Multiple Myeloma PDF

MULTIPLE MYELOMA NUR NAJMI MOHAMAD ANUAR Multiple myeloma (plasma Neoplastic disease characterized by : plasma cell accumulation in the bone marrow cell PC undergone Ig switch and somatic hypermutation and secrete paraprotein in serum neoplasm) pr...

MULTIPLE MYELOMA NUR NAJMI MOHAMAD ANUAR Multiple myeloma (plasma Neoplastic disease characterized by : plasma cell accumulation in the bone marrow cell PC undergone Ig switch and somatic hypermutation and secrete paraprotein in serum neoplasm) presence of monoclonal protein (M protein) in serum and/or urine related organ or tissue damage unknown cause – common Afro-Carribean 98% cases occur in age above 40 years – peak incidence in 70-80 years Often no symptoms are noticed initially – asymptomatic with no organ or tissue damage Multiple myeloma (plasma cell neoplasm) Monoclonal  When myeloma advanced these signs and symptoms occurs: Bone pain – backache due to vertebral collapse and fracture Bleeding – due to impaired platelet function; thrombocytopenia CLINICAL Frequent infections – due to deficient antibody production, neutropenia, cell –mediated immunity FEATURES Anemia – lethargy, weakness, dyspnoe, pallor, tachycardia Renal failure and/or hypercalcemia – features such as polydipsia, polyuria, anorexia, vomiting, constipation, mental disturbance Amyloidosis occurs as complication to myeloma symptoms Hyperviscosity syndrome with purpura, visual failure, heart failure Lab test : Immuno electrophoresis Finding : Presence of Bence Jones Protein LABORATORY FINDINGS Bone lesion in X-ray – with osteoporosis, vertebral collapse Elevated serum calcium High serum creatinine – proteinaceous deposits from heavy light chain proteinuria, hypercalcemia, uric acid, amyloid Low serum albumin in advanced disease Raised serum B2-macroglobulin - indicator for prognosis High ESR – due to raised abnormal Ig [inflammation Cytogenetic abnormality – hyperdiploid, translocations, 13q monoalelleic loss Increased plasma cells in BM >20% Immunophenotyping - CD38, CD138 Bence Jones Protein Abnormal protein SPEP/UPEP – M protein > - electrophoresis  Bone marrow of MULTIPLE MYELOMA Normal Bone Marrow Multiple Myeloma Bone Marrow  Bone marrow of MULTIPLE MYELOMA Co 138 - Multiple Myeloma Bone Marrow CD138 immunolabeling of MM  Bilobed This bone marrow aspirate smear shows large immature-appearing plasma cells prominent nucleoli and finer nuclear chromatin (large top arrow) to smaller plasma cells with prominent nucleoli (long bottom arrow). A binucleate plasma cell is also seen (arrowhead). Electrophoresis Gamma region S Protein electrophoresis Electrophoresis + Immunofixation G O 1 Ab ↳ prominent in urine Bence Jones protein cast (myeloma cast) from the urinary sediment of a patient with lambda-Bence Jones type multiple myeloma. Sternheimer stein, X200. Serum protein eletrophoresis showed an evident band of Lamda chain (L) of immunoglobulin (A). The Bence-Jones Protein (BJP) was detected after urine electrophoresis (B). PROGNOSIS  Based on ß2-microglobulin and albumin in serum of patients by International Prognostic Index  Poor prognosis: NLDH - ↑ risk of cytogenetics ß2M > 5.5 mg/L Alb < 35 g/L 3 Stage III  Median survival with non-intensive chemotherapy is 3-4 years; may add another 1-2 year with autologous transplantation Stage 1 :. BLM : < 3. 5mg/dL N LDH  Stem cell transplant – in younger patients Intensive therapy – age < 65-70 years Non-intensive therapy – age > 70 years, several courses of TREATMENT chemotherapy Drugs used – Prednisolone, Thalidomide, Lenalidomide, Bortezomib Radiotherapy for areas of bone pain and spinal cord compression