¼±Åà - È­»ìǥŰ/¿£ÅÍŰ ´Ý±â - ESC

 

I. IDA & other hypoproliferative anemias

1. hypoproliferative anemia

1) Á¤ÀÇ : normocytic and normochromic red cell +

abnormally low reticulocyte response(reti < 2.5)

2) ¹üÁÖ

i) early iron deficiency(hypochromic, microcytic red cellsÀÌ ¹ß»ýÇϱâ Àü´Ü°è)

ii) acute & chronic inflammation(´Ù¾çÇÑ malignancyÆ÷ÇÔ)

iii) renal disease

iv) hypometabolic states

: protein malnutrition, endocrine deficiencies, marrow damage·Î ÀÎÇÑ ºóÇ÷ µî

2. Iron metabolism

1) Á¤»óöºÐÆ÷ Tab 105-1

2) ÀÏÀÏöÇʿ䷮

³²: 1mg °¡Àӱ⠿©¼º: 1.4mg

3) öÀÇ Èí¼ö: proximal duodenum

Èí¼ö¹æÇØÀÎÀÚ: phytate, phosphate

Èí¼ö¼¼Æ÷ÀÇ brush border¿¡ ÀÖ´Â ferrireductase¿¡ ÀÇÇØ

ferric iron(Fe+++) -> ferrous iron(Fe++)À¸·Î ÀüȯµÈÈÄ Èí¼öµÈ´Ù.

°¢°¢ÀÇ À½½Ä¿¡¼­ Èí¼öµÇ´Â öÀÇ ¾çÀº ferrous salt(Á¦1ö, F++)ÀÇ ¾ç¿¡ ºñ·ÊÇÑ´Ù. ä¼Ò¿¡ ÇÔÀ¯

µÈ öÀº ´ÜÁö 1/20¸¸ Èí¼öµÇ°í, ´Þ°¿ÀÇ °æ¿ì´Â 1/8, °£Àº 1/2, hemeÀº 1/2-2/3°¡ Èí¼öµÈ´Ù.

±×·¯¹Ç·Î °£°ú hemeÀÇ Ã¶Àº À½½Ä¿¡ Æ÷ÇÔµÈ °Í °ÅÀǰ¡ Èí¼öµÇÁö¸¸ ä¼Ò³ª ´Þ°¿ÀÇ Ã¶Àº ´ú

Èí¼öµÈ´Ù. À¯¾Æ, ¾î¸°ÀÌ ¹× û¼Ò³âÀº ¼ºÀå±âÀÇ ¸¹Àº ¼ö¿ä¿Í ¼·Ãë ºÒÃæºÐÀ¸·Î ÀÎÇÏ¿© Á¤»óÀû

ÀΠö ±ÕÇüÀ» À¯ÁöÇÒ¼ö ¾ø´Ù. ÀӽŠ2, 3±â¶§ ÀÏÀÏ Ã¶ ¿ä±¸·®Àº 5-6mg±îÁö Áõ°¡ÇÑ´Ù. ÀÌ´Â

¼±Áø±¹ Àӽſ©¼º¿¡¼­ °ÅÀÇ º¸ÆíÀûÀ¸·Î öÀÌ Ã³¹æµÇ´Â ÀÌÀ¯ÀÌ´Ù.

4) Á¤»ó¹üÀ§

serum iron 50-150 ug/dL

TIBC 300-360 ug/dL

TS 25-50%

cf. TS = serum iron/TIBC ¡¿ 100

5) serum ferritin

* Á¤»óÄ¡: ¼ºÀÎ ³²¼ºÀº Æò±Õ 100 ug/L, ¿©¼ºÀº 30 ug/L

¾ÈÁ¤»óÅ¿¡¼­ serum ferritin levelÀº ÀúÀåöÀ» Æò°¡Çϱâ À§ÇÑ °¡Àå °£ÆíÇÑ °Ë»çÀÌ´Ù.

°ñ¼ö¿¡¼­ ö ¿°»öÀ¸·Î RE cell ÀúÀåöÀ» Æò°¡ÇÒ ¼ö´Â ÀÖÁö¸¸ serum ferritinÀÇ ÃøÁ¤À¸·Î °ñ¼ö

ÈíÀÎÀ» ´ë½ÅÇÒ¼ö ÀÖ´Ù(Tab 105-3). serum ferritin levelÀº °ñ¼ö ö ¿°»öº¸´Ù iron overloadÀÇ

´õ ÁÁÀº Ç¥ÁöÀÚÀÌ´Ù.

3. IDA

1) microcytic & hypochromic anemiaÀÏ ¶§ IDA¿Ü¿¡ °í·ÁÇØ¾ß ÇÒ »óȲ

2) Stages of iron deficiency Fig 105-2

¨ç negative iron balance

ù´Ü°è´Â "negative iron balance"·Î ö¿ä±¸·®ÀÌ Èí¼ö ö·®À» ÃʰúÇÏ´Â ´Ü°èÀÌ´Ù.

ÀÌ ´Ü°è´Â ½ÇÇ÷, ÀÓ½Å, û¼Ò³â±â, ºÎÁ·ÇÑ ¼·Ãë¿Í °°Àº »ý¸®Àû ±âÀü¿¡ ÀÇÇØ ¹ß»ýÇÑ´Ù.

°¡Àå ÈçÇϱâ·Î´Â žƳª ±Þ¼ÓÈ÷ ¼ºÀåÇÏ´Â ¾ÆÀÌ·Î HbÇÕ¼ºÀ» À§ÇØ ÇÊ¿äÇÑ Ã¶ÀÇ ¾çÀº

°³°³ÀÎÀÇ Ã¶ Èí¼ö´É·ÂÀ» ÃʰúÇÑ´Ù.

ÇÏ·ç 10-20mlÀÌ»óÀÇ ½ÇÇ÷Àº Á¤»ó ½ÄÀ̷κÎÅÍ Èí¼öµÉ¼ö Àִ öÀÇ ¾çÀ» ÃʰúÇÑ´Ù.

ÀÌ·± »óȲ¿¡¼­ ö °áÇÌÀº RE storage site¿¡¼­ öÀÇÀ̵¿¿¡ ÀÇÇØ º¸ÃæµÇ¾î¾ß ÇÑ´Ù.

ÀÌ ½Ã±â¿¡ serum ferritin level ȤÀº °ñ¼öÈíÀο¡¼­ ¿°»ööÀÇ Á¸Àç¿Í °°Àº ¹æ¹ýÀ¸·Î

ö ÀúÀå´ÉÀ» ÃøÁ¤Çغ¸¸é °¨¼ÒÇØ ÀÖ´Ù.

ö ÀúÀåÀÌ Á¸ÀçÇϰí À̵¿ÇÒ¼ö ÀÖ´Â ÇÑ, serum iron, TIBC, red cell protoporphyrin levelsÀº

Á¤»ó¹üÀ§ÀÌ´Ù. ¶ÇÇÑ RBC morpholology & indexµµ Á¤»óÀÌ´Ù. ö ÀúÀåÀÌ °í°¥µÇ¸é, serum

ironÀº °¨¼ÒÇϱ⠽ÃÀÛÇÑ´Ù. Á¡Â÷ red cell protoporphyrin levelsó·³ TIBC´Â Áõ°¡ÇÑ´Ù.

¨è iron deficient erythropoiesis

Á¤ÀÇ¿¡ ÀÇÇϸé, serum ferritin levelÀÌ 15 ug/L¹Ì¸¸ÀÌ¸é °ñ¼ö ö ÀúÀåÀº ¾ø´Ù.

serum ironÀÌ Á¤»ó¹üÀ§¿¡ ÀÖ´Â ÇÑ Ã¶ ÀúÀå´ÉÀÌ °¨¼ÒÇÔ¿¡µµ ºÒ±¸Çϰí HbÇÕ¼ºÀº ¿µÇâÀ»

¹ÞÁö¾Ê´Â´Ù. ÀÏ´Ü transferrin saturation(TS)ÀÌ 15-20%±îÁö ¶³¾îÁö¸é HbÇÕ¼ºÀº ¼Õ»óµÈ´Ù.

ÀÌ ½Ã±â´Â iron-deficient erythropoiesis½Ã±âÀÌ´Ù.

PB smearÇϸé, microcytic cellsÀÌ Ã³À½ ÃâÇöÇÑ´Ù.

¨é iron deficiency anemia

Á¡Â÷, Hb & HctÀº °¨¼ÒÇϱ⠽ÃÀÛÇÏ¿© IDA°¡ µÈ´Ù. À̶§ TS´Â 10-15%ÀÌ´Ù.

ÁߵÀÇ ºóÇ÷À϶§(Hb 10-13g/dL) °ñ¼ö´Â hypoproliferative»óÅÂÀÌ´Ù.

º¸´Ù ºóÇ÷ÀÌ ½ÉÇØÁö¸é(Hb 7-8 g/dL), hypochromia & microcytosis´Â º¸´Ù Àú¸íÇØÁö°í,

misshapen red cells(poikilocytes)´Â PB¿¡¼­ cigar or pencil-shaped forms & target

cells·Î º¸À̸ç erythroid marrow´Â ineffectiveÇÏ°Ô µÈ´Ù. °á°úÀûÀ¸·Î severe prolonged

IDA¿¡¼­´Â hypoproliferationº¸´Ù´Â erythroid marrow hyperplasia°¡ ¹ß»ýÇÑ´Ù.

ö°áÇ̽à saturation levelÀº 18%¹Ì¸¸ÀÌ´Ù. serum ironÀ» ÃøÁ¤ÇÒ¶§´Â diurnal variationÀÌ ÀÖÀ½

À» ¾Ë¾Æ¾ß ÇÑ´Ù. TS > 50%´Â transferrin¿¡ °áÇÕµÈ Ã¶ÀÇ ¾çÀÌ ºñÁ¤»óÀûÀ¸·Î ¸¹À½À» ÀǹÌÇÑ´Ù.

