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Chronic idiopathic neutropenia

MedGen UID:
384521
•Concept ID:
C2267231
•
Disease or Syndrome
Synonyms: Chronic Idiopathic Neutropenia; Idiopathic agranulocytosis
SNOMED CT: Chronic idiopathic neutropenia (248693006); Idiopathic agranulocytosis (248693006)

Definition

A rare hematologic disorder characterized by neutropenia lasting more than three months with no identifiable cause. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic idiopathic neutropenia

Professional guidelines

PubMed

Discerning clinicopathological features of congenital neutropenia syndromes: an approach to diagnostically challenging differential diagnoses.
Parisi X, Bledsoe JR
J Clin Pathol 2024 Aug 16;77(9):586-604. doi: 10.1136/jcp-2022-208686. PMID: 38589208
An update on the diagnosis and treatment of chronic idiopathic neutropenia.
Dale DC, Bolyard AA
Curr Opin Hematol 2017 Jan;24(1):46-53. doi: 10.1097/MOH.0000000000000305. PMID: 27841775Free PMC Article
Pathophysiologic mechanisms, clinical features and treatment of idiopathic neutropenia.
Papadaki HA, Pontikoglou C
Expert Rev Hematol 2008 Dec;1(2):217-29. doi: 10.1586/17474086.1.2.217. PMID: 21082926

Recent clinical studies

Etiology

Natural history of chronic idiopathic neutropenia of the adult.
Fattizzo B, Bosi A, Sorrenti M, Murgia D, Pettine L, Bortolotti M, Croci GA, Passamonti F, Barcellini W
Sci Rep 2024 Sep 19;14(1):21891. doi: 10.1038/s41598-024-71719-2. PMID: 39300133Free PMC Article
Discerning clinicopathological features of congenital neutropenia syndromes: an approach to diagnostically challenging differential diagnoses.
Parisi X, Bledsoe JR
J Clin Pathol 2024 Aug 16;77(9):586-604. doi: 10.1136/jcp-2022-208686. PMID: 38589208
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
An update on the diagnosis and treatment of chronic idiopathic neutropenia.
Dale DC, Bolyard AA
Curr Opin Hematol 2017 Jan;24(1):46-53. doi: 10.1097/MOH.0000000000000305. PMID: 27841775Free PMC Article
Primary and secondary autoimmune neutropenia.
Capsoni F, Sarzi-Puttini P, Zanella A
Arthritis Res Ther 2005;7(5):208-14. Epub 2005 Aug 31 doi: 10.1186/ar1803. PMID: 16207350Free PMC Article

Diagnosis

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
Autoimmune hemolytic anemia, autoimmune neutropenia and aplastic anemia in the elderly.
Barcellini W, Fattizzo B, Cortelezzi A
Eur J Intern Med 2018 Dec;58:77-83. doi: 10.1016/j.ejim.2018.05.034. PMID: 30527923
The relationship between idiopathic cytopenias/dysplasias of uncertain significance (ICUS/IDUS) and autoimmunity.
Barcellini W
Expert Rev Hematol 2017 Jul;10(7):649-657. Epub 2017 Jun 15 doi: 10.1080/17474086.2017.1339597. PMID: 28586251
An update on the diagnosis and treatment of chronic idiopathic neutropenia.
Dale DC, Bolyard AA
Curr Opin Hematol 2017 Jan;24(1):46-53. doi: 10.1097/MOH.0000000000000305. PMID: 27841775Free PMC Article
Congenital and acquired neutropenia.
Berliner N, Horwitz M, Loughran TP Jr
Hematology Am Soc Hematol Educ Program 2004:63-79. doi: 10.1182/asheducation-2004.1.63. PMID: 15561677

