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.