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Idiopathic Cytopenia of Undetermined Significance

MedGen UID:
1715670
Concept ID:
C5392077
Disease or Syndrome
Synonyms: ICUS Idiopathic Cytopenias of Undetermined Significance; Idiopathic Cytopenias of Undetermined Significance

Definition

Cytopenia in one or more hematopoietic cell lineages that remains unexplained following thorough evaluation. The hematopoietic cell dysplasia is mild and does not fulfill the criteria for myelodysplastic syndrome, but the cytopenia may be severe. There is no evidence of clonal hematopoiesis or the variant allele frequency (VAF) is less that 2 percent. There is no other evidence of hematologic malignancy. It may progress to an overt myelodysplastic syndrome or other myeloid neoplasms such as acute myeloid leukemia, myeloproliferative neoplasm, or mast cell disorder. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic Cytopenia of Undetermined Significance

Professional guidelines

PubMed

Zang M, Li N, Chen Q, Ran N, Fu R, Shao Z, Wang T
J Clin Lab Anal 2022 Oct;36(10):e24677. Epub 2022 Sep 9 doi: 10.1002/jcla.24677. PMID: 36086857Free PMC Article
Breinholt MF, Nielsen K, Schejbel L, Fassi DE, Schöllkopf C, Novotny GW, Mortensen BK, Ahmad A, Høgdall E, Nørgaard P
Int J Lab Hematol 2022 Jun;44(3):531-537. Epub 2022 Feb 10 doi: 10.1111/ijlh.13802. PMID: 35142436
Valent P
Best Pract Res Clin Haematol 2020 Jun;33(2):101137. Epub 2019 Dec 15 doi: 10.1016/j.beha.2019.101137. PMID: 32460976

Recent clinical studies

Etiology

Gorak EJ, Otterstatter M, Al Baghdadi T, Gillis N, Foran JM, Liu JJ, Bejar R, Gore SD, Kroft SH, Harrington A, Saber W, Starczynowski D, Rollison DE, Zhang L, Moscinski L, Wilson S, Thompson J, Borchert C, Sherman S, Hebert D, Walker ME, Padron E, DeZern AE, Sekeres MA
Blood Adv 2023 Oct 24;7(20):6120-6129. doi: 10.1182/bloodadvances.2023010061. PMID: 37552083Free PMC Article
Abel GA, Hebert D, Lee C, Rollison D, Gillis N, Komrokji R, Foran JM, Liu JJ, Al Baghdadi T, Deeg J, Gore S, Saber W, Wilson S, Otterstatter M, Thompson J, Borchert C, Padron E, DeZern A, Cella D, Sekeres MA
Blood Adv 2023 Jul 25;7(14):3506-3515. doi: 10.1182/bloodadvances.2022009000. PMID: 37146263Free PMC Article
Choi EJ, Cho YU, Hur EH, Park HS, Choi Y, Lee JH, Lee KH, Kim M, Hwang SH, Jang S, Park CJ, Seo EJ, Lee JH
Br J Haematol 2022 Aug;198(4):703-712. Epub 2022 May 25 doi: 10.1111/bjh.18273. PMID: 35612271
Tsaknakis G, Gallì A, Papadakis S, Kanellou P, Elena C, Todisco G, Bono E, Rizzo E, Molteni E, Fragiadaki I, Mavroudi I, Pontikoglou C, Batas A, Maxouri S, Linardaki E, Tavernarakis N, Malcovati L, Papadaki HA
Blood 2021 Oct 7;138(14):1249-1257. doi: 10.1182/blood.2021010815. PMID: 34166485
Shin DY, Park JK, Kim SM, Im K, Kim JA, Kim SY, Hwang SM, Yoon SS, Lee DS
Leuk Lymphoma 2019 Mar;60(3):756-763. Epub 2018 Oct 10 doi: 10.1080/10428194.2018.1492129. PMID: 30301403

