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Nonimmune chronic idiopathic neutropenia of adults

MedGen UID:
375050
•Concept ID:
C1842930
•
Disease or Syndrome
Synonyms: Neutropenia, Nonimmune Chronic Idiopathic, Adult; NI-CINA; Nonimmune Chronic Idiopathic Neutropenia of Adults
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Gene (location): GFI1 (1p22.1)
 
Monarch Initiative: MONDO:0011922
OMIM®: 607847
Orphanet: ORPHA2688

Definition

Nonimmune chronic idiopathic neutropenia of adults (NI-CINA) is a relatively mild form of neutropenia diagnosed in adults but predisposing to leukemia in a subset of patients (Papadaki et al., 2002). [from OMIM]

Clinical features

From HPO
Acute myeloid leukemia
MedGen UID:
9730
•Concept ID:
C0023467
•
Neoplastic Process
A clonal expansion of myeloid blasts in the bone marrow, blood or other tissues. The classification of acute myeloid leukemias (AMLs) encompasses four major categories: 1) AML with recurrent genetic abnormalities; 2) AML with multilineage dysplasia; 3) Therapy-related AML; 4) AML not otherwise specified. The required bone marrow or peripheral blood blast percentage for the diagnosis of AML is 20% (WHO classification)
See: Feature record | Search on this feature
Neutropenia
MedGen UID:
163121
•Concept ID:
C0853697
•
Finding
An abnormally low number of neutrophils in the peripheral blood.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNonimmune chronic idiopathic neutropenia of adults
Follow this link to review classifications for Nonimmune chronic idiopathic neutropenia of adults in Orphanet.

Recent clinical studies

Etiology

Increased serum IgA and decreased IgG3 strongly correlate with increased serum TGF-beta1 levels in patients with nonimmune chronic idiopathic neutropenia of adults.
Papadaki HA, Palmblad J, Kapsimali V, Anagnou NP, Eliopoulos GD
Eur J Haematol 2000 Oct;65(4):237-44. doi: 10.1034/j.1600-0609.2000.065004237.x. PMID: 11073164

Diagnosis

Two patients with nonimmune chronic idiopathic neutropenia of adults developing acute myeloid leukemia with aberrant phenotype and complex karyotype but no mutations in granulocyte colony-stimulating factor receptor.
Papadaki HA, Kosteas T, Gemetzi C, Alexandrakis M, Psyllaki M, Eliopoulos GD
Ann Hematol 2002 Jan;81(1):50-4. Epub 2001 Dec 14 doi: 10.1007/s00277-001-0401-z. PMID: 11807637

Clinical prediction guides

Two patients with nonimmune chronic idiopathic neutropenia of adults developing acute myeloid leukemia with aberrant phenotype and complex karyotype but no mutations in granulocyte colony-stimulating factor receptor.
Papadaki HA, Kosteas T, Gemetzi C, Alexandrakis M, Psyllaki M, Eliopoulos GD
Ann Hematol 2002 Jan;81(1):50-4. Epub 2001 Dec 14 doi: 10.1007/s00277-001-0401-z. PMID: 11807637

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