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Gamopati Monoklonal: Diah Puspita Rini, DR., SPPK

This document discusses multiple myeloma, a type of hematological disorder caused by abnormal plasma cells that produce monoclonal paraprotein. It begins by defining multiple myeloma and related conditions. It then describes how paraproteinemia can cause issues like decreased normal immunoglobulin production, kidney dysfunction, and increased blood viscosity. The document outlines the clinical features of multiple myeloma including effects on bone, marrow, immunoglobulin secretion and laboratory abnormalities. It closes by listing the diagnostic criteria for multiple myeloma.

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Hammadum Irnah
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313 views16 pages

Gamopati Monoklonal: Diah Puspita Rini, DR., SPPK

This document discusses multiple myeloma, a type of hematological disorder caused by abnormal plasma cells that produce monoclonal paraprotein. It begins by defining multiple myeloma and related conditions. It then describes how paraproteinemia can cause issues like decreased normal immunoglobulin production, kidney dysfunction, and increased blood viscosity. The document outlines the clinical features of multiple myeloma including effects on bone, marrow, immunoglobulin secretion and laboratory abnormalities. It closes by listing the diagnostic criteria for multiple myeloma.

Uploaded by

Hammadum Irnah
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PPTX, PDF, TXT or read online on Scribd
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GAMOPATI

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

Paraproteinemia
PARAPROTEINEMIA

MENYEBABKAN :

1. Penurunan produksi imunoglobulin normal

2. Gangguan fungsi ginjal

3. Faktor pembekuan terganggu

4. Viskositas plasma meningkat


Plasma Cells

The bone marrow aspirate in multiple myeloma


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
 ♂>♀
FAKTOR RESIKO MM
 Exposure to pesticides
 Radiation
 Benzene
 ? Presence of IL-6 and HHV8 (Kaposi’s
sarcoma Herpes Virus)
Multiple Myeloma
Klinis:

TUMOR PARAPROTEINEMIA
EFFECTS
Bone lession
EFFECTS
Renal failure
Marrow Immune
failure suppression
Plasmacytom Hiperviscosity
a Amyloid
Multiple Myeloma

Tumor Effects
Neoplasm of C: hypercalcemia from
plasma cells in bone bone resorption
marrow which R: renal failure caused by
secretes a free light chain (myeloma
monoclonal kidney)
immunoglobulin A: anemia from marrow
failure
B: bone lesions
Multiple Myeloma (Plasma Cell
Myeloma) Ig secretion
52% Ig G
18% Ig A
11% Ig M
1% Ig D
rare Ig E
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
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
The amount of paraprotein produced is roughly proportional to
the size of the proliferating cells
www.freelivedoctor.com
Plasma Cells
Rouleoux formation

Lytic bone
lesion
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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