      2. Monoclonal Gammopathy of Undetermined Significance (MGUS) in 20% of cases
  3. Symptoms (Asymptomatic in 34% of cases)
    1. Back pain or bone pain (58%)
    2. Fatigue (32%)
    3. Pathologic Fracture (up to 34% of cases)
    4. Anorexia (24%)
    5. Paresthesias (5%)
      1. Wrist Pain (Carpal Tunnel related Neuropathy)
  4. Signs: Bone Findings
    1. Osteolytic lesions
    2. Pathologic Fractures
    3. Palpable swellings on accessible bones
    4. Location
      1. Sternum
      2. Skull
      3. Ribs
      4. Vertebrae (May result in spinal cord compression)
  5. Differential Diagnosis
    1. Common
      1. Monoclonal Gammopathy of Undetermined Significance (MGUS)
    2. Uncommon
      1. Waldenstrom Macroglobulinemia
      2. Amyloidosis
      3. B-Cell non-Hodgkin Lymphoma
    3. Rare
      1. Plasmacytoma
      2. Plasma Cell Leukemia
  6. Labs: Findings
    1. Serum Protein Electrophoresis and Urine Protein electrophoresis for Monoclonal Peak
      1. M Protein in either serum or urine: 97% of patients
      2. Serum M Protein by electophoresis (82%) or immunofixation (93%)
      3. Urine M Protein by electrophoresis: 75%
    2. Chemistry panel with Serum Calcium
      1. Hypercalcemia
        1. Serum Calcium >11 mg/dl (present in 13% of patients)
      2. Renal Insufficiency
        1. Serum Creatinine >2 mg/dl (present in 23% of patients)
    3. Complete Blood Count with platelets
      1. Normochromic Normocytic Anemia
        1. Hemoglobin <12 grams/dl (present in 65% of patients)
    4. Bone Marrow Aspiration and biopsy
      1. HemeoncMultipleMyelomaMarrow.jpg
    5. Peripheral Smear
      1. Myeloma Cells
      2. Rouleaux of Red Blood Cells
    6. Erythrocyte Sedimentation Rate (ESR)
      1. Increased >50 mm/hour in most cases (except bence-jones Myeloma)
    7. Serum Viscosity
    8. Urinalysis
      1. Bence-Jones Protein
  7. Imaging: Skeletal radiographic study (including Skull XRay)
    1. Classic "punched out" lytic lesions (66% of patients)
    2. Pathologic Fractures (26% of patients)
    3. Osteoporosis (23% of patients)
  8. Differential Diagnosis
    1. Monoclonal Gammopathy
  9. Diagnosis
    1. Criteria
      1. Marrow Plasmacytosis >10%
      2. Serum M Protein 3 grams/dl
      3. Symptomatic or end organ findings?
        1. Asymptomatic Multiple Myeloma (smoldering)
        2. Symptomatic Multiple Myeloma
    2. Variants with low M Protein (represent 20% of cases)
      1. Minimally elevated M Protein (<1 g/dl)
      2. Normal M Protein
        1. Bence Jones Myeloma (Increased urine excretion of light chains)
      3. Undetectable M Protein
        1. Non-secretory Multiple Myeloma (abnormal serum-free light chain ratio)
  10. Staging
    1. Stage I
      1. Serum B2 Microglobulin <3.5 mg/L
      2. Serum Albumin >= 3.5 g/dl
    2. Stage 2
      1. Serum B2 Microglobulin 3.5 to 5.5 mg/L
    3. Stage 3
      1. Serum B2 Microglobulin >=5.5 mg/L
  11. Management: Combination Therapy
    1. Indication
      1. Symptomatic Multiple Myeloma
    2. Protocol 1: Age under 65 years (or older patients who are otherwise physically healthy)
      1. Autologous stem cell transplant and
      2. High dose Chemotherapy
        1. Vincristine
        2. Doxorubicin
        3. Dexamethasone
        4. Thalidomide (in some cases)
    3. Protocol 2: Age over 65 years
      1. Melphalan
      2. Prednisolone
      3. Thalidomide (in some cases)
    4. Efficacy
      1. Palliative (Not curative)
      2. Relapse is common
  12. Complications
    1. Immune Suppression
