Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry

Farruggia, Piero; Fioredda, Francesca; Puccio, Giuseppe; Onofrillo, Daniela; Russo, Giovanna; Barone, Angelica; Bonanomi, Sonia; Boscarol, Gianluca; Finocchi, Andrea; Ghilardi, Roberta; Giordano, Paola; Ladogana, Saverio; Lassandro, Giuseppe; Luti, Laura; Lanza, Tiziana; Mandaglio, Rosalba; Marra, N; Martire, Baldassare; Mastrodicasa, Elena; Motta, Milena; Notarangelo, Lucia Dora; Pillon, Marta; Porretti, Laura; Serafinelli, Jessica; Trizzino, Angela; Tucci, Fábio C.; Veltroni, Marinella; Verzegnassi, Federico; Ramenghi, Ugo; Dufour, Carlo

doi:10.1002/ajh.25353

Cited by 23 publications

(48 citation statements)

References 47 publications

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“…With regard to leuko-lymphopenia, lymphocytic typing showed a marked reduction in CD8+ T-and CD16+ lymphocytes (NK cells). In the absence of ongoing infections, a suggestive medical history of primitive immunodeficiency, leukopenia was considered a benign idiopathic form (23).…”

Section: Discussionmentioning

confidence: 99%

Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review

et al. 2020

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Section: Discussionmentioning

confidence: 99%

Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review

et al. 2020

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“…The most prevalent PID entity was humoral defects and SIgAD (66.6%) was found as the most frequent disease, followed by T-cell disorders (14.2%) [57]. More recently the number of Italian patients in the ESID registry has reached 1275 [2,58], but this has not been reflected in other publications from this country with no general information regarding the genetic diagnosis of all PID patients [59,60].…”

Section: Europementioning

confidence: 99%

Global systematic review of primary immunodeficiency registries

Abolhassani

Azizi

Sharifi

et al. 2020

Expert Review of Clinical Immunology

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Introduction: During the last 4 decades, registration of patients with primary immunodeficiencies (PID) has played an essential role in different aspects of these diseases worldwide including epidemiological indexes, policymaking, quality controls of care/life, facilitation of genetic studies and clinical trials as well as improving our understanding about the natural history of the disease and the immune system function. However, due to the limitation of sustainable resources supporting these registries, inconsistency in diagnostic criteria and lack of molecular diagnosis as well as difficulties in the documentation and designing any universal platform, the global perspective of these diseases remains unclear. Areas covered: Published and unpublished studies from January 1981 to June 2020 were systematically reviewed on PubMed, Web of Science and Scopus. Additionally, the reference list of all studies was hand-searched for additional studies. This effort identified a total of 104614 registered patients and suggests identification of at least 10590 additional PID patients, mainly from countries located in Asia and Africa. Molecular defects in genes known to cause PID were identified and reported in 13852 (13.2% of all registered) patients. Expert opinion: Although these data suggest some progress in the identification and documentation of PID patients worldwide, achieving the basic requirement for the global PID burden estimation and registration of undiagnosed patients will require more reinforcement of the progress, involving both improved diagnostic facilities and neonatal screening.

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“…Anti‐neutrophil antibodies are sometimes detectable, but the results of this testing have low sensitivity and specificity in diagnosing AIN 50 . Moreover, in a large Italian cohort study of AIN in children, the presence or absence of anti‐neutrophil antibodies was not associated with risk or frequency of infections, age at recovery, or overall prognosis 51 . Almost all children with AIN normalize their absolute neutrophil counts within a median of 20 months (range, 6–54 months), with no risk of recurrence 48,49,51 …”

Section: Discussionmentioning

confidence: 99%

The ASH‐ASPHO Choosing Wisely Campaign: 5 hematologic tests and treatments to question

O’Brien

Badawy

Rotz

et al. 2021

Pediatric Blood & Cancer

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Choosing Wisely is a medical stewardship and quality‐improvement initiative led by the American Board of Internal Medicine Foundation in collaboration with leading medical societies in the United States. The American Society of Hematology (ASH) has been an active participant in the Choosing Wisely project. In 2019, ASH and the American Society of Pediatric Hematology/Oncology (ASPHO) formed a joint task force to solicit, evaluate, and select items for a pediatric‐focused Choosing Wisely list. By using an iterative process and an evidence‐based method, the ASH‐ASPHO Task Force identified 5 hematologic tests and treatments that health care providers and patients should question because they are not supported by evidence, and/or they involve risks of medical and financial costs with low likelihood of benefit. The ASH‐ASPHO Choosing Wisely recommendations are as follows: (1) avoid routine preoperative hemostatic testing in an otherwise healthy child with no previous personal or family history of bleeding, (2) avoid platelet transfusion in asymptomatic children with a platelet count 10 × 103/μL unless an invasive procedure is planned, (3) avoid thrombophilia testing in children with venous access‐associated thrombosis and no positive family history, (4) avoid packed red blood cells transfusion for asymptomatic children with iron deficiency anemia and no active bleeding, and (5) avoid routine administration of granulocyte colony‐stimulating factor for prophylaxis of children with asymptomatic autoimmune neutropenia and no history of recurrent or severe infections. We recommend that health care providers carefully consider the anticipated risks and benefits of these identified tests and treatments before performing them.

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Idiopathic neutropenia of infancy: Data from the Italian Neutropenia Registry

Cited by 23 publications

References 47 publications

Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review

Mesothelioma in Familial Mediterranean Fever With Colchicine Intolerance: A Case Report and Literature Review

Global systematic review of primary immunodeficiency registries

The ASH‐ASPHO Choosing Wisely Campaign: 5 hematologic tests and treatments to question

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