Diagnosis

Gorak EJ, Otterstatter M, Al Baghdadi T, Gillis N, Foran JM, Liu JJ, Bejar R, Gore SD, Kroft SH, Harrington A, Saber W, Starczynowski D, Rollison DE, Zhang L, Moscinski L, Wilson S, Thompson J, Borchert C, Sherman S, Hebert D, Walker ME, Padron E, DeZern AE, Sekeres MA
Blood Adv 2023 Oct 24;7(20):6120-6129. doi: 10.1182/bloodadvances.2023010061. PMID: 37552083Free PMC Article
Abel GA, Hebert D, Lee C, Rollison D, Gillis N, Komrokji R, Foran JM, Liu JJ, Al Baghdadi T, Deeg J, Gore S, Saber W, Wilson S, Otterstatter M, Thompson J, Borchert C, Padron E, DeZern A, Cella D, Sekeres MA
Blood Adv 2023 Jul 25;7(14):3506-3515. doi: 10.1182/bloodadvances.2022009000. PMID: 37146263Free PMC Article
Choi EJ, Cho YU, Hur EH, Park HS, Choi Y, Lee JH, Lee KH, Kim M, Hwang SH, Jang S, Park CJ, Seo EJ, Lee JH
Br J Haematol 2022 Aug;198(4):703-712. Epub 2022 May 25 doi: 10.1111/bjh.18273. PMID: 35612271
Tsaknakis G, Gallì A, Papadakis S, Kanellou P, Elena C, Todisco G, Bono E, Rizzo E, Molteni E, Fragiadaki I, Mavroudi I, Pontikoglou C, Batas A, Maxouri S, Linardaki E, Tavernarakis N, Malcovati L, Papadaki HA
Blood 2021 Oct 7;138(14):1249-1257. doi: 10.1182/blood.2021010815. PMID: 34166485
Jain M, Tripathi A
Expert Rev Hematol 2017 Oct;10(10):915-920. Epub 2017 Sep 1 doi: 10.1080/17474086.2017.1371588. PMID: 28832236

Therapy

Gorak EJ, Otterstatter M, Al Baghdadi T, Gillis N, Foran JM, Liu JJ, Bejar R, Gore SD, Kroft SH, Harrington A, Saber W, Starczynowski D, Rollison DE, Zhang L, Moscinski L, Wilson S, Thompson J, Borchert C, Sherman S, Hebert D, Walker ME, Padron E, DeZern AE, Sekeres MA
Blood Adv 2023 Oct 24;7(20):6120-6129. doi: 10.1182/bloodadvances.2023010061. PMID: 37552083Free PMC Article
Patel JL, Erba HP, Savona MR, Grinblatt DL, Clark M, Clive TC, Smart TB, Makinde AY, DeGutis IS, Yu E, Eggington JM, George TI
J Mol Diagn 2023 Aug;25(8):611-618. doi: 10.1016/j.jmoldx.2023.05.002. PMID: 37517825
Abel GA, Hebert D, Lee C, Rollison D, Gillis N, Komrokji R, Foran JM, Liu JJ, Al Baghdadi T, Deeg J, Gore S, Saber W, Wilson S, Otterstatter M, Thompson J, Borchert C, Padron E, DeZern A, Cella D, Sekeres MA
Blood Adv 2023 Jul 25;7(14):3506-3515. doi: 10.1182/bloodadvances.2022009000. PMID: 37146263Free PMC Article
Raskovalova T, Jacob MC, Bulabois CE, Mariette C, Scheffen L, Park S, Labarère J
Ann Hematol 2021 May;100(5):1149-1158. Epub 2021 Feb 10 doi: 10.1007/s00277-021-04446-7. PMID: 33569703
Fu R, Liu H, Wang Y, Liu H, He H, Chen J, Wang H, Yu H, Ding K, Huang L, Wang H, Xing L, Song J, Li L, Shao Z
Clin Exp Immunol 2014 Aug;177(2):412-8. doi: 10.1111/cei.12347. PMID: 24730511Free PMC Article

