Are all cases of paediatric essential thrombocythaemia really myeloproliferative neoplasms? Analysis of a large cohort

Sporadic essential thrombocythaemia (ET) is rare in paediatrics, and the diagnostic and clinical approach to paediatric cases cannot be simply copied from experience with adults. Here, we assessed 89 children with a clinical diagnosis of ET and found that 23 patients (258%) had a clonal disease. The JAK2 V617F mutation was identified in 14 children, 1 child had the MPL W515L mutation, and 6 had CALR mutations. The monoclonal X-chromosome inactivation pattern was seen in six patients (two with JAK2 V617F and two with CALR mutations). The other 66 patients (742%) had persistent thrombocytosis with no clonality. There were no clinical or haematological differences between the clonal and non-clonal patients. The relative proportion of ET-specific mutations in the clonal children was much the same as in adults. The higher prevalence of nonclonal cases suggests that some patients may not have myeloproliferative neoplasms, with significant implications for their treatment

Favorable outcome of SARS-CoV-2 infection in pediatric hematology oncology patients during the second and third pandemic waves in Italy: a multicenter analysis from the Infectious Diseases Working Group of the Associazione Italiana di Ematologia e Oncologia Pediatrica (AIEOP)

COVID-19 has a mild clinical course with low mortality rate in general pediatric population, while variable outcomes have been described in children with cancer. Infectious diseases working party of the AIEOP collected data on the clinical characteristics and outcomes of SARS-CoV-2 infections in pediatric oncology/hematology patients from April 2020 to May 2021, including the second and the third waves of the pandemic in Italy. Factors potentially associated with moderate, severe, or critical COVID-19 were analyzed. Of the 153 SARS-Cov2 infections recorded, 100 were asymptomatic and 53 symptomatic. The course of COVID-19 was mild in 41, moderate in 2, severe in 5, and critical in 5 children. A total of 40.5% of patients were hospitalized, ten requiring oxygen support and 5 admitted to the intensive care unit. Antibiotics and steroids were the most used therapies. No patient died due to SARS-CoV-2 infection. Infections occurring early (< 60 days) after the diagnosis of the underlying disease or after SCT were associated to moderate, severe, and critical disease compared to infections occurring late (> 60 days) or during maintenance therapy. In the patients on active chemotherapy, 59% withdrew the treatment for a median of 15 days. SARS-CoV-2 presented a favorable outcome in children with cancer in Italy during the pandemic. Modification of therapy represents a major concern in this population. Our findings suggest considering regular chemotherapy continuation, particularly in patients on maintenance therapy or infected late after the diagnosis

Consensus on COVID‐19 Vaccination in Pediatric Oncohematological Patients, on Behalf of Infectious Working Group of Italian Association of Pediatric Hematology Oncology

Vaccines represent the best tool to prevent the severity course and fatal consequences of the pandemic by the new Coronavirus 2019 infection (SARS‐CoV‐2). Considering the limited data on vaccination of pediatric oncohematological patients, we developed a Consensus document to support the Italian pediatric hematological oncological (AIEOP) centers in a scientifically correct communication with families and patients and to promote vaccination. The topics of the Consensus were: SARS‐CoV‐2 infection and disease (COVID‐19) in the pediatric subjects; COVID‐19 vaccines (type, schedule); who and when to vaccinate; contraindications and risk of serious adverse events; rare adverse events; third dose and vaccination after COVID‐19; and other general prevention measures. Using the Delphi methodology for Consensus, 21 statements and their corresponding rationale were elaborated and discussed with the representatives of 31 centers, followed by voting. A high grade of Consensus was obtained on topics such as the potential risk of severe COVID‐19 outcome in pediatric oncohematological patients, the need for vaccination as a preventative measure, the type, schedule and booster dose of vaccine, the eligibility of the patients for vaccination, and the timing, definition, and management of contraindications and serious adverse events, and other general prevention measures. All 21 of the statements were approved. This consensus document highlights that children and adolescents affected by hematological and oncological diseases are a fragile category. Vaccination plays an important role to prevent COVID‐ 19, to permit the regular administration of chemotherapy or other treatments, to perform control visits and hospital admissions, and to prevent treatment delays

Specie esotiche invasive di rilevanza unionale in Italia: aggiornamenti e integrazioni