ÀÌ »óŰ¡ ¿À·¡ Áö¼ÓÇϸé, tissue iron overload°¡ ¹ß»ýÇÑ´Ù.

4. IDAÀÇ Ä¡·á

IDAÀÇ ÁßÁõµµ ¹× ¿øÀο¡ µû¶ó Ä¡·á¹æÄ§À» °áÁ¤ÇÑ´Ù.

¿¹¸¦ µé¸é, severe IDA¸¦ °¡Áø Áõ»óÀÖ´Â ³ëÀÎȯÀÚ¿Í cardiovascular instability°¡ ÀÖÀ¸¸é

¼öÇ÷ÀÌ ÇÊ¿äÇÏ´Ù. ºóÇ÷¿¡ ´ëÇØ º¸»óÇÒ¼ö ÀÖ´Â ÀþÀº »ç¶÷Àº iron replacement·Î º¸Á¸ÀûÀ¸·Î

Ä¡·áÇÒ¼ö ÀÖ´Ù.

ÈÄÀÚÀÇ °¡Àå Å« À̽´´Â ö°áÇÌÀÇ Á¤È®ÇÑ ¿øÀÎÀ» ÆÄ¾ÇÇÏ´Â °ÍÀÌ´Ù. ö°áÇÌ ´ëºÎºÐ(Àӽſ©¼º,

¼ºÀå±â û¼Ò³â, °£ÇæÀû ÃâÇ÷, ºÒÃæºÐÇÑ Ã¶¼·Ãë)Àº oral iron tx·Î ÃæºÐÇÏ´Ù.

1) red cell transfusion

i) Áõ»óÀÖ´Â °æ¿ì

ii) cardiovascular instability

iii) continued & excessive blood loss

À̶§´Â Áï°¢ ¼öÇ÷ÀÌ ÇÊ¿äÇÏ´Ù.

2) oral iron tx

¹«Áõ»ó ȯÀÚ¿¡´Â oral iron tx¸¸À¸·Î ÃæºÐÇÏ´Ù.

simple iron salt¿¡¼­ ¼ÒÀå¿¡¼­ Áö¼ÓÀûÀ¸·Î À¯¸®µÇµµ·Ï ¼³°èµÈ complex iron compounds

±îÁö ´Ù¾çÇÏ°Ô ³ª¿ÍÀÖ´Ù.

Tab 105-5 oral iron preparations

ÀüÇüÀûÀ¸·Î iron replacement tx¸¦ À§Çؼ­´Â ¸ÅÀÏ Çʼöö 300mgÀÌ ÇÊ¿äÇϸç 50-65 mg

elemental ironÀÌ ÇÔÀ¯µÈ öºÐÁ¦Á¦ 3-4Á¤ÀÇ º¹¿ëÀÌ ÇÊ¿äÇÏ´Ù. ÀÌ»óÀûÀ¸·Î´Â °øº¹½Ã º¹¿ë

ÇØ¾ß ÇÑ´Ù. ¿Ö³ÄÇϸé À½½ÄÀÌ Ã¶Èí¼ö¸¦ ¹æÇØÇϱ⠶§¹®ÀÌ´Ù.

Ä¡·á¸ñÇ¥´Â ºóÇ÷»óÅÂÀÇ È¸º¹ »Ó¸¸ ¾Æ´Ï¶ó ÀûÀ¸·Î ö 0.5-1gÀ» ÀúÀåÇϴµ¥ ÀÖ´Ù. À̸¦ À§ÇØ

ºóÇ÷ÀÌ ±³Á¤µÈ ÈÄ Áö¼ÓÀûÀ¸·Î 6-12°³¿ù Ä¡·á°¡ ÇÊ¿äÇÏ´Ù.

oral iron txÇÕº´Áõ Áß¿¡¼­ GI distress°¡ °¡Àå µÎµå·¯Áö¸ç, ȯÀÚÀÇ 15-20%¿¡¼­ ¹ß°ßµÈ´Ù.

ÀÌ·± ȯÀڵ鿡°Ô¼­ abdominal pain, nausea, vomiting, constipationÀº noncompliance¸¦

¾ß±âÇÑ´Ù. ºñ·Ï ¼Ò·®ÀÇ Ã¶°ú öºÐÁ¦Á¦°¡ ´Ù¼Ò µµ¿òÀÌ µÇÁö¸¸ GI side effect´Â ¸¹Àº ȯÀÚ

µé¿¡°Ô¼­ È¿°úÀû Ä¡·á¸¦ ÀúÇØÇÏ´Â ÁÖ ¿øÀÎÀÌ´Ù. ÀüÇüÀûÀ¸·Îreti count´Â 4-7ÀÏ¿¡ Áõ°¡Çϱâ

½ÃÀÛÇÏ¿© 1.5ÁÖ(10ÀÏ)°æ¿¡ ÃÖ°í¿¡ µµ´ÞÇÑ´Ù. ¹ÝÀÀÀÌ ¾ø´Â °æ¿ì´Â Èí¼ö°¡ ¾ÈµÇ°Å³ª

noncompliance(more common) ȤÀº À߸øµÈ Áø´Ü¿¡ ÀÇÇÑ´Ù. ö°áÇÌÀÌ Áö¼ÓµÇ¸é ÁÖ»çÁ¦·Î

ÀüȯÇÒ Çʿ䰡 ÀÖ´Ù.

3) parenteral iron therapy

°æ±¸Á¦¿¡ °áµô¼ö ¾øÀ»¶§ »ç¿ëÇÒ¼ö ÀÖ´Ù. º¸Åë IV°¡ »ç¿ëµÈ´Ù.

* Á¾·ù : IV iron dextran, IV iron gluconate

* µÎ°¡Áö »ç¿ë ¹æ¹ýÀÌ ÀÖ´Ù.

i) HbÀ» ±³Á¤Çϱâ À§ÇØ ÇÊ¿äÇÑ ÃÑ·®À» Åõ¿©Çϰí

ÀúÀåöÀ» À§ÇØ Àû¾îµµ 500mgÀ» ´õ Åõ¿©ÇÏ´Â ¹æ¹ý

ii) ¼Ò·®ÀÇ parenteral ironÀ» ¿À·£±â°£µ¿¾È ¹Ýº¹Åõ¿©ÇÏ´Â ¹æ¹ý

Åõ¼®¼¾ÅÍ¿¡¼­ recombinant erythropoietin tx¿¡ ´ëÇÑ ¹ÝÀÀÀ» ³ôÀ̱â À§ÇØ µÎ ¹øÂ° ¹æ¹ýÀÌ

ÈçÈ÷ »ç¿ëµÇ´Âµ¥ ÁÖ´ç elemental iron 100mg ¡¿ 10ÁÖ¸¦ Åõ¿©ÇÑ´Ù.

ÇÊ¿äÇÑ Ã¶ÀÇ ¾çÀº ´ÙÀ½ °ø½Ä¿¡ ÀÇÇØ °è»êÇÒ¼ö ÀÖ´Ù.

B.W(kg) ¡¿ 2.3 ¡¿ (15-pt's Hb) + 500 or 1000 mg

Á¤¸ÆÀ¸·Î öÀ» Åõ¿©ÇÒ ¶§ anaphylaxis¿¡ Ç×»ó ÁÖÀÇÇØ¾ß ÇÑ´Ù.

»õ·Î¿î Á¦Á¦¿¡¼­ anaphylaxis´Â ´ú ÈçÇÏ´Ù.

* serious anaphylactic-like reaction°ú °ü°èµÈ ÀÎÀÚ

i) multiple allegies Hx

ii) prior allergic reaction to dextran

´Ù·®ÀÇ Ã¶ºÐ Åõ¿©ÈÄ ¼öÀϳ» Àü½ÅÁõ»óÀ¸·Î arthralgias, skin rash, low-grade fever°¡ Àִµ¥

ÀÌ´Â ¿ë·®¿¡ ºñ·ÊÇÑ´Ù.

ÃÖ±Ù, iron dextran¿¡ ´ëÇÑ sensitivity¸¦ °¡Áø ȯÀÚ´Â iron gluconate>100 mgÀ» Åõ¿©ÇÒ¶§´Â

5% D/W or 0.9% NaCl solution¿¡ Èñ¼®ÇØ¾ß Çϸç 60-90ºÐÀÌ»ó ¼­¼­È÷ ÁÖÀÇÇÏ¿© Åõ¿©Çؾß

ÇÑ´Ù.

test dose(25mg)°¡ ÇÊ¿äÇÏ´Ù. öÅõ¿©Ãʱ⿡ chest pain, wheezing, Ç÷¾ÐÇϰ­ ȤÀº ´Ù¸¥

Àü½ÅÁõ»óÀÌ ¹ß»ýÇϸé ö Åõ¿©¸¦ Áï½Ã Áß´ÜÇØ¾ß ÇÑ´Ù.

5. Anemia of acute & chronic inflammatory infection(The anemia of chronic disease)

ÀÓ»ó¿¡¼­ º¼¼ö ÀÖ´Â °¡Àå ÈçÇÑ ºóÇ÷Áß ÇϳªÀ̰í IDA °¨º°Áø´Ü¿¡¼­ °¡Àå Áß¿äÇÏ´Ù. ¿Ö³ÄÇϸé

¸¹Àº ºóÇ÷ÀÌ ÀúÀåöÀº Á¤»ó ȤÀº Áõ°¡µÇ¾î ÀÖÀ¸³ª °ñ¼ö·ÎºÎÅÍ Ã¶À̵¿ÀÌ ºÎÀûÀýÇϱ⠶§¹®

ÀÌ´Ù.

serum ferritin ¼öÄ¡´Â true IDA¿Í inflammation°ú °ü·ÃÇÑ iron-deficient erythropoiesis¸¦

±¸º°Çϴµ¥ °¡Àå Áß¿äÇÑ ¼Ò°ßÀÌ´Ù. ÀüÇüÀûÀ¸·Î ¿°ÁõÀÌ ÀÖÀ»¶§´Â serum ferritinÀÌ 3¹è Áõ°¡

ÇÑ´Ù.

