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Very Severe Aplastic Anemia

MedGen UID:
1732130
Concept ID:
C5417733
Disease or Syndrome

Definition

Aplastic anemia that is characterized by bone marrow (BM) cellularity less than 25% (or less than 50% if BM is comprised of less than 30% hematopoietic cells), and two or more of the following: absolute neutrophil count of less than 200 per microliter; platelet count of less than 20,000 per microliter; or an absolute reticulocyte count of less than 20,000 per microliter. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVery Severe Aplastic Anemia

Professional guidelines

PubMed

Wali R, Fadoo Z, Adil S, Naqvi MA
J Coll Physicians Surg Pak 2011 Apr;21(4):219-22. PMID: 21453618
Shichino H, Mugishima H, Takamura M, Shimada T, Suzuki T, Chin M, Harada K, Ryo S, Kojima S
Acta Paediatr Jpn 1996 Dec;38(6):644-7. doi: 10.1111/j.1442-200x.1996.tb03724.x. PMID: 9002302
Facon T, Walter MP, Fenaux P, Morel P, Dupriez B, Gardin C, Jouet JP, Bauters F
Ann Hematol 1991 Aug;63(2):89-93. doi: 10.1007/BF01707279. PMID: 1912037

Recent clinical studies

Etiology

Tan X, Tian Y, Li Z, Cui S, Wang Z, Zhou Y, Liu Z, Kong F, Wang Y, Xu R
Nutr Hosp 2022 Dec 20;39(6):1289-1297. doi: 10.20960/nh.03957. PMID: 36173198
Wang ZJ, Chen HB, Zhou F, Yu H, Wu XY, Shen YQ, Qiu YN, Jin RM
Curr Med Sci 2022 Apr;42(2):379-386. Epub 2022 Mar 8 doi: 10.1007/s11596-022-2519-2. PMID: 35258748
Norasetthada L, Wongkhantee S, Chaipokam J, Charoenprasert K, Chuncharunee S, Rojnuckarin P, Sirijerachai C, Wanachiwanawin W, Issaragrisil S; Thai Aplastic Anemia Study Group
Ann Hematol 2021 Oct;100(10):2443-2452. Epub 2021 Jul 16 doi: 10.1007/s00277-021-04566-0. PMID: 34269837Free PMC Article
Vaht K, Göransson M, Carlson K, Isaksson C, Lenhoff S, Sandstedt A, Uggla B, Winiarski J, Ljungman P, Brune M, Andersson PO
Haematologica 2017 Oct;102(10):1683-1690. Epub 2017 Jul 27 doi: 10.3324/haematol.2017.169862. PMID: 28751565Free PMC Article
Gupta V, Kumar S, Sonowal R, Singh SK
J Pediatr Hematol Oncol 2017 Apr;39(3):214-216. doi: 10.1097/MPH.0000000000000724. PMID: 28060106

Diagnosis

Sharma V, Kumar P, Kumar R, Chakraborty S, Namdeo M, Sazawal S, Kanga U, Seth T, Mitra DK
Clin Exp Immunol 2022 May 12;207(3):272-278. doi: 10.1093/cei/uxab006. PMID: 35553631Free PMC Article
Wang ZJ, Chen HB, Zhou F, Yu H, Wu XY, Shen YQ, Qiu YN, Jin RM
Curr Med Sci 2022 Apr;42(2):379-386. Epub 2022 Mar 8 doi: 10.1007/s11596-022-2519-2. PMID: 35258748
Norasetthada L, Wongkhantee S, Chaipokam J, Charoenprasert K, Chuncharunee S, Rojnuckarin P, Sirijerachai C, Wanachiwanawin W, Issaragrisil S; Thai Aplastic Anemia Study Group
Ann Hematol 2021 Oct;100(10):2443-2452. Epub 2021 Jul 16 doi: 10.1007/s00277-021-04566-0. PMID: 34269837Free PMC Article
Gupta V, Kumar S, Sonowal R, Singh SK
J Pediatr Hematol Oncol 2017 Apr;39(3):214-216. doi: 10.1097/MPH.0000000000000724. PMID: 28060106
Höchsmann B, Moicean A, Risitano A, Ljungman P, Schrezenmeier H
Bone Marrow Transplant 2013 Feb;48(2):168-73. Epub 2012 Dec 3 doi: 10.1038/bmt.2012.220. PMID: 23208312

