cytopenia(aplastic anemia, agranulocytosis)

posted on 25 Apr 2013 15:52 by ononmedicine in diesease
Aplastic anemia
ภาวะไขกระดูกฝ่อ
เกิดจากการทีไขกระดูกสร้างเม็ดเลือดได้น้อย ทำให้ผู้ป่วยมีภาวะ pancytopenia
โรคนี้พบไม่บ่อยมีอุบัติการณ์ ในประเทศไทยมีอุบัติการณ์ประมาณ 3.9-5.0 ต่อประชากรล้านคนต่อปี โดยพบที่ภาคตะวันออกเฉียงเหนือสูงที่สุด
พยาธิกำเนิดของโรคนี้ส้วนใหญ่เกี่ยวข้องกับระบบภูมิคุ้มกันที่ผิดปกติซึ่งมีผลทำลายเซลล์ต้นกำเนิด ในไขกระดูก การตดิเช้ือไวรัสบางชนิดเช่น  Parvovirus B19, Epstein-Barr virus , Hepatitis B virus และการได้รับรังสีในขนาดสูง
แม้ว่าโรคไขกระดูกฝ่อจะพบได้น้อยแต่ก็มีความส าคัญเนื่องจากเป็ นโรคที่มีอาการรุนแรงผู้ป่วยมีอัตราตายสูง
 
[Severe Aplastic anemia]
วินิจฉัยว่าผู้ป่วยมีอาการรุนแรง เมื่อผู้ป่วยตรวจเลือดตามเกณฑ์อย่างน้อย 2 ข้อ ร่วมกับภาวะ
hypocellular bone marrow ตามเกณฑ์ที่กำหนดคือ
 Anemia with corrected reticulocyte count <1%
 Absolute neutrophil count (ANC) <0.5 x 109/L
 Platelet count < 20 x 109/L
 Hypocellular bone marrow
(<25% of cellularity or moderate hypocellularity with <30% of hematopoietic cells)
 
[Very Severe Aplastic Anemia] วินิจฉัยว่าโรคมีอาการรุนแรงมาก  เมื่อผู้ป่วยมีผลการตรวจเลือดและไขกระดูกเข้าเกณฑ์การวินิจฉัย
severe aplastic anemia แต่มี  ANC <0.2 x 109/L
 
[Non Severe Aplastic Anemia] วินิจฉัยว่าโรคมีอาการไม่รุนแรง  เมื่อผู้ป่วยเป็นโรคไขกระดูกฝ่อแต่มีผลการตรวจเลือดและไขกระดูก
ไม่เข้าเกณฑ์ severe aplastic anemia โดยมีผลการตรวจเลือดตามเกณฑ์อย่างน้อย 2 ข้อ
ร่วมกับมีภาวะ hypocellular bone marrow ตามเกณฑ์ที่กำหนดคือ
 Anemia with corrected reticulocyte count <1%
 Absolute neutrophil count (ANC)<1.5 x 109/L
 Platelet count <50 x 109/L
 Hypocellular bone marrow  มีจำนวนเซลล์ในไขกระดูกน้อยกว่าปกติ
 แต่ไม่เข้าเกณฑ์ severe aplastic anemia
 
[แนวทางการวินิจฉัย]
การวินิจฉัยโรคไขกระดูกฝ่อ อาศัยผลการตรวจเลือด พบมีภาวะ pancytopenia
ร่วมกับการตรวจไขกระดูกพบมี  hypocellular bone marrow
ควรซักประวัติการทำงาน การได้รับเคมีบำบัด สัมผัสรังสี ประวัติคนในครอบครัว
เพื่อแยกอย่างอื่นที่เหมือนกัน
คือ hypoplastic myelodysplastic syndrome (MDS)
     hypoplastic leukemia
ในผู้ป่วยเด็กจำเป็นต้องแยกภาวะ acquired aplastic anemia(ภาวะไขกระดูกฝ่อชนิดพันธุกรรม)
ลักษณะของไขกระดูกฝ่อชนิดพันธุกรรม
คือการพบไขกระดูกฝ่อร่วมกับความผิดปกติทางกายภาพ ที่พบบ่อยมี 3 โรค
1.Fanconi anemia
2.Dyskeratosis congenita
3.Shwachman-Diamond syndrome
ผู้ป่วยกลุ่มนี้มักมีน้ำหนักแรกคลอดน้อย
 
[Agranulocytosis]
คือ ภาวะที่มีเม็ดเลือดขาวน้อยกว่า 250 ตัวฝ/มม.
 
http://cai.md.chula.ac.th/lesson/lesson4403-6/content/main_story03.html
 

edit @ 25 Apr 2013 22:04:56 by Ying_orn_lunlar

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