ÀÌ·± º¯È­ ¸ðµÎ´Â erythropoiesisÀÇ ¸î ´Ü°è¿¡¼­ inflammatory cytokinesÀÇ ¿µÇâ ¶§¹®ÀÌ´Ù.

Tab 105-4

IL-1Àº Á÷Á¢ erythropoietin»ý»êÀ» °¨¼Ò½ÃŲ´Ù. IL-1Àº IFN-¥ãÀÇ À¯¸®¸¦ ÅëÇÏ¿© erythropoietin

¿¡ ´ëÇÑ erythroid marrow response¸¦ ¾ïÁ¦ÇÑ´Ù. ±× ¿Ü¿¡, TNF´Â IFN-¥âÀÇ À¯¸®¸¦ ÅëÇÏ¿©

erythropoietin¿¡ ´ëÇÑ ¹ÝÀÀÀ» ¾ïÁ¦ÇÑ´Ù. chronic inflammation/infection¿¡¼­ 1Â÷ÁúȯÀÌ

ºóÇ÷ÀÇ ÁßÁõµµ¿Í Ư¡À» °áÁ¤ÇÑ´Ù.

neoplasm, bacterial infection(TNF, ¥â-IFN)

EPO + Fe <------> marrow -> RBC

RA(IL-1, ¥ã-IFN)

6. Anemia of renal disease

CRF´Â ÈçÈ÷ moderate to severe hypoproliferative anemia°¡ µ¿¹ÝµÈ´Ù. ºóÇ÷ÀÇ Á¤µµ´Â

½ÅºÎÀü Á¤µµ¿Í ºñ·ÊÇÑ´Ù. erythropoietin»ý»ê ½ÇÆÐ ¹× ÀûÇ÷±¸ ¼ö¸í °¨¼Ò·Î ÀÎÇÑ ºóÇ÷ÀÌ´Ù.

HUSȯÀÚ¿¡¼­´Â Åõ¼®ÀÌ ÇÊ¿äÇÑ ½ÅºÎÀüÀÏÁö¶óµµ ¿ëÇ÷¿¡ ¹ÝÀÀÇÏ¿© erythropoiesis°¡ Áõ°¡

ÇÑ´Ù.

polycystic renal disease ¶ÇÇÑ ½ÅºÎÀü Á¤µµ¿¡ ºñÇØ erythropoietin°áÇÌÀÌ ´úÇÏ´Ù.

¹Ý´ë·Î ´ç´¢È¯ÀÚ´Â ÁÖ¾îÁø ½ÅºÎÀü Á¤µµ¿¡ ºñÇØ º¸´Ù ½ÉÇÑ erythropoietin°áÇÌÀ» º¸ÀδÙ.

renal disease anemia¿¡¼­ serum iron, TIBC, ferritin levelÀº Á¤»óÀÌ´Ù.

±×·¯³ª ¸¸¼º Ç÷¾× Åõ¼®È¯ÀÚ¿¡¼­´Â Åõ¼®Áß ¹ß»ýÇÏ´Â ½ÇÇ÷·Î ÀÎÇÏ¿© IDA°¡ ¹ß»ýÇÒ¼ö ÀÖ´Ù.

ÀÌ·± ȯÀÚ´Â erythropoietin tx¿¡ ÀûÀýÇÑ ¹ÝÀÀÀ» È®º¸Çϱâ À§ÇØ Ã¶ºÐÀ» º¸ÃæÇØ ÁÖ¾î¾ß ÇÑ´Ù.

*Ä¡·á: CRFȯÀÚ¿¡¼­ Åë»óÀÇ erythropoietin¿ë·®Àº 50-150 U/kg ¡¿ ÁÖ3ȸ SCÀÌ´Ù.

¾ÏȯÀÚ¿¡¼­ ºóÇ÷À» ±³Á¤Çϱâ À§ÇØ ÇÊ¿äÇÑ ¾çÀº ´õ ¸¹´Ù. (¡­300 U/kg ÁÖ´ç 3ȸ±îÁö)

Hb 10-12 g/dL´Â iron levelÀÌ ÀûÀýÇÏ´Ù¸é 4-6ÁÖ³»¿¡ µµ´ÞÇÑ´Ù.

ÀÏ´Ü ¸ñÇ¥ Hb level¿¡ µµ´ÞÇϸé erythropoietin¿ë·®À» 75 U/kg ¡¿ ÁÖ3ȸ·Î ÁÙÀϼö ÀÖ´Ù.

7. Anemia in hypometabolic states

1) endocrine deficiency states

³²³à»çÀÌÀÇ Hb¼öÄ¡ Â÷ÀÌ´Â androgen°ú estrogenÀÇ erythropoiesis¿¡ ¹ÌÄ¡´Â ¿µÇâ°ú

°ü°èÀÖ´Ù.

testosterone & anabolic steroid´Â erythropoiesis¸¦ Áõ°¡½ÃŲ´Ù.

³²ÀÚ¿¡¼­ °Å¼¼Çϰųª estrogenÀ» Åõ¿©Çϸé erythropoiesis°¡ °¨¼ÒÇÑ´Ù.

°©»ó¼± ±â´ÉÀúÇϳª ³úÇϼöü È£¸£¸ó °áÇÌ¿¡¼­ °æÇѺóÇ÷ÀÌ ¹ß»ýÇÒ¼ö ÀÖ´Ù.

º´ÀÎÀº öºÐ, ¿±»ê Èí¼ö¿Í °°Àº ¿µ¾ç°áÇ̰ú °°ÀÌ º¹ÀâÇÏ´Ù.

º¸Åë È£¸£¸ó °áÇÌÀ» ±³Á¤ÇÏ¸é ºóÇ÷Àº ȸº¹µÈ´Ù.

Addison's disease¿¡¼­´Â ºóÇ÷ÀÌ º¸´Ù ½ÉÇÏ´Ù. ±×·¯³ª ºóÇ÷Àº plasma volumeÀÌ °¨¼Ò

ÇϹǷΠmaskµÈ´Ù. cortisol & volume replacementÇϸé Hb levelÀÌ ±Þ°ÝÈ÷ °¨¼ÒÇÑ´Ù.

hyperparathyroidism¿¡ ÇÕº´µÈ °æÇÑ ºóÇ÷Àº hypercalcemiaÀÇ renal effect·Î ÀÎÇÏ¿©

erythropoietin»ý»êÀÌ °¨¼ÒÇϰųª erythroid progenitorsÀÇ »ý»ê¿¡ Àå¾Ö°¡ Àֱ⠶§¹®ÀÌ´Ù.

2) protein starvation

´Ü¹éÁú ¼·Ãë°¡ ºÎÁ·Çϸé mild to moderate hypoproliferative anemia°¡ ÃÊ·¡µÈ´Ù. ÀÌ·± ÇüÅÂ

ÀÇ ºóÇ÷Àº ³ëÀο¡¼­ ÈçÇÏ´Ù.

3) anemia in liver disease

¸¸¼º°£Áúȯ¿¡¼­ °æÇÑ hypoproliferative anemia°¡ ¹ß»ýÇÒ¼ö ÀÖ´Ù. PB smearÇϸé burr cells

& stomacytes¸¦ º¼¼ö ÀÖ´Ù. ÀÌ´Â lecithin cholesterol acyltransferase°áÇÌÀ¸·Î ÀÎÇÏ¿© ¼¼Æ÷

¸·¿¡ cholesterolÀÌ °ú´ÙÇÏ°Ô ÃàÀûµÇ¾î »ý±ä´Ù.

II. Megaloblastic anemias

Megaloblastic anemia´Â DNAÇÕ¼º Àå¾Ö·Î ¹ß»ýÇÑ´Ù. ´Ù¼Ò ºü¸¥ turnover¸¦ °¡Áö´Â ¼¼Æ÷,

ƯÈ÷ hematopoietic precursors & GI epithelial cells¿¡¼­ 1Â÷ÀûÀ¸·Î ¹ß»ýÇÑ´Ù.

¼¼Æ÷ºÐ¿­Àº ´À¸®Áö¸¸, ¼¼Æ÷ÁúÀº Á¤»óÀûÀ¸·Î ¹ß´ÞÇϹǷÎ, megaloblastic cellsÀº Ä¿Áö°Ô µÇ°í

RNA:DNAºñ°¡ Áõ°¡ÇÏ°Ô µÈ´Ù. megaloblastic erythroid progenitors´Â °ñ¼ö¿¡¼­ ÆÄ±«µÈ´Ù.

±×·¯¹Ç·Î marrow cellularity´Â Áõ°¡µÇÁö¸¸ ÀûÇ÷±¸ »ý»êÀº °¨¼ÒÇϴµ¥ À̸¦ "ineffective

erythropoiesis"¶ó ÇÑ´Ù.

´ëºÎºÐÀÇ megaloblastic anemia´Â cobalamin(vitB12) and/or folic acid°áÇÌÀ¸·Î ¹ß»ýÇÑ´Ù.

1. Physiology

1) Folic acid

ÀÏÀÏ Ãּҿ䱸·®: 50 ug

ÀúÀå·®: 5-20 mg(±×Áß ¹ÝÀº liver¿¡ ÀúÀå)

* Fig 107-1 folate metabolism

2) Cobalamin

ÀÏÀÏ Ãּҿ䱸·®: 2.5 ug

* Fig 107-2 The assimilation of cobalamin

2. Cobalamin deficiency

cobalamin deficiencyÀÇ ÀÓ»óÀû Ư¡Àº Ç÷¾×, À§Àå°ú ½Å°æ°è¸¦ ħ¹üÇÑ´Ù.

¨ç hematologic manifestations

°ÅÀÇ ÀüÀûÀ¸·Î ºóÇ÷ÀÌ´Ù.

