Pure red cell aplasia(PRCA)
pure white cell aplasia¿Í amegakaryocytic thrombocytopenia´Â ±ØÈ÷ µå¹°¸ç PRCAó·³
destructive Ab or lymphocyte¿¡ ÀÇÇØ ¹ß»ýÇÏ¸ç ¸é¿ª¾ïÁ¦Ä¡·á¿¡ ¹ÝÀÀÇÑ´Ù. ÀÌ ¸ðµç single
lineage failure syndrome¿¡¼ pancytopenia³ª leukemia·ÎÀÇ ÁøÇàÀº µå¹°´Ù.
1) Á¤ÀÇ ¹× °¨º°Áø´Ü
anemia, reticulocytopenia, BM¿¡¼ erythroid precursor cellÀÇ °áÇÌÀÌ Æ¯Â¡ÀÌ´Ù.
*ºÐ·ù: Tab 109-4
i) ¼ºÀο¡¼± acquired
ii) Diamond-Blackfan anemia(=congenital PRCA)
: Ãâ»ý½Ã or early childhood¶§ Áø´ÜµÇ¸ç glucocorticoid¿¡ ¹ÝÀÀÇÑ´Ù.
iii) temporary red cell failure: acute parvoviral infection¿¡ ÀÇÇØ hemolytic anemiaÀÇ
transient aplastic crisis
iv) Á¤»ó ¾î¸°ÀÌ¿¡¼ transient erythroblastopenia
2) ÀÓ»óÀû °ü·Ã ¹× ¿øÀÎ
immune system disease¿Í µ¿¹ÝµÈ´Ù.
ÀϺδ thymoma¿¡¼ ¹ß»ýÇÏ°í º¸´Ù ÈçÇϱâ·Î´Â large granular lymphocytosisÀÇ major
manifestationÀϼö ÀÖ°í, CLL¿¡¼ ¹ß»ýÇϱ⵵ ÇÑ´Ù.
ÀϺδ hypogammaglobulinemic.
agranulocytosisó·³ ¾à¹°¿¡ ÀÇÇÑ idiosyncratic reaction¿¡ ÀÇÇØ ¹ß»ýÇϱ⵵ ÇÑ´Ù.
aplsatic anemiaó·³ ´Ù¾çÇÑ ±âÀüÀ¸·Î ¹ß»ýÇÑ´Ù.
RBC precursor¿¡ ´ëÇÑ Ç×ü°¡ ÈçÈ÷ ¹ß°ßµÇÁö¸¸ T cell inhibitionÀÌ ´õ ÈçÇÑ immune
mechanismÀÌ´Ù.
* persistent parvovirus B19 infection: transient aplsatic crisis¾ß±â
3) Ä¡·á
i) drug exposureÈ®ÀÎ -> Áß´Ü
ii) thymomaÀÖ´ÂÁö CXR·Î È®ÀÎ
ÀÖ´Ù¸é tumor excision, ±×·¯³ª ¼ö¼ú·Î ºóÇ÷ÀÌ ¹Ýµå½Ã ȸº¹µÇ´Â °ÍÀº ¾Æ´Ï´Ù.
iii) Parvovirus infectionÁø´Ü: viral DNA sequence detection(IgG & IgM Ab: ÈçÈ÷ absent)
IV IG(0.4 g/kg/d ¡¿ 5 days)¿¡ ´ëºÎºÐ ¹ÝÀÀÇÑ´Ù.
ºñ·Ï Àç¹ß°ú ÀçÄ¡·á°¡ ¿¹»óµÇÁö¸¸... ƯÈ÷ AIDS¿¡¼
iv) erythroid colony
erythroid colonyÀÇ Á¸Àç´Â idiopathic PRCA¿¡¼ ¸é¿ª¾ïÁ¦Ä¡·á¿¡ ¹ÝÀÀÇÒ °ÍÀÓÀ» ¿¹ÃøÇÒ ¼ö
ÀÖ´Ù.
v) PRCA´Â supportive care´Üµ¶À¸·Îµµ long survivalÇÑ´Ù.
transfusion & iron chelation
vi) idiopathic PRCA ´ëºÎºÐÀº ¸é¿ª¾ïÁ¦Ä¡·á¿¡ Àß µè´Â´Ù.
1st course of glucocorticoid
-> ¹ÝÀÀÀÌ ¾øÀ¸¸é cyclosporine, ATG, azathioprine or cyclophosphamide