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PARA PROTEINEMIAS

PARA PROTEINEMIAS. Sheikha. Professor Anwar Sheikha MD, FRCP, FRCPath ., FCAP, FRCPA, FRCPI, FACP Senior Consultant Clinical & Lab. Hematologist Clinical Professor of Hematology University of Mississippi Medical Center, Jackson, Mississippi Professor of Hematology,

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PARA PROTEINEMIAS

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  1. PARA PROTEINEMIAS Sheikha

  2. Professor Anwar Sheikha MD, FRCP, FRCPath., FCAP, FRCPA, FRCPI, FACP Senior Consultant Clinical & Lab. Hematologist Clinical Professor of Hematology University of Mississippi Medical Center, Jackson, Mississippi Professor of Hematology, University of Salahaddin, Erbil, Kurdistan, IRAQ

  3. PARAPROTEINEMIAS Sheikha

  4. Sheikha

  5. MULTIPLE MYELOMA PARA PROTEINEMIAS WALDENSTROM’S MACROGLOBULINEMIA PRIMARY AMYLOIDOSIS HEAVY CHAIN DISEASES M-GUS Sheikha

  6. ANEMIA BLEEDING INFECTION BONE PAIN # VERTEBRAL COLLAPSE LYTIC BONE LESIONS ORTHOPEDIC SURGEON HEMATOLOGIST NEPHRO- LOGISTS NEURO- LOGIST RENAL FAILURE HYPERVISCOSITY Sheikha

  7. 1% of All Cancers 2% of All Cancer Deaths MULTIPLE MYELOMA Average Age ~ 65 Black: White = 2:1

  8. MULTIPLE MYELOMA BONE MARROW INFILTRATION PARAPROTEIN PRODUCTION OSTEOLYTIC BONE LESIONS Sheikha

  9. ↓ PLATELET ANEMIA ↓ WBC PANCYTOPENIA BONE MARROW INFILTRATION MULTIPLE MYELOMA Sheikha

  10. Sheikha INFECTION BLEEDING ↓ PLATELET ↓ WBC ANEMIA PANCYTOPENIA IMMUNE SUPPRESSION BONE MARROW INFILTRATION MULTIPLE MYELOMA

  11. INFECTION  Chemotherapy myelosuppression  Steroid immunosuppression MULTIPLE MYELOMA ↓ WBC PANCYTOPENIA IMMUNE SUPPRESSION BONE MARROW INFILTRATION Sheikha

  12. MULTIPLE MYELOMA ANEMIA BONE PAIN OSTEOLYTIC BONE LESIONS BONE # VERTEBRAL COLLAPSE ↑ Ca++ RENAL FAILURE Sheikha

  13. MULTIPLE MYELOMA ANEMIA HEMO- DILUTION PARAPROTEIN PRODUCTION HYPER VISCOSITY CNS SYMPTOMS Sheikha

  14. MULTIPLE MYELOMA INTERFERENCE WITH CLOTTING FACTORS BLEEDING ANEMIA PARAPROTEIN PRODUCTION COATING OF PLATELETS Sheikha

  15. MULTIPLE MYELOMA LIGHT CHAINS PARAPROTEIN PRODUCTION RENAL FAILURE AMYLOID Sheikha

  16. INFECTION ?RENAL FAILURE PYELONEPHRITIS LIGHT CHAINS ↑ Ca++ RENAL FAILURE AMYLOID MULTIPLE MYELOMA Sheikha

  17. ?BLEEDING PARAPROTEIN INTERFERENCE WITH CLOTTING FACTORS BONE MARROW INFILTRATION BLEEDING MULTIPLE MYELOMA PARAPROTEIN COATING OF PLATELETS Sheikha

  18. MULTIPLE MYELOMA ?ANEMIA BLEEDING BONE MARROW INFILTRATION HEMO- DILUTION RENAL FAILURE Sheikha

  19. BONE MARROW INFILTRATION Sheikha OSTEOLYTIC BONE LESIONS PARAPROTEIN PRODUCTION INFECTION BLEEDING RENAL FAILURE MULTIPLE MYELOMA BONE PAIN, # & VERT. COLLAPSE ANEMIA HYPER- VISCOSITY ↑ Ca++

  20. Sheikha

  21. Rouleaux ↑ESR

  22. The cytoplasm of Myeloma Cells contains abundant Endoplasmic Reticulum (ER) , which may contain retained, condensed or crystallised cytoplasmic Ig producing a variety of morphologically distinctive findings, including: Multiple pale bluish-white, grape-like accumulation  Mott or Morula Cells Cherry-red refractive round bodies  Russell Bodies Vermilion staining glycogen-rich IgA  Flame Cells Overstuffed fibrils  Gaucher-like cells; thesaurocytes & Crystalline Rods THESE CHANGES ARE NOT PATHOGNOMONIC FOR MM SINCE THEY MAY BE FOUND IN REACTIVE PLASMA CELLS

  23. IgG >50% Sheikha MULTIPLE MYELOMA IgA 25% Light Chain 20% Bi-clonal IgD rare Non- Secretory ? IgM

  24. NORAMAL

  25. Immunofixation performed on serum from a patient with monoclonal immunoglobulin Gk (IgGk) & a patient without a monoclonal protein IgG k normal

  26. Sheikha

  27. Sheikha

  28. OAF (IL-1/ TNF) OSTEOCLASTS PLASMA CELLS PDGF/ IL-6 IL- 6 BMSC “Bone Marrow Stromal Cells” Sheikha

  29. Sheikha

  30. ﺣﺮﺱ ﺍﻝﺠﻤﻫﻭﺭﻱ ﺻﺪﺍﻡ Sheikha

  31. Sheikha

  32. PC Osteoclasts Sheikha

  33. Interleukin-6-mediated myeloma cell growth BMSC: bone marrow stromal cell IL: interleukin NF: nuclear factor TGF: transforming growth factor MM rely on contact with BM Stromal Cells “BMSC” Adhesive interaction between MM cells & BMSC induce cells to secrete IL-6 which then acts a paracrine growth factor promoting survival of MM cells & inhibiting apoptosis

  34. IL-1 β TGF- β Other Cytokines Osteoclast Activation Osteoblast Suppression OSTEO- LYTIC BONE LESIONS OAF

  35. STAGING OF MYELOMA Sheikha 1 trillion PC (1012) = 1 Kg I < 1 Kg PC III > 2 Kg PC II 1 to 2 Kg PC HIGH CELL MASS >1.2 X 1012/m2 LOW CELL MASS <0.6 x 1012/m2

  36. Durie-Salmon Myeloma Staging System Subclassification: a: Relatively normal renal function (serum creatinine value <2.0 mg/dL) b: Abnormal renal function (serum creatinine >2.0 mg/dL) Sheikha

  37. Durie-Salmon Myeloma Staging System < 1 Kg PC 1 to 2 Kg PC > 2 Kg PC Subclassification: a: Relatively normal renal function (serum creatinine value <2.0 mg/dL) b: Abnormal renal function (serum creatinine >2.0 mg/dL) Sheikha

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