ºóÇ÷ÀÇ Áõ»óÀ¸·Î weakness, light-headedness, vertigo, tinnitus, palpitation, angina,

congestive failure Áõ»óÀÌ ³ªÅ¸³¯ ¼ö ÀÖ´Ù. bilirubin levelÀÌ Áõ°¡ÇÑ °ÍÀº °ñ¼ö¿¡¼­ erythroid

cell turnover°¡ ºü¸£±â ¶§¹®ÀÌ´Ù.

¨è GI manifestations

sore tongueÀ» È£¼ÒÇϰí, smooth & beefy red color¸¦ º¸ÀδÙ.

anorexia with moderate wt loss°¡ Àú¸íÇØÁö°í ¼³»ç ȤÀº ´Ù¸¥ À§Àå°ü Áõ»óÀÌ µ¿¹ÝµÈ´Ù.

¨é neurologic manifestations

½Å°æÇÐÀû Áõ»óÀº ÈçÈ÷ Ä¡·á¸¦ ÇØµµ ¿ÏÀüÈ÷ ȸº¹µÇÁö ¾Ê´Â´Ù. º´¸®ÀûÀ¸·Î demyelinationÀ¸·Î

½ÃÀÛÇÏ¿© axonal degeneration, °á±¹¿£ neuronal death¸¦ ÃÊ·¡ÇÑ´Ù. ÀÌ´Â ºñ°¡¿ªÀûÀÌ´Ù.

* ħ¹üµÇ´Â °÷

: peripheral nerves, spinal cordÀÇ post & lat. columns, cerebrum

* Áõ»ó ¹× ¡ÈÄ: numbness, paresthesia in the extremities(earlist neurologic sx),

weakness,ataxia, sphincter disturbance

reflex´Â °¨¼ÒÇϰųª Áõ°¡

Romberg & Babinski sign¾ç¼º

position & vibration sense °¨¼Ò

mental disturbance

neurologic disease´Â Á¤»ó Hct & Á¤»ó RBC index¸¦ °¡Áø ȯÀÚ¿¡¼­µµ ¹ß»ýÇÒ¼ö ÀÖÀ½À»

¸í½ÉÇØ¾ß ÇÑ´Ù. folate°ø±ÞÀº ¸¹Àº µµ¿òÀÌ µÇÁö¸¸, cobalamin deficiency¶§´Â ½Å°æÇÐÀû ¹ßÇö

À» Áõ°¡½Ãų¼ö ÀÖ´Ù.

1) cobalmin À¯¸®Àå¾Ö

À½½Ä³» cobalaminÀº °í±â³» È¿¼Ò¿Í °­ÇÏ°Ô °áÇյǸç À§³» HCl°ú pepsin¿¡ ÀÇÇØ È¿¼Ò¿Í

ºÐ¸®µÈ´Ù.

70¼¼ ÀÌ»ó ³ëÀÎÀº À½½Ä¿¡¼­ cobalaminÀ¯¸®°¡ ÈçÈ÷ µÇÁö ¾ÊÁö¸¸ crystalline B12(multivitamin

¿¡¼­ °¡Àå ÈçÈ÷ ¹ß°ßµÇ´Â ÇüÅÂ)¸¦ Èí¼öÇÒ ´É·ÂÀº °¡Áö°í ÀÖ´Ù.

cobalamin release defectÀÇ Á¤È®ÇÑ ¹ß»ýÀº ¾Ë±â ¾î·ÆÁö¸¸ 70¼¼ À̻󿡼­ ´ë·« 10¡­50%

ÀÌ´Ù.

À§»ê »ý¼ºÀ» ¾ïÁ¦ÇÏ´Â ¾à(¿¹: omeprazole)Àº À½½Ä¿¡¼­ cobalaminÀ¯¸®¸¦ ¹æÇØÇÑ´Ù.

2) pernicious anemia

cobalamin deficiencyÀÇ °¡Àå ÈçÇÑ ¿øÀÎÀ̸ç IFÀÇ °áÇÌÀ¸·Î ¹ß»ýÇÑ´Ù.

IF °áÇÌÀº gastric mucosa atrophy³ª parietal cellÀÇ autoimmune destruction¿¡ ÀÇÇÑ´Ù.

ºÏÀ¯·´°ú ¾ÆÇÁ¸®Ä«°è ¹Ì±¹Àο¡¼­ °¡Àå ÈçÈ÷ ¹ß°ßµÇ°í ³²À¯·´°ú ¾Æ½Ã¾Æ¿¡¼­´Â ´ú ÈçÇÏ´Ù.

³²³àºñ´Â µ¿ÀÏÇÏ¸ç Æò±Õ 60¼¼¿¡ ¹ß»ýÇÏ°í ºñ·Ï typical RA°¡ 10¼¼ ¹Ì¸¸¿¡¼­ º¼¼ö ÀÖÁö¸¸

30¼¼ ¹Ì¸¸Àº µå¹°´Ù.

Graves' disease, myxedema, thyroiditis, idiopathic adrenocortical insufficiency, vitiligo,

hypoparathyroidism°ú °°Àº ¸é¿ªÁúȯ¿¡¼­ ºóµµ°¡ Áõ°¡ÇÑ´Ù.

¶ÇÇÑ È¯ÀÚÀÇ 90%¿¡¼­ antiparietal cell Ab¾ç¼ºÀ̰í, 60%¿¡¼­ anti-IF Ab¸¦ °¡Áö°í ÀÌ´Ù.

antiparietal cell Ab´Â gastric atrophy without pernicious anemiaȯÀÚÀÇ 50%¿¡¼­ ¹ß°ßµÇ°í

unselected ptÀÇ 10-15%¿¡¼­µµ ¹ß°ßµÈ´Ù. ±×·¯³ª ÀÌ·± °æ¿ì¿¡¼­µµ anti-IF Ab´Â ¾ø´Ù.

ȯÀÚÀÇ Ä£Ã´¿¡¼­ ºóµµ°¡ Áõ°¡ÇÑ´Ù. parietal cellÀÇ ÆÄ±«´Â cytotoxic T cell¿¡ ÀÇÇØ ¸Å°³µÇ´Â

°ÍÀ¸·Î »ý°¢µÈ´Ù. agammaglobulinemiaȯÀÚ¿¡¼­ ¸Å¿ì ÈçÇѵ¥ ÀÌ´Â º´Àο¡¼­ cellular

immune systemÀÌ

ÁÖ¿ªÇÒÀ» ÇÔÀ» ÀǹÌÇÑ´Ù.

ÀûÀýÇÑ replacement tx¸¦ ÅëÇÏ¿© ȯÀÚ´Â cobalamin deficiency·Î ÀÎÇÑ ¸ðµç ÀÌ»óÀÌ ¿ÏÀüÈ÷

±³Á¤µÈ´Ù(½Å°æ°èÀÇ ºñ°¡¿ªÀû º¯È­¸¦ Á¦¿ÜÇϰí´Â).

3) postgastrectomy

total gastrectomy or gastric mucosaÀÇ extensive damageÈÄ IF source°¡ ¾ø¾îÁö¹Ç·Î

megaloblastic anemia°¡ ¹ß»ýÇÑ´Ù. megaloblastic anemia´Â partial gastrectomyÈÄ¿¡µµ

»ý±æ¼ö ÀÖÀ¸³ª ºóµµ´Â total gastrectomyº¸´Ù ³·´Ù.

partial gastrectomyÈÄ cobalamin deficiency¿øÀÎÀº ºÐ¸íÇÏÁö ¾ÊÁö¸¸ À½½ÄÀ¸·ÎºÎÅÍ

cobalamin À¯¸®Àå¾Ö, Àå³»¼¼±ÕÁõ½ÄÀÌ Á¦½ÃµÇÁö¸¸ Ç×»ýÁ¦¿¡ ´ëÇÑ ¹ÝÀÀÀº ÈçÄ¡ ¾Ê´Ù.

4) intestinal organisms

stricture, diverticulum, anastomosis, "blind loops"¿Í °°Àº ÇØºÎÇÐÀû º´º¯ ȤÀº DM,

scleroderma, amyloid°°Àº pseudoobstruction¿¡ ÀÇÇØ Á¤Ã¼°¡ »ý±â¸é megaloblastic

anemia°¡ »ý±æ ¼ö ÀÖ´Ù. ÀÌ·± ºóÇ÷Àº ¼ÒÀå³» ¼¼±ÕÀÇ Áõ½Ä¿¡ ÀÇÇÏ¸ç ¼¼±ÕÀÌ Èí¼öÀüÀÇ

cobalaminÀ» ¼ÒºñÇϱ⠶§¹®ÀÌ´Ù.

5) ileal abnormalities

tropical sprue¿¡¼­ cobalamin deficiency°¡ ÈçÇѵ¥ nontropical sprue¿¡¼± µå¹® ÇÕº´ÁõÀÌ´Ù.

distal ileumÀÇ Èí¼ö´É¿¡ ¼Õ»óÀÌ °¡´Â °æ¿ì¿¡ cobalamin deficiency°¡ »ý±ä´Ù.

ƯÁ¤ÁúȯÀ¸·Î´Âregional enteritis, Whipple's disease, tuberculosis°¡ ÀÖ´Ù.

ileal resectionÈÄ¿¡ cobalamin malabsorptionÀ» º¼¼ö ÀÖ´Ù.

ZES¶§µµ cobalamin malabsorptionÀ» ÀÏÀ¸Å³¼ö Àִµ¥ ¼ÒÀåÀ» »ê¼ºÈ­½ÃÅ´À¸·Î½á IF¿¡ ´ëÇÑ

R binderÀÇ À̵¿À» ´À¸®°Ô ÇÔÀ¸·Î½á cobalamin-IF complex¿Í ileal receptor¿ÍÀÇ °áÇÕÀ»

¹æÇØÇÑ´Ù.