Therapy

Wang ZJ, Chen HB, Zhou F, Yu H, Wu XY, Shen YQ, Qiu YN, Jin RM
Curr Med Sci 2022 Apr;42(2):379-386. Epub 2022 Mar 8 doi: 10.1007/s11596-022-2519-2. PMID: 35258748
Weiler-Normann C, Hartl J, Weidemann S, von Pein UM, Fiedler W, Schramm C, Brinkert F, Kröger N, Christopeit M
Z Gastroenterol 2018 Jan;56(1):51-54. Epub 2018 Jan 9 doi: 10.1055/s-0043-121737. PMID: 29316578
Gupta V, Kumar S, Sonowal R, Singh SK
J Pediatr Hematol Oncol 2017 Apr;39(3):214-216. doi: 10.1097/MPH.0000000000000724. PMID: 28060106
Höchsmann B, Moicean A, Risitano A, Ljungman P, Schrezenmeier H
Bone Marrow Transplant 2013 Feb;48(2):168-73. Epub 2012 Dec 3 doi: 10.1038/bmt.2012.220. PMID: 23208312
Gupta V, Kumar A, Tilak V, Saini I, Bhatia B
Indian J Pediatr 2012 Dec;79(12):1587-91. Epub 2012 Jan 25 doi: 10.1007/s12098-012-0691-2. PMID: 22274992

Prognosis

Tan X, Tian Y, Li Z, Cui S, Wang Z, Zhou Y, Liu Z, Kong F, Wang Y, Xu R
Nutr Hosp 2022 Dec 20;39(6):1289-1297. doi: 10.20960/nh.03957. PMID: 36173198
Sharma V, Kumar P, Kumar R, Chakraborty S, Namdeo M, Sazawal S, Kanga U, Seth T, Mitra DK
Clin Exp Immunol 2022 May 12;207(3):272-278. doi: 10.1093/cei/uxab006. PMID: 35553631Free PMC Article
Norasetthada L, Wongkhantee S, Chaipokam J, Charoenprasert K, Chuncharunee S, Rojnuckarin P, Sirijerachai C, Wanachiwanawin W, Issaragrisil S; Thai Aplastic Anemia Study Group
Ann Hematol 2021 Oct;100(10):2443-2452. Epub 2021 Jul 16 doi: 10.1007/s00277-021-04566-0. PMID: 34269837Free PMC Article
Höchsmann B, Moicean A, Risitano A, Ljungman P, Schrezenmeier H
Bone Marrow Transplant 2013 Feb;48(2):168-73. Epub 2012 Dec 3 doi: 10.1038/bmt.2012.220. PMID: 23208312
Sharma R, Chandra J, Sharma S, Pemde H, Singh V
J Pediatr Hematol Oncol 2012 Mar;34(2):93-5. doi: 10.1097/MPH.0b013e31823c287b. PMID: 22278201

Clinical prediction guides

Sharma V, Kumar P, Kumar R, Chakraborty S, Namdeo M, Sazawal S, Kanga U, Seth T, Mitra DK
Clin Exp Immunol 2022 May 12;207(3):272-278. doi: 10.1093/cei/uxab006. PMID: 35553631Free PMC Article
Norasetthada L, Wongkhantee S, Chaipokam J, Charoenprasert K, Chuncharunee S, Rojnuckarin P, Sirijerachai C, Wanachiwanawin W, Issaragrisil S; Thai Aplastic Anemia Study Group
Ann Hematol 2021 Oct;100(10):2443-2452. Epub 2021 Jul 16 doi: 10.1007/s00277-021-04566-0. PMID: 34269837Free PMC Article
Gupta V, Kumar S, Sonowal R, Singh SK
J Pediatr Hematol Oncol 2017 Apr;39(3):214-216. doi: 10.1097/MPH.0000000000000724. PMID: 28060106
Chandar R, Chandrasekaran V, Jagadisan B, Kumar D, Biswal N
J Pediatr Hematol Oncol 2017 Jan;39(1):e43-e45. doi: 10.1097/MPH.0000000000000671. PMID: 27548335
Höchsmann B, Moicean A, Risitano A, Ljungman P, Schrezenmeier H
Bone Marrow Transplant 2013 Feb;48(2):168-73. Epub 2012 Dec 3 doi: 10.1038/bmt.2012.220. PMID: 23208312

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