Hematology and Oncology Book

http://www.fpnotebook.com/

Multiple MyelomaAka: Myeloma, Plasmacytoma

Advertisement

  1. Epidemiology
    1. Elderly (mean age 68 years)
    2. Associated with certain occupational exposures
      1. Farmers
      2. Petroleum workers
      3. Woodworkers
      4. Leather workers
  2. Pathophysiology
    1. Malignant proliferation of Plasma Cells
    2. Plasmacytoma may also form solitary plasma cell tumor
  3. Symptoms and Signs
    1. Bone pain
      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)
    2. Weight Loss
    3. Pallor
  4. Complications
    1. Immune Suppression
      1. Infection presenting complaint in 25% of patients
    2. Hypercalcemia
    3. Renal Failure
    4. Neuropathy (Nerve infiltration by amyloid)
    5. Anemia (80%)
    6. Hyperviscosity Syndrome
      1. Fatigue
      2. Headache
      3. Visual disturbance
      4. Retinopathy
  5. Diagnosis: Classic Triad
    1. Marrow Plasmacytosis >10%
    2. Lytic bone lesions
    3. Serum or Urine M Component
  6. Differential Diagnosis
    1. Benign Monoclonal Gammopathy
      1. Progresses to Myeloma in 11% of cases
  7. Labs
    1. Complete Blood Count with platelets
      1. Normochromic Normocytic Anemia
    2. Bone Marrow Aspiration and biopsy
      1. HemeoncMultipleMyelomaMarrow.jpg
    3. Peripheral Smear
      1. Myeloma Cells
      2. Rouleaux of Red Blood Cells
    4. Erythrocyte Sedimentation Rate (ESR) elevated
    5. Serum and Urine Protein electrophoresis
      1. Monoclonal peak
      2. Quantitate M Protein
    6. Serum Calcium
      1. Hypercalcemia
    7. Renal Function Testing
      1. Renal Insufficiency
    8. Serum Viscosity
    9. Urinalysis
      1. Bence-Jones Protein
  8. Radiology
    1. Skeletal radiographic study (including Skull XRay)
    2. Classic "punched out" lytic lesions
  9. Management: Combination Therapy
    1. Duration
      1. Course: 4 to 7 days
      2. Repeat: every 4 to 6 weeks
      3. Duration: 1 to 2 years
    2. Efficacy
      1. Palliative (Not curative)
    3. Regimen
      1. Alkylating Agent
        1. Melphalan or
        2. Cyclophosphamide or
        3. Chlorambucil
      2. Prednisone
  10. Monitoring
    1. Symptomatic improvement
    2. Decrease in M Component
  11. Prognosis
    1. Invariably fatal but relates to staging
    2. Treated patients live asymptomatically for years
    3. Mortality from cause unrelated to Myeloma: 25%
  12. 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

Multiple Myeloma (C0026764)

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.
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)(mye-eh-LO-ma) Cancer that arises in plasma cells (white blood cells that produce antibodies).
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)
ConceptsNeoplastic Process (T191)
ICD9203.0
EnglishKAHLER DISEASE, Kahler's disease, Multiple Myeloma, Multiple myeloma / Plasma cell neoplasm, multiple myeloma and other plasma cell neoplasms, MULTIPLE MYELOMA MYELOMATOSIS, Multiple Myelomas, Myeloma, Myelomatosis, MYELOMATOSIS MULTIPLE, Plasma cell myeloma, plasma cell neoplasm, plasma cell neoplasms, Plasmacytic myeloma
Spanishenfermedad de Kahler, enfermedad de Khaler, mieloma, mieloma de células plasmáticas, mieloma de celulas plasmaticas, mieloma múltiple, mieloma multiple, mielomatosis
CreditsDerived from the NIH UMLS (Unified Medical Language System)


Plasmacytoma (C0032131)

Definition (MSH)Any discrete, presumably solitary, mass of neoplastic PLASMA CELLS either in BONE MARROW or various extramedullary sites.
Definition (NCI)(PLAS-ma-sye-TOE-ma) Cancer of the plasma cells (white blood cells that produce antibodies) that may turn into multiple myeloma.
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 (NCI)A single focus of clonal (malignant) plasma cells either in the bone or in another anatomic site without peripheral blood involvement. --2003
ConceptsNeoplastic Process (T191)
ICD9238.6
EnglishMonostotic myeloma, Plasma cell tumor, Plasma Cell Tumors, Plasma cell tumour, plasmacytoma, Plasmacytoma of bone, Plasmacytomas, Plasmocytoma, Plasmocytomas, Solitary myeloma, Solitary Plasmacytoma
Spanishmieloma monostótico, mieloma monostotico, mieloma solitario, plasmocitoma, plasmocitoma solitario, plasmocitoma solitario óseo, plasmocitoma solitario oseo, tumor de células plasmáticas, tumor de celulas plasmaticas
CreditsDerived from the NIH UMLS (Unified Medical Language System)



Navigation Tree