La Commissione Europea (CE) ha inserito ad oggi 36 taxa esotici vegetali nella lista delle specie esotiche invasive di rilevanza unionale ai sensi del Regolamento (UE) n. 1143/2014 del Parlamento Europeo e del Consiglio, recante disposizioni volte a prevenire e gestire l’introduzione e la diffusione delle specie esotiche invasive. La lista delle specie di rilevanza unionale viene periodicamente aggiornata e include quelle specie che rappresentano una grave minaccia per la biodiversità, ma anche per la salute dei cittadini e le attività economiche nei territori dell’Unione Europea e che necessitano di una gestione concertata a livello comunitario. La CE vigila sullo stato di ogni taxon grazie anche a periodiche rendicontazioni da parte dei paesi dell'Unione. In vista di tali report, tra il 2020 e il 2021 è stata definita e integrata la distribuzione di queste specie in Italia

La febbre: un sintomo frequente non sempre di facile interpretazione

Viene descritto il caso di un bambino di 7 anni con episodi mensili di iperpiressia e per il riscontro occasionale di neutropenia. In considerazione del quadro clinico ed escluse le cause più frequenti di febbre, ci si è indirizzati verso la sindrome da iper- IgD e la neutropenia ciclica. In assenza del riscontro di una ciclicità della neutropenia e in mancanza della mutazione genetica classica per la sindrome da iper-IgD è stata ipotizzata una neutropenia benigna cronica dell’infanzia

Polyarteritis nodosa of childhood. A review

Systemic vasculitides are uncommon in childhood and their treatment is based mainly on the use of corticosteroids and other immunosuppressive drugs. The combined use of cortisone and cyclophosphamide, in particular, induce a remission rate nearly 90%

A CASE OF INFECTIOUS MONONUCLEOSIS WITH ATYPICAL SEROLOGY

We present a case of infectious mononucleosis (IM) in a 14-year-old boy characterized by typical symptoms associated with an unclear serological pattern. At the beginning, an acute EBV infection was excluded because only anti viral capsid antigen (VCA)-IgG antibodies were found without anti VCAIgM. After searching other aetiologies, including the neoplastic ones, serological tests were repeated and anti VCA-IgM antibodies were eventually found, characteristic for acute IM. Probably this was the case of a primary acute IM with delayed onset of anti VCA-IgM

Le diverse facce dell'infezione da Bartonella henselae

E' presentato il caso di una infezione da B.henselae caratterizzato da febbre persistente.Si sottolinea la necessità di considerare l’infezione da B. henselae tra le diagnosi differenziali in caso di febbri persistenti associate a quadri di coinvolgimento reumatologico: artralgie, miopatie, artriti e tendiniti sono manifestazioni cliniche dell’infezione da Bartonella meno note rispetto alla forma classica di malattia e il loro riconoscimento permette di risparmiare indagini invasive, stressant

Langerhans cell histiocytosis and juvenile xanthogranuloma. Two case reports.

BACKGROUND: Histiocytoses represent a large, puzzling group of diseases which may involve the skin and other organs. At present, juvenile xanthogranuloma is the disorder most often confused with Langerhans cell histiocytosis. A complex overlap exists between juvenile xanthogranuloma and Langerhans cell histiocytosis, with lesions showing clinical and/or pathological features of both disorders. OBSERVATIONS: We report 2 patients affected by Langerhans cell histiocytosis who, during chemotherapy, presented cutaneous lesions with clinical and histological features of juvenile xanthogranuloma. During the therapy, in both cases, histological examination of new biopsies revealed the presence of Touton giant cells in the dermis with a few histiocytic cells; immunohistochemical staining was negative for CD1a, and no Birbeck granules were seen by ultrastructural examination. RESULTS AND CONCLUSION: A possible explanation for the link between Langerhans cell histiocytosis and juvenile xanthogranuloma regards the lineage development and the relationships of histiocytes. We suggest that chemotherapy can modify the production of cytokines by influencing the conversion or 'maturation' of pathological cells into macrophages or xanthomatous cells and fusing them to form multinucleated giant Touton cells. In our opinion, the modification of the cutaneous lesions during chemotherapy in Langerhans cell histiocytosis patients, as observed in our cases, could be a favorable prognostic factor