6) Nitric oxide

¸¶ÃëÁ¦·Î NO¸¦ ÈíÀÔÇϸé endogenous cobalaminÀ» ÆÄ±«ÇÑ´Ù.

3. Folic acid deficiency

1) inadequate intake

alcoholics: ¼·ÃëºÎÁ·, folate metabolismÀå¾Ö

narcotic addicts: malnutrition

2) ¿ä±¸·® Áõ°¡

chronic hemolytic anemia, very active erythropoiesis, pregnant woman, chronic

hemodialysis

3) malabsorption: tropical sprue

4. Drugs

1) direct inhibitors of DNA synthesis

purine analogues: 6-TG, azathioprine, 6-MP

pyrimidine analogues: 5-FU, cytosine arabinoside

±âŸ: hydorxyurea, procarbazine

antiviral agent: zidovudine

2) folate antagonists

MTX: DHF reductaseÀÇ powerful inhibitor

±× ¿Ü DHF reductase inhibitor: pentamidine, trimethoprim, triamterene, pyrimethamine

3) ±âŸ : anticonvulsants(phenytoin, pyrimedone, phenobarbital)

5. Áø´Ü

significant macrocytosis(MCV>100fL)´Â megaloblastic anemia¸¦ ½Ã»çÇÑ´Ù.

*other causes of macrocytosis

i) hemolysis

ii) liver disease

iii) alcoholism, hypothyroidism

iv) aplastic anemia

macrocytosis°¡ ½ÉÇØÁö¸é(MCV > 110 fL) ´õ¿í´õ megaloblastic anemiaÀÏ °¡´É¼ºÀÌ Ä¿Áø´Ù.

ÇϳªÀÇ ¼¼Æ÷°¡ ¿©¼¸ ¿±ÀÇ ÇÙÀ» °¡Áö´Â Ư¡Àû ¼Ò°ßÀÌ Àִµ¥ À̶§´Â megaloblastic anemia

¸¦ ¹Ù·Î ÀǽÉÇÒ¼ö ÀÖ´Ù. reti count´Â ³·´Ù.

BM´Â hypercellular & M/E ratio°¨¼Ò, ¸¹Àº stainable ironÀ» º¸ÀδÙ.

RBC precursor´Â ºñÁ¤»óÀûÀ¸·Î Å©°í, cytoplasm¿¡ ºñÇØ ´ú ¼º¼÷ÇÑ ÇÙÀ» °¡Áø´Ù

(nuclear-cytoplasmic asynchrony).

megaloblastic anemia´Â ineffective erythropoiesis°¡ Ư¡ÀÌ´Ù.

RBC precursorÀÇ 90%°¡ Ç÷ÁßÀ¸·Î ³ª°¡±â Àü¿¡ ÆÄ±«µÈ´Ù(Á¤»óÀÎÀº 10-15%).

erythroblastÀÇ °ñ¼ö³» ÆÄ±«·Î ÀÎÇÏ¿© unconjugated bilirubin°ú LDH(iso 1)°¡ Áõ°¡ÇÑ´Ù.

megaloblastic anemiaȯÀÚ¸¦ Æò°¡Çϴµ¥ serum cobalamin°ú folate levelÀ» ÃøÁ¤ÇÔÀ¸·Î½á

¾î´À vitamin°áÇÌÀÎÁö °áÁ¤ÇÏ´Â °ÍÀº Áß¿äÇÏ´Ù.

cobalaminÀÇ Á¤»ó¹üÀ§´Â 200-900 pg/mlÀε¥ 100 pg/ml¹Ì¸¸À϶§´Â significant deficiency¸¦

ÀǹÌÇÑ

´Ù. TC II¿¡ °áÇÕµÈ cobalaminÀÇ ÃøÁ¤ÀÌ º¸´Ù »ý¸®ÀûÀÎ ÃøÁ¤ÀÌÁö¸¸ ÀÌ´Â ¾ÆÁ÷ º¸ÆíÀûÀ¸·Î

»ç¿ëµÇÁö´Â ¾Ê´Â´Ù.

folic acidÀÇ Á¤»ó³óµµ´Â 6-20 ng/mlÀÌ´Ù. 4 ng/mlÀÌÇÏÀ϶§´Â ÀϹÝÀûÀ¸·Î folate deficiency

Áø´ÜÀ» °í·ÁÇÒ¼ö ÀÖ´Ù.

ÀÏ´Ü cobalamin deficiency°¡ È®ÀÎµÇ¸é º´ÀÎÀº Schilling test¿¡ ÀÇÇØ °áÁ¤ÇÑ´Ù.

6. Ä¡·á

1) Cobalamin deficiency

Ä¡·áÀÇ ±âº»Àº replacement txÀÌ´Ù. cobalamin °áÇÌÀº °ÅÀÇ Ç×»ó Èí¼öÀå¾Ö¿¡ ÀÇÇϹǷÎ

ȯÀÚ´Â ÀϹÝÀûÀ¸·Î parenteral tx(º¸Åë IM cyanocobalamin)ÇÑ´Ù.

*1000 ug ÁÖ1ȸ ¡¿ 8ÁÖ -> ±×ÈÄ 1000 ug ¿ù1ȸ Æò»ý

±×·¯³ª 2mg crystalline B12 ¸ÅÀÏ POÇϴ°͵µ ¸Å¿ì È¿°úÀûÀÌ´Ù.

Ä¡·áÈÄ marrow morphology´Â ¼ö½Ã°£ÈÄ Á¤»óÈ­µÇ¸ç reticulocytosis´Â 4-5ÀÏÈÄ ½ÃÀ۵Ǿî

7Àϰ ÃÖ°íÁ¶¿¡ ´ÞÇÑ´Ù(Fig 107-3). ±×ÈÄ ¼öÁÖ¿¡ °ÉÃÄ ºóÇ÷Àº ȸº¹µÈ´Ù.

reticulocytosis°¡ ÀϾÁö ¾Ê°Å³ª Hct°¡ ±â´ë¼öÁØÀ¸·Î µµ´ÞÇÏÁö ¾ÊÀ¸¸é ºóÇ÷¿¡ ±â¿©ÇÏ´Â

ÀÎÀÚ¸¦ Á¶»çÇØ¾ß ÇÑ´Ù.

(¿¹: °¨¿°, µ¿¹ÝµÈ iron deficiency, folate deficiency or hypothyroidism)

Ä¡·áÃʱ⿡ hypokalemia & salt retentionÀÌ »ý±æ¼ö ÀÖÀ¸¸ç thrombocytosisµµ º¼¼ö ÀÖ´Ù.

´ë·®ÀÇ folate°¡ cobalamin deficiency·Î ÀÎÇÑ megaloblastic anemia¸¦ ±³Á¤ÇÒ¼ö ÀÖ´Ù

(½Å°æÇÐÀû ÀÌ»ó Á¦¿Ü).

folate tx·Î ½Å°æÇÐÀû Áõ»óÀº ¾ÇÈ­µÇ±âµµ ÇÑ´Ù. ´ë·®ÀÇ folate Åõ¿©È¯ÀÚ¿¡¼­ cobalamin

deficiency°¡ maskµÉ¼ö ÀÖ´Ù. ÀÌ·± ÀÌÀ¯·Î folate Åõ¿©·Î hematologic response¸¦ º¸ÀδÙ

°í ÇØ¼­ cobalamin deficiency¸¦ ¹èÁ¦ÇÏ¸é ¾ÈµÈ´Ù.

cobalamin deficiency´Â ´ÜÁö ÀûÀýÇÑ °Ë»ç°á°ú·Î ¹èÁ¦ÇØ¾ß ÇÑ´Ù.

³ëÀο¡¼­ cobalamin Èí¼öÀå¾Ö ºóµµ°¡ ³ô°í Ç÷¾×ÇÐÀû Áõ»óº¸´Ù ½Å°æÇÐÀû Áõ»óÀ¸·Î ¹ßÇöµÇ´Â

overt cobalamin deficiencyÀ§ÇèÀÌ Áõ°¡ÇϹǷΠÀϺÎÀü¹®°¡´Â 65¼¼ ÀÌ»ó ³ëÀο¡°Ô 0.1mg/d

oral crystalline cobalamin prophylaxis¸¦ ±ÇÀ¯Çϱ⵵ ÇÑ´Ù.

2) folate deficiency

¿ª½Ã replacement txÇÑ´Ù.

1mg/d PO(Åë»ó¿ë·®), ±×·¯³ª Èí¼öÀå¾ÖÀÎ °æ¿ì °í¿ë·®(¡­5mg/d)ÀÌ ÇÊ¿äÇÒ¼ö ÀÖ´Ù.

Ä¡·áÈÄ hematologic response´Â cobalamin deficiency¿Í À¯»çÇÏ´Ù.

Ä¡·á±â°£Àº °áÇÌ»óÅ¿¡ µû¶ó °áÁ¤ÇÑ´Ù.

3)´Ù¸¥ ¿øÀÎÀ϶§

¾à¿¡ ±âÀÎÇÑ °æ¿ì ÇÊ¿äÇÏ¸é ¾àÀ» ÁÙÀ̰ųª Áß´ÜÇÔÀ¸·Î½á Ä¡·áÇÒ¼ö ÀÖ´Ù.

folate antagonist(DHF reductase inhibitor)ÀÇ ¿µÇâÀº folinic acid(5-THF) 100-200 mg/d¸¦

Åõ¿©ÇÔÀ¸·Î½á »ó¼âµÉ¼ö ÀÖ´Ù.

III. Hemolytic anemias

1. ºÐ·ù

i) molecular defect(hemoglobinopathy or enzymopathy) inside the red cell

ii) membrane structure & functionÀÇ ÀÌ»ó

iii) environmental factor such as mechanical trauma or an autoantibody

hemolytic anemia´Â intracorpuscular type vs extracorpuscular typeÀ¸·Î ºÐ·ùÇϰųª

inherited vs acquired·Î ºÐ·ùÇÒ¼öµµ ÀÖ´Ù.

