¼±Åà - È­»ìǥŰ/¿£ÅÍŰ ´Ý±â - ESC

 

Platelet disorder ´ëÀå°æ Á¤¸®

1. Gp Ib/IX¿¡ °áÇÕ: vWF(adhesion)

Gp IIb/IIIa : fibrinogen(aggregation)

2. vWFÀÇ ±â´É: platelet adhesion, factor VIIIÀÇ plasma carrier

3. Bernard-Soulier syndrome: Gp Ib°áÇÔÀ¸·Î platelet adhesionÀå¾Ö

vWD¿Í ÇÔ²² ristocetin¿¡ ÀÀ°í¾ÈµÊ

4. Ç÷¼ÒÆÇ »ý»êÀ» Áõ°¡½ÃŰ´Â cytokines: IL-3, 6, 11, thrombopoietin

cf. thrombopoietinÀÇ ºÎÀÛ¿ë: thrombosis, splenomegaly, myelofibrosis

neutralizing AbÃâÇö(thrombocytopenia) = °¡Àå ½É°¢ÇÑ ºÎÀÛ¿ë

flu-like sx, fatigue, fluid retentionÀº º¸°íµÇÁö ¾Ê¾Ò´Ù.

5. Thrombopoietin

erythropoietin°ú ±¸Á¶°¡ À¯»çÇÏ¿© BFU-EÀÇ Áõ½ÄÀ» Á¶ÀåÇÑ´Ù.

megakaryocyte¼ö¸¦ Áõ°¡½Ã۰í ploidy»óŸ¦ Áõ°¡½ÃŲ´Ù.

´Ù¸¥ cytokine(IL-3, SCF)µé°ú º´ÇÕÈ¿°ú°¡ ÀÖ´Ù.

6. c-mpl: thrombopoietinÀÌ °áÇÕÇÏ´Â receptor

CD34 cell, megakaryocyte, platelet

7. drug-induced thrombocytopenia

1) myelosuppression: alcohol, thiazide

2) immunologic destruction: phenytoin, gold, heparin, methyldopa

8. aspirinÀÇ anti-platelet fx ±âÀü

platelet granule release¾ïÁ¦, platelet aggregation inhibition

9. °áÇÙ¾àÁß¿¡¼­ thrombocytopeniaÀÏÀ¸Å°´Â °Í : rifampin

10. thrombocytopenia:

splenomegaly(+) : BM°¡ Á¤»óÀ̸é congested splenomegaly, liver disease, storage

disease

BMÀÌ»ó½Ã leukemia, lymphomaµî

splenomegaly(-) : BM°¡ Á¤»óÀ̸é excess destructionÀǽÉ

immune = ITP, drug-induced

nonimmune = vasculitis, DIC/sepsis, prosthesis

BM ÀÌ»ó½Ã hematologic disorder(production defect)

11. renal failure½Ã platelet dysfunction¿¡ ÀÇÇÑ bleeding tendency¶§ Ä¡·á

estrogen, DDAVP, cryoprecipitate

12. spleenÀÇ encapsulated bacteria ¹æ¾î±âÀü

spleen cordÀÇ meshwork, IgM»ý»ê, opsonin»ý¼º

13. splenectomyÈÄ¿¡ bacteriaÀ§Ç輺ÀÌ Ä¿Áö´Â ÀÌÀ¯

opsonized bacteriaÁ¦°ÅÀå¾Ö, T-cell independent Ab»ý»êÀå¾Ö

* viral infection¿¡ ´ëÇÑ °¨¼ö¼ºÀº Áõ°¡ÇÏÁö ¾Ê´Â´Ù.