      1. Infection presenting complaint in 25% of patients
      2. Start empiric antibiotics for febrile illness
      3. See prevention below for Immunizations
    2. Hypercalcemia
      1. Initial Management: Normal Saline Infusion with Corticosteroids
      2. Additional management in Refractory Cases: Furosemide, Bisphosphonates
    3. Renal Failure
      1. Dialysis as indicated
    4. Neuropathy (Nerve infiltration by amyloid)
    5. Anemia
      1. Consider differential diagnosis for Anemia
      2. Often improves with Multiple Myeloma treatment
      3. Consider Erythropoietin or transfusion
    6. Vertebral Fractures
      1. See Vertebral Fracture
      2. Intravenous Bisphosphonates (Pamidronate, Zoledronic acid)
        1. Continue indefinately
        2. Reduces FractureIncidence and pain
      3. Surgical Intervention: Percutaneous Vertebroplasty or Kyphoplasty
        1. Indicated in refractory cases
      4. Radiation Therapy
        1. Indicated for spinal cord compression
    7. Hyperviscosity Syndrome
      1. Findings: Fatigue, Headache, Visual disturbance, Retinopathy
      2. Treat with plasmaphoresis
  13. Prevention: Immunizations
    1. Influenza Vaccine
    2. Pneumococcal Vaccine (Pneumovax)
    3. Haemophilus influenzae B Vaccine
  14. Monitoring
    1. Symptomatic improvement
    2. Decrease in M Component
  15. Prognosis
    1. Invariably fatal but relates to staging
      1. Stage I: 62 Month median survival
      2. Stage 3: 29 Month median survival
    2. Treated patients live asymptomatically for years
    3. Mortality from cause unrelated to Myeloma: 25%
  16. Resources
    1. Multiple Myeloma Research Web Server
      1. http://myeloma.med.cornell.edu
    2. Cleveland Clinic Multiple Myeloma and Amyloidosis
      1. http://www.clevelandclinic.org/myeloma
  17. References
    1. Nau (2008) Am Fam Physician 78(7): 853-9
    2. Kyle (2002) N Engl J Med 346: 564-9
    3. Kyle (2003) Mayo Clin Proc 78:21-33
    4. Rajkumar (2005) Mayo Clin Proc 80:1371-82

Plasmacytoma (C0032131)

Definition (NCI) A type of cancer that begins in plasma cells (white blood cells that produce antibodies). A plasmacytoma may turn into multiple myeloma.
Definition (NCI) A single focus of clonal (malignant) plasma cells either in the bone or in another anatomic site without peripheral blood involvement. --2003
Definition (NCI) A malignant (clonal) proliferation of plasma cells that are cytologically and immunophenotypically identical to those of plasma cell myeloma, but manifest a localized osseous or extraosseous growth pattern. (WHO, 2001)
Definition (MSH) Any discrete, presumably solitary, mass of neoplastic PLASMA CELLS either in BONE MARROW or various extramedullary sites.
Concepts Neoplastic Process (T191)
MSH D010954
ICD10 M9731/3, C90.3, C90.30
SnomedCT 188719003, 190017008, 188720009, 190019006, 190016004, 308122007, 10639003, 154644004, 189507001, 269652000, 109987008, 415112005, 302852008, 274907000
English Monostotic myeloma, Plasma Cell Tumors, Plasmacytoma, Plasmacytomas, Plasmocytoma, Plasmocytomas, PLASMA CELL TUMORS, Plasmacytoma, NOS, Plasma Cell Tumor, Plasma cell tumor NOS, Plasma cell tumour NOS, Tumor, Plasma Cell, Tumors, Plasma Cell, [M]Plasma cell tumor NOS, [M]Plasma cell tumour NOS, [M]Plasma cell tumors, [M]Plasma cell tumours, Plasma cell tumors, Plasma cell tumours, [M]Plasmacytoma NOS, Plasmacytoma NOS, malignant plasmacytoma (diagnosis), 973 PLASMA CELL TUMORS, malignant plasmacytoma, [M]Plasmacytoma NOS (disorder), Plasmacytoma NOS (disorder), plasmacytoma, PLASMACYTOMA, [M]Plasma cell tumors (morphologic abnormality), [M]Plasma cell tumor NOS (morphologic abnormality), Solitary plasmacytoma NOS, Plasmacytoma [Disease/Finding], plasmacytomas, plasmocytomas, plasma cell tumors, bone plasmacytoma, plasma cell tumor, plasma cell neoplasms, plasmacytomas solitary, plasmocytoma, Plasma cell tumor, Solitary myeloma, Solitary plasmacytoma, Plasma cell tumour, Plasmacytoma of bone, solitary plasmacytoma, monostotic; myeloma, plasma cell, monostotic; myeloma, myeloma; monostotic, plasma cell, myeloma; monostotic, myeloma; solitary, plasma cell; tumor, solitary; myeloma, tumor; plasma cell, Myeloma, solitary, Myeloma - solitary, Plasma cell tumor (clinical), Solitary myeloma (clinical), Solitary Plasmacytoma, Plasmacytoma (disorder), Plasmacytoma - disorder, Solitary plasmacytoma of bone (morphologic abnormality), Plasma cell tumor (morphologic abnormality), Plasma cell tumour (clinical), Plasmacytoma (morphologic abnormality)
Italian Plasmocitoma, Tumore plasmacellulare, Tumore delle plasmacellule, Plasmacitoma
Dutch plasmaceltumor, monostotisch; myeloom, plasmacel, monostotisch; myeloom, myeloom; monostotisch, plasmacel, myeloom; monostotisch, myeloom; solitair, plasmacel; tumor, solitair; myeloom, tumor; plasmacel, plasmacytoom, Plasmaceltumor, Plasmacytoom
French Tumeur à cellules plasmatiques, Plasmocytome solitaire, Plasmocytome
German Plasmazellentumor, Plasmazelltumor, Plasmozytom
Portuguese Tumor de plasmócitos, Plasmacitoma, Neoplasia de Plasmócitos, Plasmocitoma, Tumor de Plasmócitos, Tumor de Células Plasmáticas
Spanish Tumor de células plasmáticas, mieloma monostótico, tumor de células plasmáticas, tumor de células plasmáticas (anomalía morfológica), plasmocitoma (anomalía morfológica), Myeloma - solitary, Solitary myeloma, Plasmacytoma, Plasmacytoma NOS, Myeloma, solitary, [M]Plasmacytoma NOS, [M]plasmocitomas, [M]plasmocitomas (anomalía morfológica), [M]plasmocitomas, SAI, [M]plasmocitomas, SAI (anomalía morfológica), mieloma solitario (clínico), mieloma solitario, plasmocitoma solitario, plasmocitoma, SAI (trastorno), plasmocitoma, SAI, tumor de células plasmáticas (clínico), tumor de células plasmáticas, SAI, plasmocitoma (trastorno), plasmocitoma, plasmocitoma solitario óseo (anomalía morfológica), plasmocitoma solitario óseo, Plasmacitoma, Plasmocitoma, Tumor de Celulas Plasmaticas, Tumor de Células Plasmáticas
Japanese 形質細胞腫瘍, ｹｲｼﾂｻｲﾎﾞｳｼｭ, ｹｲｼﾂｻｲﾎﾞｳｼｭﾖｳ, 骨髄腫, 形質細胞腫, プラスマ細胞腫, プラスモサイトーマ, ミエローマ
Swedish Plasmacytom
Czech plazmocytom, Plazmocytom, Nádor z plazmatických buněk
Finnish Plasmosytooma
Russian PLAZMOTSITOMA, ПЛАЗМОЦИТОМА
Croatian PLAZMOCITOM
Polish Guz plazmatycznokomórkowy, Plazmocytoma
Hungarian Plazmasejtes tumor, Plasmocytoma
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Multiple Myeloma (C0026764)

Definition (MEDLINEPLUS)

Multiple myeloma is a cancer that begins in plasma cells, a type of white blood cell. These cells are part of your immune system, which helps protect the body from germs and other harmful substances. In time, myeloma cells collect in the bone marrow and in the solid parts of bone.

No one knows the exact causes of multiple myeloma, but it is more common in older people and African-Americans. Early symptoms may include

  • Bone pain, often in the back or ribs
  • Broken bones
  • Weakness or fatigue
  • Weight loss
  • Repeated infections

Myeloma is hard to cure. Treatment may help control symptoms and complications. Options include chemotherapy, stem cell transplantation and radiation.