2. Inherited hemolytic anemia

ÀûÇ÷±¸ÀÇ ÁÖ¿ä ¼¼°¡Áö ±¸¼ºÀÎÀÚÁß ÇϳªÀÇ ¼±ÃµÀû °áÇÔÀ¸·Î ÇØ¼­ ¹ß»ýÇÑ´Ù.

ÀÌ ¼¼°¡Áö ±¸¼ºÀÎÀÚ¿¡¼­ membrane, enzyme°ú hemoglobinÀÌ ÀÖ´Ù.

1) Red cell membrane disorders

ÀûÇ÷±¸ÀÇ ÇüÅÂÇÐÀû ÀÌ»óÀÌ ¹ß°ßµÇ´Âµ¥

i) hereditary spherocytosis

ii) hereditary ellipotocytosis(hereditary pyropoikilocytosisÆ÷ÇÔ)

iii) hereditary stomacytosis µîÀÌ ÀÖ´Ù.

(1) hereditary spherocytosis

RBC membrane cytoskeletonÀÇ ´Ü¹éÁß ÇϳªÀÇ °áÇÔÀ¸·Î ¹ß»ýÇϴµ¥ ¸·ÀÌ ¼Ò½ÇµÇ°í

Ç¥¸éÀû: ºÎÇÇÀÇ ºñ°¡ °¨¼ÒÇÏ¿© spherocytosis°¡ »ý±ä´Ù.

»ó¿°»öü ¿ì¼º(AD)ÀÇ À¯Àü¹æ½ÄÀ» º¸ÀÌ¸ç ºóµµ´Â 1:1000 ¡­ 1:4500Á¤µµÀÌ´Ù.

¶§·Î´Â ¾î¸° ¾Æ±â¶§ ¹ßÇöÇϱ⵵ ÇÏÁö¸¸ ÁÖ·Î ¼ºÀζ§±îÁö ¹ß°ß¾ÈµÈ´Ù.

¨ç ÀÓ»ó¹ßÇö: anemia, splenomegaly, jaundice

bile pigment »ý¼ºÁõ°¡·Î pigmented gallstoneÀÌ ¾ÆÀ̵éÇÑÅ×µµ ÈçÇÏ´Ù.

°ñ¼öÀÇ compensatory erythroid hyperplasia°¡ ÀϾ´Ù.

erythropoiesis¸¦ 6-8¹è Áõ°¡½ÃŰ´Â °ñ¼öÀÇ ´É·Â ¶§¹®¿¡ ºóÇ÷Àº °æÇϰųª ÁߵÀ̸ç,

¾øÀ»¼öµµ ÀÖ´Ù.

º¸»ó±âÀüÀº °¨¿°(ƯÈ÷ parvovirus), trauma, surgery, pregnancy¿¡ ÀÇÇØ erythroid

hyperplasia°¡ Áß´ÜµÉ ¼öµµ ÀÖ´Ù. splenomegaly°¡ ¸Å¿ì ÈçÇÏ´Ù. Ư¡ÀûÀÎ ÀûÇ÷±¸ ÀÌ»ó

Àº spherocyteÀÌ´Ù.

MCV´Â º¸Åë Á¤»óÀ̰ųª ¾à°£ °¨¼ÒÇϸç, MCHC´Â 350-400 g/L±îÁö Áõ°¡ÇÑ´Ù.

spheroidicity´Â RBC¸¦ hypoosmotic solutions¿¡ ³ëÃâ½ÃÄÑ osmotic fragility¸¦ ÃøÁ¤ÇÔ

À¸·Î½á Á¤·®ÀûÀ¸·Î Æò°¡ÇÒ¼öÀÖ´Ù.

Çö¹Ì°æ»ó spherocyte´Â Å©±â°¡ ÀÛÀ¸¸ç central pallor°¡ ¾ø´Ù.

autohemolysis test´Â sterile incubation 48½Ã°£ÈÄ¿¡ ÀÚ¹ßÀû ¿ëÇ÷ÀÌ ¹ß»ýÇÏ´Â °ÍÀ» º¸´Â

°ÍÀε¥ ÀÌ ¶ÇÇÑ À¯¿ëÇÏ´Ù.

¨è º´ÀÎ

cytoskeletal network¿¡ ºÙÀº lipid bilayer¸¦ ħ¹üÇÑ´Ù.

°ÅÀÇ ¸ðµç ȯÀÚ°¡ spectrinÀÌ °á¼ÕµÇ¾î ÀÖ´Ù.

50%´Â ankyrin defect°¡ Àִµ¥ ÀÌ´Â protein 3¿Í spectrin»çÀÌÀÇ ´Ù¸®¿ªÇÒÀ» ÇÏ´Â ´Ü¹é

ÀÌ´Ù(Fig 108-2).

homozygotes(recessive pattern for ankyrin defect) : more severe anemia

heterozygotes: more common

25%´Â protein 3 mutation·Î mild anemia¸¦ ÀÏÀ¸Å°°í ³ª¸ÓÁö´Â spectrin mutationÀ¸·Î

RBCÇÕ¼ºÀå¾Ö°¡ »ý±ä´Ù. ¥â-spectrin defectÀ϶§´Â mild, dominant inheritance, ¥á-spectrin

defectÀ϶§´Â severeÇϰí recessive inheritance¸¦ º¸ÀδÙ.

¸ð¾ç ¹× °æÁ÷¼º ¶§¹®¿¡ spherocyte´Â ºñÀåÀ» Åë°úÇÒ¼ö ÀÖ´Ù.

¨é Áø´Ü

family history(anemia and/or splenectomy)

immune spherocytossi¿Í °¨º°ÇØ¾ß ÇÑ´Ù.

immune spherocytosisÀÇ Áø´ÜÀº positive direct Coombs test·Î ½±°Ô Áø´ÜÇÒ¼ö ÀÖ´Ù.

¸î¸î spherocytes´Â ´Ù¾çÇÑ ¿ëÇ÷¼º ÁøÈ¯ÀÇ °úÁ¤¿¡¼­ º¼¼ö ÀÖ´Ù(ƯÈ÷ G6PD deficiency).

¨ê Ä¡·á

* splenectomy: RBCÇüÅ´ Áö¼ÓµÇÁö¸¸ ºóÇ÷Àº ±³Á¤ÇÒ¼ö ÀÖ´Ù. ¼ö¼úÀ§ÇèÀº ³·´Ù.

splenectomyÈÄ RBC»ýÁ¸Àº Á¤»óÈ­µÈ´Ù. ºóÇ÷ÀÌ Áö¼ÓµÈ´Ù¸é accessory spleen or other

diagnosis¸¦ °í·ÁÇØ¾ß ÇÑ´Ù.

gallstone, BM hypoplasia or hemolytic crisisÀÇ À§Ç輺 ¶§¹®¿¡ splenectomy´Â Áõ»óÀÖ´Â

ȯÀÚ¿¡¼­ ½ÃÇàÇØ¾ß ÇÑ´Ù. cholecystectomy´Â splenectomy¾øÀÌ ½ÃÇàÇØ¼­´Â ¾ÈµÇ´Âµ¥

¿Ö³ÄÇϸé intrahepatic gallstoneÀÌ »ý±æ¼ö Àֱ⠶§¹®ÀÌ´Ù.

¾ÆÀÌ¿¡°Ô¼­ splenectomy´Â 4¼¼±îÁö ¿¬±âÇØ¾ß Çϴµ¥ ±× ÀÌÀ¯´Â G(+) encapsulated

organism¿¡ ÀÇÇÑ severe infectionÀ§ÇèÀ» ÃÖ¼ÒÈ­Çϱâ À§ÇÔÀÌ´Ù. polyvalent

pneumococcal vaccineÀº ¼ö¼úÀü ÃÖ¼Ò 2ÁÖÀü¿¡ Åõ¿©ÇØ¾ß ÇÑ´Ù. severe hemolysisȯÀÚ

¿¡¼­´Â folic acid(1mg/d)¸¦ ¿¹¹æÀûÀ¸·Î Åõ¿©ÇØ¾ß ÇÑ´Ù.

(2) Hereditary elliptocytosis & hereditary pyropoikilocytosis

ºóµµ´Â 1/4000-5000À̸ç hereditary spherocytosis¿Í À¯»çÇÏ´Ù.

erythrocyte spectrinÀÌ»óÀ¸·Î ÀÎÇÏ¿© cytoskeletonÀÇ assemblyÀå¾Ö°¡ ÃÊ·¡µÈ´Ù.

ÀϺο¡¼­´Â membrane protein 4.1 deficiency°¡ Àֱ⵵ ÇÑ´Ù.

´ëºÎºÐ mild hemolysis(Hb > 12 g/dL, reti < 4%, haptoglobin°¨¼Ò)¸¦ º¸À̸ç

10-15%¿¡¼­´Â ½ÉÇÑ ÀÌ»óÀ» º¸ÀδÙ(RBC»ýÁ¸ 5ÀÏ, reti > 20%).

(3) Hereditary stomatocytosis

RBC membrane protein 7.2(stomatin) defect

2) Red cell enzyme defect

RBC°¡ ¼º¼÷ÇÏ´Â µ¿¾È¿¡ nucleus, ribosome, mitochondria°¡ ¼Ò½ÇµÇ¸é ´Ü¹éÇÕ¼º ¹×

oxidative phosphorylation´É·ÂÀÌ »ó½ÇµÈ´Ù. Embden-Meyerhof pathway(glycolysis)¿¡¼­

ATP°¡ ¹ß»ýÇØ¾ß ÇÑ´Ù. RBC¿¡ ÀÇÇØ ¼ÒºñµÇ´Â ´çÀÇ 10%´Â hexose-monophosphate shunt

¿¡ ÀÇÇØ ´ë»çµÈ´Ù(Fig108-3).

