Design
This retrospective study included 47 patients since 2000 to 2019
suffering from immune cytopenias at Pediatric Hematology and Immunology
Units of the University-Hospital Sant’Orsola – Bologna.
Inclusion criteria were:
Diagnosis of at least one hematological disorder among
persistent/chronic ITP, AIHA, AIN;
Age 0–18 years at initial presentation of hematological disease
and/or of immune-dysregulation.
Persistent and chronic ITP were defined as a platelet count of
<100x10⁹/L with no known cause lasting respectively for more
than 3 and 12 months.
AIHA was defined by anemia (hemoglobin < -2 standard
deviations) and a positive direct antiglobulin test associated to signs
of hemolysis.
AIN was defined by a neutrophil count <1.5×10⁹/L after the
first year of life and the detection of anti-neutrophils antibodies
using indirect flow cytometry.
ES was defined by the synchronous/metachronous presence of at least two
immune cytopenias4.
The diagnosis of IEI met the 2019 revised criteria established by the
European Society of Immunodeficiencies (ESID)11.
We performed an extended clinical-laboratory characterization of the
cohort reported in Supplementary table.