NIH: National Cancer Institute
Definition (NCI) A type of cancer that begins in plasma cells (white blood cells that produce antibodies).
Definition (CSP) malignant neoplasm of plasma cells usually arising in the bone marrow and manifested by skeletal destruction, bone pain, and the presence of anomalous immunoglobulins.
Definition (NCI) A bone marrow-based plasma cell neoplasm characterized by a serum monoclonal protein and skeletal destruction with osteolytic lesions, pathological fractures, bone pain, hypercalcemia, and anemia. Clinical variants include non-secretory myeloma, smoldering myeloma, indolent myeloma, and plasma cell leukemia. (WHO, 2001)
Definition (MSH) A malignancy of mature PLASMA CELLS engaging in monoclonal immunoglobulin production. It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. Other features include ANEMIA; HYPERCALCEMIA; and RENAL INSUFFICIENCY.
Concepts Neoplastic Process (T191)
MSH D009101
ICD9 203.0
ICD10 C90.0, M9732/3, C90.00
SnomedCT 94705007, 154585004, 55921005, 109989006
English Multiple Myeloma, Multiple Myelomas, Myeloma, Multiple, Myeloma, Plasma Cell, Myelomas, Multiple, MULTIPLE MYELOMA MYELOMATOSIS, KAHLER DISEASE, KAHLER-BOZZOLO DISEASE, MULTIPLE MYELOMA, MYELOMA, MYELOMATOSIS MULTIPLE, Myeloma, NOS, Myeloma, Plasma-Cell, Myelomas, Plasma-Cell, PLASMA CELL MYELOMA, Plasma-Cell Myeloma, Plasma-Cell Myelomas, myeloma, Multiple myeloma / Plasma cell neoplasm, MYELOMA, MULTIPLE, multiple myeloma (diagnosis), multiple myeloma, Myeloma (disorder), Multiple myeloma, no ICD-O subtype, Plasma-cell myeloma, Peripheral plasma cell myeloma, Multiple myeloma myelomatosis, Myelomatosis multiple, Plasma Cell Myeloma, Cell Myeloma, Plasma, Cell Myelomas, Plasma, Plasma Cell Myelomas, Myelomas, Plasma Cell, Multiple myeloma NOS, Multiple Myeloma [Disease/Finding], Myeloma-Multiple, Kahler Disease, Myelomatoses, Disease, Kahler, Myelomatosis, Myeloma-Multiples, Myeloma Multiple, myelomas, kahler disease, Myeloma;multiple, multiple myeloma (MM), multiple myelomatosis, kahler's disease, multiple myelomas, Plasma-cell Myeloma, Kahler disease, myelomatosis, Multiple myelomas, Plasma cell myeloma, Plasmacytic myeloma, Myeloma, Multiple myeloma, Kahler's disease, [M]Plasma cell myeloma, Multiple myeloma (disorder), plasma cell neoplasm, neoplasm, plasma cell, myeloma, plasma cell, plasma cell myeloma, myeloma, multiple, multiple myeloma and other plasma cell neoplasms, plasma cell neoplasms, Kahler, myelomata; multiple, Multiple myeloma (clinical), Multiple myeloma, no ICD-O subtype (morphologic abnormality), Multiple myeloma, morphology (morphologic abnormality)
French MYELOME MULTIPLE, Myélome à cellules plasmatiques, Myélomatose, Myélome périphérique à cellules plasmatiques, Myélomatose avec myélomes multiples, Myélomatose multiple, Myélome, MYELOMATOSE MULTIPLE, Myélomes multiples, Myélome multiple, Maladie de Kahler