(1) Defects in the Embden-Meyerhof pathway

RBC´Â Á¾Á¾ ATP°¡ °áÇ̵Ǵµ¥ ÀÌ·¯¸é ¼¼Æ÷¾È¿¡¼­ ¹ÛÀ¸·Î K+À» ÆÛ³¾¼ö ¾ø°Ô µÈ´Ù.

ÀÌ·¯ÇÑ RBC´Â °æÁ÷µÇ°í mononuclear phagocyte system¿¡ ÀÇÇØ ½±°Ô °É·¯Áø´Ù.

glycolytic pathwayÀÇ ÀÓ»óÀûÀ¸·Î Áß¿äÇÑ ÀÌ»óÁß 95%´Â PK deficiency¿¡ ÀÇÇÑ´Ù.

³ª¸ÓÁö 4%´Â glucose phosphate isomerase deficiency¿¡ ÀÇÇÑ´Ù.

¨ç ÀÓ»ó¹ßÇö: severe hemolysis´Â ÈçÈ÷ ¾î¸°¾ÆÀ̶§ ¹ßÇöÇÑ´Ù: anemia, jaundice,

splenomegaly

¨è Lab; normocytic(or slightly macrocytic), normochromic anemia with reticulocytosis

PK deficiency: bizarre erythrocytes(spiculated cells), spherocyte´Â ¾ø´Ù.

µû¶ó¼­ congenital nonspherocytic hemolytic anemia¶ó ºÎ¸¥´Ù.

hereditary spherocytosis¿Í ´Þ¸® osmotic fragility´Â ÈçÈ÷ Á¤»óÀÌ´Ù.

Áø´ÜÀº specific enzyme assay¿¡ ÀÇÇÑ´Ù.

¨é Ä¡·á: ´ëºÎºÐ Ä¡·á¸¦ ¿äÇÏÁö ¾Ê´Â´Ù.

severe hemolysisÀִ ȯÀÚ´Â folic acid(1mg/d)¸¦ Åõ¿©ÇÑ´Ù.

hypoplastic crisis¶§´Â ¼öÇ÷ÀÌ ÇÊ¿äÇÏ´Ù.

PK deficiency¸¦ °¡Áø ¿©¼ºÀº ÀÓ½ÅÁß ºóÇ÷ÀÌ ´õ ½ÉÇØÁú¼ö Àִµ¥ ¶§·Î´Â ÀӽŶ§ óÀ½

Áø´ÜµÇ±âµµ ÇÑ´Ù. PK deficiencyȯÀÚ´Â splenectomy°¡ µµ¿òÀÌ µÉ ¼öµµ ÀÖ´Ù.

glucose phosphate isomerase deficiency¶ÇÇÑ splenectomyÈÄ È£ÀüµÉ¼ö ÀÖ´Ù.

splenectomy°¡ ´Ù¸¥ glycolytic enzymopathies¿¡¼­´Â È¿°úÀûÀ̶ó°í Áõ¸íµÇÁö´Â

¾Ê¾Ò´Ù.

(2) Defects in the hexose-monophosphate shunt

normal RBC´Â oxidant stress¿¡ Àß °ßµ®³½´Ù. ¼¼Æ÷°¡ oxygen radicalÀ» »ý¼ºÇÏ´Â drug or

toxin¿¡ ³ëÃâµÉ ¶§ hexose-monophosphate shunt¸¦ ÅëÇÑ ´ç´ë»ç´Â Á¤»óÀûÀ¸·Î ¼ö¹è Áõ°¡

ÇÑ´Ù. °¨¼ÒµÈ glutathioneÀº Àç»ý»êµÇ¾î Hb & RBC membrane sulfhydryl groupÀ»

oxidationÀ¸·ÎºÎÅÍ º¸È£ÇÑ´Ù.

hexose-monophosphate shunt¿¡ ¼±ÃµÀû °áÇÔÀÌ ÀÖ´Â »ç¶÷Àº RBC¿¡ glutathione levelÀ»

ÀûÀýÈ÷ À¯ÁöÇÒ¼ö ¾ø¾î sulfhydryl groupÀº »êÈ­µÇ°í RBC³»¿¡ ÃàÀûµÇ¾î Heinz body¸¦ Çü¼º

ÇÑ´Ù.

¨ç G6PD deficiency

°¡Àå ÈçÇÑ ¼±ÃµÀû shunt defectÀÌ´Ù.

G6PD geneÀº X chromosome¿¡ À§Ä¡ÇÑ´Ù. deficiency state´Â sex-linked traitÀÌ´Ù.

´ëºÎºÐÀÇ female carrier´Â ¹«Áõ»óÀÌ´Ù. deficient cellÀÇ ¸¹Àº ºÎºÐÀº male hemizygotesÀÌ´Ù.

hemolytic episodes´Â viral & bacterial infection¿¡ ÀÇÇØ Ã˹ߵǴµ¥ ±âÀüÀº ¾Ë·ÁÁ® ÀÖÁö

¾Ê´Ù.

±× ¿Ü¿¡ drug & toxinÀÌ ¿ëÇ÷À» ÀÏÀ¸Å²´Ù.

Tab 108-4: G6PD deficiencyȯÀÚ¿¡¼­ ¿ëÇ÷À» À¯¹ßÇÏ´Â ¾à¹°Àº sulfa drugs, antimalarials,

nitrofurantonµîÀÌ ÀÖ´Ù.

¨è ÀÓ»ó ¹× Lab Ư¡

oxidant stress¿¡ ³ëÃâµÈÈÄ ¼ö½Ã°£³» acute hemolytic crisis¸¦ °æÇèÇÑ´Ù. ±× °á°ú

hemoglobinuria & peripheral vascular collapse¸¦ ÀÏÀ¸Å²´Ù. ³ëÈÄµÈ RBC¸¸ ±Þ°ÝÈ÷ ÆÄ±«

µÇ¹Ç·Î oxidant°¡ Áö¼ÓÇÏ´õ¶óµµ hemolytic crisis´Â ÀÚ¿¬È¸º¹µÈ´Ù.

G6PD deficiency Áø´ÜÀº ¾ÆÇÁ¸®Ä« ȤÀº ÁöÁßÇØ ³²ÀÚ¿¡¼­ acute hemolytic episode°¡ ÀÖÀ»

¶§ °í·ÁÇØ¾ß ÇÑ´Ù. enzyme activity³ª deficiency effect¸¦ Æò°¡ÇÏ´Â °Ë»ç·Î Áø´ÜÇÒ¼ö ÀÖÁö

¸¸ °Ë»ç´Â false-negative result°¡ ÀÖÀ»¼ö ÀÖ´Ù.

¨é Ä¡·á

ÈçÈ÷ ÀÚ¿¬È¸º¹µÇ¸ç Ưº°ÇÑ Ä¡·á´Â ÇÊ¿äÄ¡ ¾Ê´Ù.

splenectomy´Â µµ¿òÀÌ µÇÁö ¾Ê´Â´Ù.

hemolytic episodesÀÇ ¿¹¹æÀÌ ÃÖ¼±ÀÌ´Ù. °¨¿°Àº Áï°¢ Ä¡·áÇØ¾ß Çϸç oxidant drug & fava

beans(ÀáµÎ)¿¡ Æø·ÎµÉ À§Çè¿¡ ÁÖÀÇÇØ¾ß ÇÑ´Ù. oxidant drugÀ» »ç¿ëÇÏ´Â ¾ÆÇÁ¸®Ä« ȤÀº

ÁöÁßÇØ »ç¶÷Àº G6PD deficiency¿¡ ´ëÇØ screenÇÏ¿©¾ß ÇÑ´Ù.

3. Acquired hemolytic anemia

ÀûÇ÷±¸´Â Á¤»óÀûÀ¸·Î ¸¸µé¾îÁöÁö¸¸ Ç÷¾×¼øÈ¯Áß¿¡ ¼Õ»óµÇ¾î ÆÄ±«µÈ´Ù. ÀÌ·± ¼Õ»óÀº

antibody or toxin¿¡ ÀÇÇϱ⵵ ÇÏ°í ¼øÈ¯°è ÀÚüÀÌ»ó¿¡ ÀÇÇϱ⵵ ÇÑ´Ù.

1) Hypersplenism

ºñÀåÀ» Åë°úÇÏ´Â Ç÷¾× ´ëºÎºÐÀº white pulp³»ÀÇ arterioles¿¡¼­ red pulp³» sinus·Î Áö³ª

venous systemÀ¸·Î µé¾î°£´Ù. ¹Ý´ë·Î ÀϺÎ(1-2%)´Â lymphatic white pulpÀÇ "marginal

zone"À¸·Î µé¾î°£´Ù. marginal zone¿¡ ÀÖ´Â ¼¼Æ÷´Â ޽ıâ´ÉÀÌ ¾øÀÌ mechanical filter·Î

ÀÛ¿ëÇÑ´Ù. RBC°¡ À̰÷À» Áö³ª°í red pulp·Î µé¾î°¥ ¶§ RBC´Â macrophage°¡ dzºÎÇÑ

narrow cords·Î µé¾î°¡¼­´Â sinus¿Í ¿¬°áµÈ ¸·´Ù¸¥ °÷¿¡ µµ´ÞÇÏ°Ô µÈ´Ù. sinus¿¡ ³ª¿­µÈ

¼¼Æ÷µé »çÀÌ¿¡´Â Á¶±×¸¸ ±¸¸ÛÀÌ ³ª Àִµ¥ Æò±ÕÁ÷°æÀº 3 um°¡ µÈ´Ù(RBC´Â 4.5um).

vascular sinus·Î ´Ù½Ã µé¾î°¥¼ö ¾ø´Â ÀûÇ÷±¸´Â phagocytic cell¿¡ ÀÇÇØ Ž½ÄµÇ¾î ÆÄ±«µÈ´Ù

(Fig63-1).

splenic sequestration¿¡ ÀÇÇØ cytopenia°¡ ÀϾ ¶§ hypersplenismÀ¸·Î Áø´ÜÇÒ¼ö ÀÖ´Ù.

