¼±Åà - È­»ìǥŰ/¿£ÅÍŰ ´Ý±â - ESC

 

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