Portuguese MIELOMA MULTIPLO, Mieloma, Mieloma de plasmócitos, Mielomatose múltipla, Mieloma plasmocítico periférico, Mielomatose de mieloma múltiplo, Mielomatose, MIELOMATOSE MULTIPLA, Mieloma múltiplo, Mielomas múltiplos, Mieloma de Células Plasmáticas, Mieloma de Plasmócitos, Mieloma Múltiplo, Mieloma Plasmocitário
Spanish MIELOMA MULTIPLE, Mielomatosis, Mielomatosis múltiple, Mieloma, Mieloma de células plasmáticas periféricas, Mieloma de células plasmáticas, mieloma múltiple, morfología (anomalía morfológica), mieloma múltiple (clínico) (trastorno), Myeloma, MIELOMATOSIS MULTIPLE, mieloma múltiple, subtipo sin ICD-O (anomalía morfológica), mieloma múltiple, no clasificado como subtipo en CIE-O (anomalía morfológica), mieloma múltiple, subtipo sin ICD-O, (M)mieloma de células plasmáticas, mieloma múltiple, no clasificado como subtipo en CIE-O, enfermedad de Khaler, mieloma de células plasmáticas, mieloma múltiple, enfermedad (concepto no activo), mieloma múltiple, mielomatosis, mieloma, Mieloma múltiple, Mielomas múltiples, mieloma múltiple (clínico), enfermedad de Kahler, mieloma múltiple (trastorno), Mieloma de Células Plasmáticas, Mieloma Múltiple, Mieloma Multiple, Mieloma de Celulas Plasmaticas
German MULTIPLES MYELOM, multiples Myelom, Myelomatose, Plasmazellenmyelom, Myelomatose multiple, Myelomatose, Myelom, peripheres Plasmazellenmyelom, MYELOMATOSE MULTIPLE, Plasmozytom [Multiples Myelom], Multiple Myelome, multiples Myelom, Multiples Myelom, Myelom, Plasmazell-
Italian Mielomi multipli, Mielomatosi da mieloma multiplo, Mieloma, Mielomatosi multipla, Plasmocitoma maligno periferico, Mielomatosi, MM, Mieloma plasmacellulare, Malattia di Kahler-Bozzolo, Mieloma multiplo
Dutch myelomatosis multipel, plasmacel myeloom, multipel myeloom myelomatosis, myeloom, myelomatosis, perifeer plasmacelmyeloom, myelomata; multipel, multipel myeloom, multipele myelomen, Multipel myeloom, Myeloom, multipel, Plasmacelmyeloom
Japanese 骨髄腫症, 多発性骨髄腫症, 末梢形質細胞性骨髄腫, ミエローマ, ﾐｴﾛｰﾏ, ﾀﾊﾂｾｲｺﾂｽﾞｲｼｭｼｮｳ, ﾏｯｼｮｳｹｲｼﾂｻｲﾎﾞｳｾｲｺﾂｽﾞｲｼｭ, ｹｲｼﾂｻｲﾎﾞｳｾｲｺﾂｽﾞｲｼｭ, ﾀﾊﾂｾｲｺﾂｽﾞｲｼｭ, ｺﾂｽﾞｲｼｭｼｮｳ, 骨髄腫-多発性, Kahler病, カーレル病, プラスマ細胞性骨髄腫, 多発性骨髄腫, 多発骨髄腫, 形質細胞性骨髄腫, 形質細胞骨髄腫, 骨髄腫-形質細胞, カーラー病
Swedish Multipelt myelom
Czech myelom mnohočetný, Myelom z periferních plazmatických buněk, Myelomatóza s mnohočetným myelomem, Myelom, Plazmocytický myelom, Mnohočetný myelom, Myelomatóza, Myelomatóza mnohočetná, Mnohočetné myelomy
Finnish Multippeli myelooma
Russian MIELOMNAIA BOLEZN', MIELOMA MNOZHESTVENNAIA, MIELOMATOZ, BOLEZN' RUSTITSKOGO-KALERA, MIELOMA, RETIKULOPLAZMOTSITOZ, MNOZHESTVENNAIA MIELOMA, БОЛЕЗНЬ РУСТИЦКОГО-КАЛЕРА, МИЕЛОМА, МИЕЛОМА МНОЖЕСТВЕННАЯ, МИЕЛОМАТОЗ, МИЕЛОМНАЯ БОЛЕЗНЬ, МНОЖЕСТВЕННАЯ МИЕЛОМА, РЕТИКУЛОПЛАЗМОЦИТОЗ
Korean 다발성 골수종
Croatian MULTIPLI MIJELOM
Polish Siatkowiak plazmocytowy, Choroba Kahlera, Plasmocytoma, Szpiczak mnogi, Szpiczak plazmocytowy
Hungarian Myelomatosis, Többszörös myelomák, Multiplex myeloma myelomatosis, Myeloma, Plazmasejtes myeloma, Myelomatosis multiplex, Peripheriás plazmasejtes myeloma, Myeloma multiplex
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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