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十九世纪初， Dr. Solly 报告的首例多发骨折病人

十九世纪初， Dr. Solly 报告的首例多发骨折病人. 39yr old woman, Sarah Newbury, developed fatigue and bone pain from multiple fractures, in 1840. 发病四年后的尸检报告. Dr. Solly 的思考. An inflammatory process A “morbid action” of the blood vessels

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十九世纪初， Dr. Solly 报告的首例多发骨折病人

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  1. 十九世纪初，Dr. Solly报告的首例多发骨折病人 39yr old woman, Sarah Newbury, developed fatigue and bone pain from multiple fractures, in 1840

  2. 发病四年后的尸检报告

  3. Dr. Solly的思考 • An inflammatory process • A “morbid action” of the blood vessels • “earthy matter of the bone is absorbed and thrown out by the kidneys in the urine.”

  4. 多发性骨髓瘤 Multiple Myeloma Sir Run Run Shaw Hospital Huang Jinwen

  5. 认识多发性骨髓瘤的历程

  6. 骨髓瘤起源假设

  7. 单克隆抗体与多克隆抗体的区别

  8. 临床表现 • Bone pain • involves the rib ,sternum, spine , clavicle , skull , humerus & femur etc. • The lumbar vertebrae are one of the most common sites of pain • Persistent localized pain may indicate a pathological fracture.

  9. 临床表现 • Hypercalcemia • Pt. present with confusion, somnolence, bone pain, constipation, nausea, and thirst.

  10. 临床表现 • Anemia • normocytic and normochromic. normal BM infiltrated by tumor cells normal RBC production inhibited by cytokines.

  11. 临床表现 • Renal failure • acutely or/and chronically. • commonly due to hypercalcemia. light chains hyperuricemia, • recurrent infections, and local infiltration of tumor cells.

  12. 临床表现 • Infection • Recurrent infections • due to immune deficiency hypogammaglobulinemia leukopenia, etc.

  13. 临床表现 • Bleeding • bleeding resulting from thrombocytopenia. • monoclonal protein may absorb clotting factors • Hyperviscosity • high volume of monoclonal protein • complications such as stroke, myocardial ischemia, infarction.

  14. 临床表现 • Neurological symptoms • Weakness, confusion and fatigue • Headache, visual changes and retinopathy • Symptoms due to spinal cord compression • Neuropathies due to amyloid.

  15. ClinicalManifestations • C = Calcium elevated (25%) • R = Renal failure (20%) • A = Anemia (80%) • B = Bone lesions (70%) CRAB

  16. 体检 • Pallor: anemia • Bone: pathologic fracture... • Extramedullary: Soft tissue masses • Amyloidosis: Macroglossia, Sensory changes… • Neurologic:, Weakness

  17. Labs • Lab Studies: • CBC: anemia, thrombocytopenia, leukopenia • Peripheral blood smear : Rouleaux formation

  18. Labs • Bone marrow aspirate & biopsy

  19. Labs Serum protein electrophoresis (SPEP)

  20. Labs Serum Immunofixation

  21. Labs • A 24-hour urine collection for the Bence Jones protein (ie, lambda light chains)

  22. Images Radiographic and CT Scan

  23. Images MRI

  24. Images PET /CT Scan

  25. DiagnosisCriteria

  26. Light chain myeloma/ Non-secretory myeloma 

  27. 国际骨髓瘤工作组诊断标准 29

  28. ISS Predicts Both EFS and OS

  29. 梅奥中心：mSMART 2.0分层建议 • mSMART, 梅奥骨髓瘤分层和危险调整治疗； • FISH,荧光原位杂交；GEP, 基因表达谱 内部资料，仅供医学药学专业人士参考，严禁翻印及传播 Mlkhael JR et al. Mayo Clin Proc. 2013;88:360

  30. Initial therapy: Drugs & Combinations 烷化剂 (melphalan and CTX) 糖皮质激素 (Pred，DXM) 蛋白酶体抑制剂(Velcade, carfilzomib) 免疫调节剂(Thal, lenalidomide) 蒽环类(DOX and liposomal) 33

  31. Thomas Quasthoff

  32. Novel Agents: Lenalidomide plus DXM OS TTP Meletios Dimopoulos, et al. N Engl J Med 2007; 357:2123-2132

  33. Autologous Transplantation NEJM 2003; 348: 1875.

  34. ASCT Overall survival with maintenance thalidomide

  35. mSMART分层治疗策略-移植适合者 内部资料，仅供医学药学专业人士参考，严禁翻印及传播 Vincent Rajkuma.Am J Hematol.2014 Oct;89(10):999-1009.

  36. mSMART分层治疗策略-非移植适合者 内部资料，仅供医学药学专业人士参考，严禁翻印及传播 Vincent Rajkuma.Am J Hematol.2014 Oct;89(10):999-1009.

  37. THANKS

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