Prognosis

Gorak EJ, Otterstatter M, Al Baghdadi T, Gillis N, Foran JM, Liu JJ, Bejar R, Gore SD, Kroft SH, Harrington A, Saber W, Starczynowski D, Rollison DE, Zhang L, Moscinski L, Wilson S, Thompson J, Borchert C, Sherman S, Hebert D, Walker ME, Padron E, DeZern AE, Sekeres MA
Blood Adv 2023 Oct 24;7(20):6120-6129. doi: 10.1182/bloodadvances.2023010061. PMID: 37552083Free PMC Article
Abel GA, Hebert D, Lee C, Rollison D, Gillis N, Komrokji R, Foran JM, Liu JJ, Al Baghdadi T, Deeg J, Gore S, Saber W, Wilson S, Otterstatter M, Thompson J, Borchert C, Padron E, DeZern A, Cella D, Sekeres MA
Blood Adv 2023 Jul 25;7(14):3506-3515. doi: 10.1182/bloodadvances.2022009000. PMID: 37146263Free PMC Article
Choi EJ, Cho YU, Hur EH, Park HS, Choi Y, Lee JH, Lee KH, Kim M, Hwang SH, Jang S, Park CJ, Seo EJ, Lee JH
Br J Haematol 2022 Aug;198(4):703-712. Epub 2022 May 25 doi: 10.1111/bjh.18273. PMID: 35612271
Tsaknakis G, Gallì A, Papadakis S, Kanellou P, Elena C, Todisco G, Bono E, Rizzo E, Molteni E, Fragiadaki I, Mavroudi I, Pontikoglou C, Batas A, Maxouri S, Linardaki E, Tavernarakis N, Malcovati L, Papadaki HA
Blood 2021 Oct 7;138(14):1249-1257. doi: 10.1182/blood.2021010815. PMID: 34166485
Jain M, Tripathi A
Expert Rev Hematol 2017 Oct;10(10):915-920. Epub 2017 Sep 1 doi: 10.1080/17474086.2017.1371588. PMID: 28832236

Clinical prediction guides

Gorak EJ, Otterstatter M, Al Baghdadi T, Gillis N, Foran JM, Liu JJ, Bejar R, Gore SD, Kroft SH, Harrington A, Saber W, Starczynowski D, Rollison DE, Zhang L, Moscinski L, Wilson S, Thompson J, Borchert C, Sherman S, Hebert D, Walker ME, Padron E, DeZern AE, Sekeres MA
Blood Adv 2023 Oct 24;7(20):6120-6129. doi: 10.1182/bloodadvances.2023010061. PMID: 37552083Free PMC Article
Abel GA, Hebert D, Lee C, Rollison D, Gillis N, Komrokji R, Foran JM, Liu JJ, Al Baghdadi T, Deeg J, Gore S, Saber W, Wilson S, Otterstatter M, Thompson J, Borchert C, Padron E, DeZern A, Cella D, Sekeres MA
Blood Adv 2023 Jul 25;7(14):3506-3515. doi: 10.1182/bloodadvances.2022009000. PMID: 37146263Free PMC Article
Gallì A, Todisco G, Catamo E, Sala C, Elena C, Pozzi S, Bono E, Ferretti VV, Rizzo E, Molteni E, Zibellini S, Sarchi M, Boveri E, Ferrari J, Fiorelli N, Camaschella C, Gasparini P, Toniolo D, Cazzola M, Malcovati L
Blood 2021 Sep 16;138(11):965-976. doi: 10.1182/blood.2021011323. PMID: 34255818
Shin DY, Park JK, Kim SM, Im K, Kim JA, Kim SY, Hwang SM, Yoon SS, Lee DS
Leuk Lymphoma 2019 Mar;60(3):756-763. Epub 2018 Oct 10 doi: 10.1080/10428194.2018.1492129. PMID: 30301403
Jain M, Tripathi A
Expert Rev Hematol 2017 Oct;10(10):915-920. Epub 2017 Sep 1 doi: 10.1080/17474086.2017.1371588. PMID: 28832236

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