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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.