Plasma cell disorders ´ëÀå°æ Á¤¸®
1. multiple myelomaÀÇ °¡Àå ÈçÇÑ Áõ»óÀº bone pain(70%)À̸ç Áö¼ÓÀûÀÎ ±¹¼ÒÅëÁõÀº ´ë°³
°ñÀýÀÌ µ¿¹ÝµÇ¾úÀ½À» ÀǹÌÇÑ´Ù.
2. OAF production -> osteoclast activityÁõ°¡
ÃËÁø: IL-1, lymphotoxin, TNF
¾ïÁ¦: glucocorticoid, IFN-¥ã
3. Á¾¾çÀº »À³ª °ñ¼ö¿¡ ÁַΠħ¹üÇϸç splenomeglay, lymphadenopathy´Â ÈçÄ¡ ¾Ê´Ù.
anemia´Â ÈçÇϳª thrombocytopenia, neutropeia´Â ¸Å¿ì µå¹°´Ù.
* anemiaÀÇ ¿øÀÎ(±âÀü) 4°¡Áö
i) tumor cell expansion
ii) humoral factor
iii) hemolysis
iv) folate & vit B12 deficiency
4. neurologic sxÀÇ ¿øÀÎ: hypercalcemia, hyperviscosity,
bony damage & collapse(-> cord compression)
peripheral nerve infiltration of amyloid
5. renal failure(25%)ÀÇ ¿øÀÎ
hypercalcemia(mc), amyloid deposition, hyperuricemia, recurrent infection
myeloma cell deposit, light chain
6. plasma cell proliferation½ÃŰ´Â cytokine: IL-6
7. poor prognostic factor
¥â2-microglobulin¡è, BM plasma cell labeling index¡è
CRP¡è, LDH¡è, IL-6¡è
cytogenetic abnormality: 13(-), 11q abnormality, any translocation
advanced age, PB»ó myeloma cell, high thymidine kinase, plasmablastic morphology
cf. CRP´Â IL-6¾Æ correlation
8. °¡Àå Áß¿äÇÑ ¿¹ÈÄÀÎÀÚ 2°¡Áö: ¥â2 microglobulin, plasma cell albeling index
9. Multiple myeloma staging
I: Hb > 10g/dL, s-Ca < 12 mg/dL, normal one X-ray or solitary lesion
low M-component(IgG <5g/dL, IgA <3g/dL, light chain <4 g/24hr urine)
III: Hb < 8.5, Ca >12, advanced lytic bone lesion
high M component production(IgG >7 g/dL, IgA >5g/dL, light chain > 12g/24hr)
10. Ä¡·á: systemic chemotherapy(alkylating agent + Pd)
Áõ»óÀÌ ¸ÕÀú È£Àü(bone pain, anemia, hypercalcemia, infection)
M-component´Â 4-6ÁÖÈÄ¿¡¾ß °¨¼ÒÇϱ⠽ÃÀÛÇÔ, light chainÀº 1ÁÖ¸¸¿¡ °¨¼Ò
* maintenance therapy: IFN-¥á
* primary therapy¿¡ resistantÇÒ ¶§ : high-dose steroid pulse therapy or VAD
11. auto-PBSCTÀÇ ±Ý±â
:long-term chemotherapy, refractory multiple myeloma
12. multiple myelomaÀÇ Ä¡·á¿¡¼ plasmapheresis°¡ µµ¿òÀÌ µÇ´Â °æ¿ì?
hyperviscosity syndrome, light chain nephropathy
13. multiple myeoma¿¡¼ M-peak°¡ ¾øÀ» °æ¿ì?
light chain disease, nonsecretory myeloma, IgD myeloma
14. smoldering myeloma
M-protein > 3 g/dL, plasma cell in BM >10%
no anemia, renal insufficiency, skeletal lesions
15. IgD myeloma(1%)
plasma cell leukemia·Î ÀÌÇàÀÌ ÀߵǸç, renal failure°¡ ÈçÈ÷ ³ªÅ¸³ª ¿¹Èİ¡ ´Ù¸¥ typeº¸´Ù
³ª»Ú´Ù.
¥ë light chain typeÀÇ B-J proteinuria°¡ ÈçÇÏ´Ù.
amyloidosis¿Í extramedullary plasmacytosisµµ ºó¹øÇÏ´Ù.
* Á¾¾ç¿¡¼ ºÐºñµÇ´Â IgÀÇ ¹Ý°¨±â´Â ¾à 2.8ÀÏÀÌ´Ù.
16. MGUS(monoclonal gammopathy of undetermined significance)
monoclonal gammopathy
M-component level(IgG <3.5, IgA <2.5, B-J protein <1)
BM plasma cells < 10%
no bone lesions
no symptoms
17. Waldenstrom's macroglobulinemia
IgM > 3 g/dL
no bone lesion, hypercalcemia(-), renal disease not common
ÁÖ·Î hyperviscosity syndromeÀ» Àß ÀÏÀ¸Å´
80%°¡ kappa type
* lymphadenopathy, hepatosplenomegaly¿Í ¿¬°üµÇ´Â °æ¿ì°¡ ¸¹À½
°í·ÉÀÇ ³²ÀÚ¿¡¼ ÈçÇÏ´Ù.
positive Coomb's test°¡ ÈçÇÔ
18. plasma cell leukemia(PB plasma cell >20%)
primary : leukemic phase(60%)
secondary: MM¿¡¼ leukemia·Î transformation(40%)
primary°¡ secondaryº¸´Ù ´Ù¼Ò ³´´Ù(7°³¿ù vs 1°³¿ù »ýÁ¸)
Ä¡·á> unsatisfactory. secondary´Â °ÅÀÇ ¹ÝÀÀ ¾ÈÇÔ.
19. Myeloma variant
30%¹Ì¸¸¿¡¼ M-component¿Í °ü·Ã
ÀþÀº ¿¬·É, 10³âÀÌ»ó »ýÁ¸
extramedullary plasmacytoma - nasopharynx or PNS
highly response to RT
solitary bone plasmacytoma´Â ´Ù¸¥ bone site¿¡ Àç¹ßÇϰí, myeloma·Î ÁøÇàÇϱ⵵ ÇÑ´Ù.
extramedullary plasmacytoma´Â °ÅÀÇ Àç¹ßÇÏÁö ¾Ê°í myeloma·Î ÁøÇàÇÏÁö ¾Ê´Â´Ù.