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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

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
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
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

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
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

Therapy

Association between myeloid disorders and adult onset-inflammatory syndromes, successful treatment with JAK-inhibitors: Case series and literature review.
Mishra R, Calabrese C, Jain AG, Singh A
Leuk Res 2024 Nov;146:107584. Epub 2024 Sep 4 doi: 10.1016/j.leukres.2024.107584. PMID: 39243744
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
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

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
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

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
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
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
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
Mutations in idiopathic cytopenia of undetermined significance assist diagnostics and correlate to dysplastic changes.
Hansen JW, Westman MK, Sjö LD, Saft L, Kristensen LS, Ørskov AD, Treppendahl M, Andersen MK, Grønbaek K
Am J Hematol 2016 Dec;91(12):1234-1238. Epub 2016 Nov 8 doi: 10.1002/ajh.24554. PMID: 27717004

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