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Gamopati monoklonal

Gamopati monoklonal. Diah Puspita Rini, dr., SpPK. Suatu kelainan hematologi yang berasal dari limfosit yang menghasilkan paraprotein (globulin gamma) yang bersifat monoklonal. Multiple Myeloma Waldenstorm Macroglobulinemia Monoclonal Gammopathy of Undetermined Significance.

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Gamopati monoklonal

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  1. Gamopati monoklonal Diah Puspita Rini, dr., SpPK

  2. Suatu kelainan hematologi yang berasal dari limfosit yang menghasilkan paraprotein (globulin gamma) yang bersifat monoklonal

  3. Multiple Myeloma • Waldenstorm Macroglobulinemia • Monoclonal Gammopathy of Undetermined Significance Paraproteinemia

  4. PARAPROTEINEMIA MENYEBABKAN : • Penurunan produksi imunoglobulin normal • Gangguan fungsi ginjal • Faktor pembekuan terganggu • Viskositas plasma meningkat

  5. Multiple Myeloma • Proliferasi neoplastik sel plasma • KHAS: Lesi osteolitik Akumulasi sel plasma abnormal dalam sutul Protein monoklonal dalam serum dan urine • Epidemiologi: • Insiden 3/100.000 pertahun • Sebagian besar umur 50-70 tahun, jarang < 40 th • ♂ > ♀

  6. FAKTOR RESIKO MM • Exposure to pesticides • Radiation • Benzene • ? Presence of IL-6 and HHV8 (Kaposi’s sarcoma Herpes Virus)

  7. Multiple Myeloma Klinis: TUMOR EFFECTS PARAPROTEINEMIA EFFECTS Renal failure Immune suppression Hiperviscosity Amyloid Bone lession Marrow failure Plasmacytoma

  8. Multiple Myeloma

  9. Multiple Myeloma (Plasma Cell Myeloma) Ig secretion 52% Ig G 18% Ig A 11% Ig M 1% Ig D rare Ig E

  10. LABORATORIUM (1) • Anemia : normokromik normositik • Rouleaux pada hapusan darah, kadang dijumpai sel plasma di darah tepi • LED meningkat • Fase lanjut: leukopenia dan trombositopenia • Sutul: sel plasma > 10%, sel myeloma yang ditemukan abnormal dengan inti besar, bizarre, ukuran bervariasi, sering multinukleus, infiltrasi sel plasma

  11. LABORATORIUM (2) • Elektroforesis protein: paraprotein (M-protein)yang membentuk “spike” pada daerah gamma • Urine: Bence jones protein • Kelainan faktor-faktor pembekuan Ureum-creatinin >> • ( Kegagalan fungsi ginjal ) Reabsorbsi Calcium meningkat • menyebabkan serum Ca ↑ Total Protein meningkat dengan rasio Albumin: Globulin terbalik

  12. The amount of paraprotein produced is roughly proportional to the size of the proliferating cells www.freelivedoctor.com

  13. Plasma Cells Rouleoux formation Lytic bone lesion

  14. DIAGNOSIS Dua dari tiga hal berikut harus ada: • paraproteinaemia atau Bence Jones protein • Radiologi: lytic bone lesions • Peningkatan sel plasma di darah tepi atau sumsum tulang

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