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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

Bone marrow free immune checkpoints as a potential biomarker for differential diagnosis of acquired bone marrow failures.
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
The value of next-generation sequencing in routine diagnostics and management of patients with cytopenia.
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
Oligo-monocytic CMML and other pre-CMML states: Clinical impact, prognostication and management.
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

Clinical implications and genetic features of clonal cytopenia of undetermined significance compared to lower-risk myelodysplastic syndrome.
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
The value of next-generation sequencing in routine diagnostics and management of patients with cytopenia.
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
Oligo-monocytic CMML and other pre-CMML states: Clinical impact, prognostication and management.
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
ASXL1 is a molecular predictor in idiopathic cytopenia of undetermined significance.
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
Idiopathic cytopenia of undetermined significance (ICUS) and idiopathic dysplasia of uncertain significance (IDUS), and their distinction from low risk MDS.
Valent P, Bain BJ, Bennett JM, Wimazal F, Sperr WR, Mufti G, Horny HP
Leuk Res 2012 Jan;36(1):1-5. Epub 2011 Sep 13 doi: 10.1016/j.leukres.2011.08.016. PMID: 21920601

Diagnosis

Clinical implications and genetic features of clonal cytopenia of undetermined significance compared to lower-risk myelodysplastic syndrome.
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
Oligo-monocytic CMML and other pre-CMML states: Clinical impact, prognostication and management.
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
ASXL1 is a molecular predictor in idiopathic cytopenia of undetermined significance.
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
ICUS/CCUS/CHIP: basics & beyond.
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
Idiopathic cytopenia of undetermined significance (ICUS) and idiopathic dysplasia of uncertain significance (IDUS), and their distinction from low risk MDS.
Valent P, Bain BJ, Bennett JM, Wimazal F, Sperr WR, Mufti G, Horny HP
Leuk Res 2012 Jan;36(1):1-5. Epub 2011 Sep 13 doi: 10.1016/j.leukres.2011.08.016. PMID: 21920601

Therapy

Discordant pathologic diagnoses of myelodysplastic neoplasms and their implications for registries and therapies.
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
Health-related quality of life and vulnerability among people with myelodysplastic syndromes: a US national study.
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
Flow cytometric analysis of peripheral blood neutrophil myeloperoxidase expression for ruling out myelodysplastic syndromes: a prospective validation study.
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
Erythropoietin Receptors and IgG Autoantibody Expression on Nucleated Erythrocytes in Some Cases of Immuno-Related Pancytopenia.
Liu H, Fu R, Li L, Ding K, Wang Y, Wang H, Zhang T, Wang G, Song J, Shao Z
Clin Lab 2015;61(7):693-8. doi: 10.7754/clin.lab.2014.141227. PMID: 26299067
Distinguishing immunorelated haemocytopenia from idiopathic cytopenia of undetermined significance (ICUS): a bone marrow abnormality mediated by autoantibodies.
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

Clinical implications and genetic features of clonal cytopenia of undetermined significance compared to lower-risk myelodysplastic syndrome.
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
Incidence and prognosis of clonal hematopoiesis in patients with chronic idiopathic neutropenia.
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
Oligo-monocytic CMML and other pre-CMML states: Clinical impact, prognostication and management.
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
ICUS/CCUS/CHIP: basics & beyond.
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
Idiopathic cytopenia of undetermined significance (ICUS) and idiopathic dysplasia of uncertain significance (IDUS), and their distinction from low risk MDS.
Valent P, Bain BJ, Bennett JM, Wimazal F, Sperr WR, Mufti G, Horny HP
Leuk Res 2012 Jan;36(1):1-5. Epub 2011 Sep 13 doi: 10.1016/j.leukres.2011.08.016. PMID: 21920601

Clinical prediction guides

Discordant pathologic diagnoses of myelodysplastic neoplasms and their implications for registries and therapies.
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
Relationship between clone metrics and clinical outcome in clonal cytopenia.
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
ASXL1 is a molecular predictor in idiopathic cytopenia of undetermined significance.
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
Impact of chromosome alterations, genetic mutations and clonal hematopoiesis of indeterminate potential (CHIP) on the classification and risk stratification of MDS.
Ganguly BB, Banerjee D, Agarwal MB
Blood Cells Mol Dis 2018 Mar;69:90-100. Epub 2017 Oct 16 doi: 10.1016/j.bcmd.2017.10.001. PMID: 29079134
ICUS/CCUS/CHIP: basics & beyond.
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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