Therapy

Diagnostic value of cell bound and circulating neutrophil antibody detection in pediatric neutropenia.
Porretti L, Farruggia P, Colombo FS, Cattaneo A, Ghilardi R, Mirra N, Notarangelo LD, Martire B, Trombetta E, Milani S, Vener C, Rebulla P
Pediatr Blood Cancer 2018 Apr;65(4) Epub 2017 Dec 12 doi: 10.1002/pbc.26904. PMID: 29230955
An update on the diagnosis and treatment of chronic idiopathic neutropenia.
Dale DC, Bolyard AA
Curr Opin Hematol 2017 Jan;24(1):46-53. doi: 10.1097/MOH.0000000000000305. PMID: 27841775Free PMC Article
Clinical manifestations and outcomes of pediatric chronic neutropenia.
Wan C, Yu HH, Lu MY, Lee JH, Wang LC, Lin YT, Yang YH, Chiang BL
J Formos Med Assoc 2012 Apr;111(4):220-7. Epub 2012 Mar 16 doi: 10.1016/j.jfma.2010.12.003. PMID: 22526211
Primary and secondary autoimmune neutropenia.
Capsoni F, Sarzi-Puttini P, Zanella A
Arthritis Res Ther 2005;7(5):208-14. Epub 2005 Aug 31 doi: 10.1186/ar1803. PMID: 16207350Free PMC Article
Chronic idiopathic neutropenia. Treatment with corticosteroids and mercaptopurine.
Lipton A
Arch Intern Med 1969 Jun;123(6):694-700. doi: 10.1001/archinte.123.6.694. PMID: 4306101

Prognosis

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
The relationship between idiopathic cytopenias/dysplasias of uncertain significance (ICUS/IDUS) and autoimmunity.
Barcellini W
Expert Rev Hematol 2017 Jul;10(7):649-657. Epub 2017 Jun 15 doi: 10.1080/17474086.2017.1339597. PMID: 28586251
Pathophysiologic mechanisms, clinical features and treatment of idiopathic neutropenia.
Papadaki HA, Pontikoglou C
Expert Rev Hematol 2008 Dec;1(2):217-29. doi: 10.1586/17474086.1.2.217. PMID: 21082926
Primary and secondary autoimmune neutropenia.
Capsoni F, Sarzi-Puttini P, Zanella A
Arthritis Res Ther 2005;7(5):208-14. Epub 2005 Aug 31 doi: 10.1186/ar1803. PMID: 16207350Free PMC Article
Congenital and acquired neutropenia.
Berliner N, Horwitz M, Loughran TP Jr
Hematology Am Soc Hematol Educ Program 2004:63-79. doi: 10.1182/asheducation-2004.1.63. PMID: 15561677

Clinical prediction guides

Natural history of chronic idiopathic neutropenia of the adult.
Fattizzo B, Bosi A, Sorrenti M, Murgia D, Pettine L, Bortolotti M, Croci GA, Passamonti F, Barcellini W
Sci Rep 2024 Sep 19;14(1):21891. doi: 10.1038/s41598-024-71719-2. PMID: 39300133Free PMC Article
Cytotoxic T cells in chronic idiopathic neutropenia express restricted antigen receptors.
Mastrodemou S, Stalika E, Vardi A, Gemenetzi K, Spanoudakis M, Karypidou M, Mavroudi I, Hadzidimitriou A, Stavropoulos-Giokas C, Papadaki HA, Stamatopoulos K
Leuk Lymphoma 2017 Dec;58(12):2926-2933. Epub 2017 May 23 doi: 10.1080/10428194.2017.1324154. PMID: 28535087
An update on the diagnosis and treatment of chronic idiopathic neutropenia.
Dale DC, Bolyard AA
Curr Opin Hematol 2017 Jan;24(1):46-53. doi: 10.1097/MOH.0000000000000305. PMID: 27841775Free PMC Article
Minor populations of paroxysmal nocturnal hemoglobinuria-type cells in patients with chronic idiopathic neutropenia.
Damianaki A, Stagakis E, Mavroudi I, Spanoudakis M, Koutala H, Papadogiannis F, Kanellou P, Pontikoglou C, Papadaki HA
Eur J Haematol 2016 Dec;97(6):538-546. Epub 2016 May 25 doi: 10.1111/ejh.12766. PMID: 27124520
Congenital and acquired neutropenia.
Berliner N, Horwitz M, Loughran TP Jr
Hematology Am Soc Hematol Educ Program 2004:63-79. doi: 10.1182/asheducation-2004.1.63. PMID: 15561677

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