2) Immunologic causes of hemolysis

¼ºÀο¡¼­ immune hemolysis´Â ÈçÈ÷ ȯÀÚÀÇ ÀûÇ÷±¸¿¡ ÀÖ´Â Ç׿ø¿¡ ´ëÇÑ IgG or IgM Ab¿¡

ÀÇÇØ ÀϾ´Ù("autoAb"¶ó ºÎ¸£±âµµ ÇÑ´Ù).

Coombs antiglobulin test´Â autoimmune hemolysis¸¦ Áø´ÜÇÏ´Â ÁÖ¿ä ¹æ¹ýÀÌ´Ù. ÀÌ °Ë»ç´Â

Ig(ƯÈ÷ IgG)À̳ª complement(ƯÈ÷ C3)°¡ ÀûÇ÷±¸¿¡ ÀÀÁýÇÏ´Â ´É·ÂÀ» º¸°í °Ë»çÇÑ´Ù. direct

Coombs test´Â anti-IgG or anti-C3 antisera°¡ ȯÀÚ RBC¿¡ ÀÀÁýÇÏ´Â ´É·ÂÀ» ÃøÁ¤ÇÏ´Â

°ÍÀÌ´Ù. IgG and/or C3ÀÇ Á¸ÀçÀ¯¹«´Â immune hemolytic anemiaÀÇ ¿øÀÎÀ» ¹àÈ÷´Âµ¥ µµ¿òÀÌ

µÈ´Ù(Tab 108-6). µå¹°°Ô IgG, complementµÑ´Ù RBC¿¡ ¹ß°ßµÇÁö ¾Ê´Â °æ¿ìµµ Àִµ¥ À̸¦

Coombs-negative immune hemolytic anemia¶ó ÇÑ´Ù. IgM Ab(ÈçÈ÷ cold-reacting)´Â

normal or fetal RBCÀÇ ÀÀÁý¿¡ ÀÇÇØ °ËÃâµÈ´Ù. indirect Coombs test¿¡ ÀÇÇØ IgG Ab°¡

°ËÃâµÇ±âµµ ÇÑ´Ù.

(1) "warm" Ab

protein Ag°ú ¹ÝÀÀÇÏ´Â Ab´Â °ÅÀÇ Ç×»ó IgGÀ̸ç ü¿Â¿¡¼­ ¹ÝÀÀÇÑ´Ù.

°£È¤ IgA, µå¹°°Ô´Â IgMÀϼöµµ ÀÖ´Ù. autologous Ab¿¡ ÀÇÇÑ ¿ëÇ÷Àº autoimmune

hemolytic(or immunohemolytic) anemia(=warm Ab type)¶ó Çϱ⵵ ÇÑ´Ù.

¨ç ÀÓ»ó¹ßÇö

warm Ab typeÀÇ immunohemolytic anemia´Â IgG Ab¿¡ ÀÇÇØ À¯µµµÇ¸ç ¸ðµç ³ªÀÌ¿¡ ¹ß»ý

ÇÒ ¼ö ÀÖÁö¸¸ ¼ºÀΠƯÈ÷ ¿©¼º¿¡¼­ ´õ ÈçÇÏ´Ù. ´ë·« ȯÀÚÀÇ 1/4ÀÌ ¸é¿ª°è¸¦ ħ¹üÇÏ´Â ±âÀú

Áúȯ, ƯÈ÷ lymphoid neoplasm, collagen vascular disease, ƯÈ÷ SLE & congenital

immunodeficiency diseaseÀÇ ÇÕº´ÁõÀ¸·Î ¹ß»ýÇÑ´Ù(Tab 108-7).

°æÇÒ¶§´Â ´ÜÁö direct Coombs test¸¸ ¾ç¼ºÀÌ´Ù.

severe immunohemolytic anemia´Â hemoglobinemia, hemoglobinuria, shock°ú µ¿¹ÝµÈ

fulminant hemolysis·Î ¹ßÇöÇÑ´Ù. ÀÌ´Â Àû±ØÀûÀ¸·Î Ä¡·áÇÏÁö ¾ÊÀ¸¸é Ä¡¸íÀûÀÌ´Ù. ÈçÈ÷

IgG(¡¾C3)¸¸ °ËÃâµÈ´Ù. immune thrombocytopenia°¡ ¶ÇÇÑ »ý±æ¼ö ÀÖ´Ù(Evans's

syndrome).

¨è º´ÀÎ

IgG Ab´Â µÎ°¡Áö ±âÀüÀ¸·Î ÀûÇ÷±¸¸¦ ÆÄ±«ÇÑ´Ù.

i) Ab & C'ÀÇ ¸Å°³¿¡ ÀÇÇØ RBC¿¡ phagocyte°¡ °áÇÕÇϵµ·Ï ÇÑ´Ù(º¸´Ù Áß¿äÇÑ ±âÀü)

ii) complement activation

Ab-coated red cellÀÌ Fc receptor¿¡ °áÇÕµÈ macrophage¿¡ Ž½ÄµÇ¾î ÆÄ±«

¨é Ä¡·á

°æÇѰæ¿ì¿£ Ä¡·á°¡ ÇÊ¿ä¾ø´Ù.

½ÉÇÑ ¿ëÇ÷½Ã glucocorticoids(Pd 1.0 mg/kg/d)

Hb »ó½ÂÀº 3-4Àϳ» ÈçÈ÷ ¹ß°ßµÇ°í 1-2ÁÖ³» ´ëºÎºÐÀÇ È¯ÀÚ¿¡¼­ ÀϾ´Ù. Hb levelÀÌ Á¤»ó

Ä¡°¡µÉ¶§±îÁö °è¼Ó »ç¿ëÇÑÈÄ 20 mg/d·Î ºü¸£°Ô ÁÙÀ̰í, ±×ÈÄ ¼ö°³¿ù¿¡ °ÉÃÄ ¼­¼­È÷

ÁÙÀδÙ.

Pd chronic tx¶§´Â alternative-day tx°¡ ´õ ³´´Ù. ȯÀÚÀÇ 75% ÀÌ»óÀº »ó´çÇÑ ¿ëÇ÷°¨¼Ò¸¦

º¸ÀÌÁö¸¸ ±×Áß ¹ÝÀº ÁÙÀ̰ųª Ä¡·á Áߴܽà Àç¹ßÇÑ´Ù.

glucocorticoids´Â µÎ°¡Áö ±âÀüÀ¸·Î ÀÛ¿ëÇÑ´Ù.

i) IgG-coated RBC°¡ mononuclear phagocyte system¿¡ ÀÇÇØ Á¦°ÅµÇ´Â °ÍÀ» ¾ïÁ¦

ii) AbÇÕ¼º ¾ïÁ¦

glucocorticoid tx¸¦ °ßµðÁö ¸øÇϰųª ¹ÝÀÀÇÏÁö ¾ÊÀ» ¶§ splenectomy°¡ ±ÇÀåµÈ´Ù.

glucocorticoid tx, splenectomy¿¡ ¹ÝÀÀÇÏÁö ¾Ê´Â ȯÀÚ´Â azathioprine or

cyclophosphamide°°Àº ¸é¿ª¾ïÁ¦Á¦·Î Ä¡·áÇÑ´Ù. ¼º°ø·üÀº ¡­50% Á¤µµ·Î º¸°íµÇ°í ÀÖ´Ù.

IV gammaglobulinÀº ºü¸£°Ô ¿ëÇ÷À» ÁßÁö½ÃŰÁö¸¸ ITP¸¸Å­ È¿°úÀûÀÌÁö´Â ¾Ê´Ù.

Ab°¡ "panagglutinin"À̹ǷΠ¸ðµç Á¤»óÀûÀÎ donor cells°úµµ ¹ÝÀÀÇÏ¿© cross-matchingÀÌ

ºÒ°¡´ÉÇÏ´Ù. ¼öÇ÷¿¡ ÇÊ¿äÇÑ Ç÷¾×À» °í¸£´Â ¸ñÀûµµ Ç׿øÀ» °¡Áø ÀûÇ÷±¸ Åõ¿©¸¦ ÇÇÇϰíÀÚ

ÇÔÀÌ´Ù.

¹æ¹ýÀº ȯÀÚÀÇ Ç÷û³» Á¸ÀçÇÏ´Â panagglutininÀ» ȯÀÚ ÀÚ½ÅÀÇ RBC¿¡ ÈíÂø½ÃŰ´Â °ÍÀÌ´Ù.

autoAb°¡ Á¦°ÅµÈ Ç÷ûÀº donor blood groups¿¡ ´ëÇÑ alloAbÀÇ Á¸À縦 °Ë»çÇÒ¼ö ÀÖ´Ù.

ABO-compatible RBC´Â immediate-type hemolytic transfusion reaction¿¡ ÁÖÀÇÇϸ鼭

õõÈ÷ Åõ¿©ÇÑ´Ù.

¨ê ¿¹ÈÄ

´ëºÎºÐ glucocorticoid ´Üµ¶ ȤÀº splenectomy¿ÍÀÇ º´ÇÕÄ¡·á·Î Á¶ÀýµÈ´Ù.

Ä¡¸í·üÀº ´ÙÀ½ ¼¼°¡Áö µå¹® °æ¿ì¿¡¼­ ¹ß»ýÇÑ´Ù.

i) overwhelming hemolysis

ii) glucocorticoids, splenectomy and/or immunosuppressive agent·Î host defense¼Õ»ó

iii) active hemolysis¿¡ µ¿¹ÝµÈ major thrombotic event

immunohemolysis°¡ ±âÀúÁúȯÀÇ ÇÕº´ÁõÀ¸·Î ¹ß»ýÇÒ ¶§ ¿¹ÈÄ´Â primary disease¿¡ ÁÖ·Î

´Þ·ÁÀÖ´Ù.