Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity.

Autor:	Guerra F; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy.; Tettamanti Research Center, University of Milano-Bicocca, University of Milano Bicocca, Monza, Italy., L'Imperio V; Pathology, Department of Medicine and Surgery, ASST Monza, San Gerardo Hospital, University of Milano-Bicocca, Monza, Italy., Bonanomi S; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy., Spinelli M; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy., Coliva TA; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy., Dell'Acqua F; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy., Ferrari GM; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy., Corti P; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy., Balduzzi A; Pediatric Hematology Department, Fondazione MBBM, University of Milano Bicocca, Monza, Italy., Biondi A; Department of Pediatrics, University of Milano-Bicocca, European Reference Network (ERN) PaedCan, EuroBloodNet, MetabERN, Fondazione MBBM/Ospedale San Gerardo, Monza, Italy., Pagni F; Pathology, Department of Medicine and Surgery, ASST Monza, San Gerardo Hospital, University of Milano-Bicocca, Monza, Italy., Saettini F; Tettamanti Research Center, University of Milano-Bicocca, University of Milano Bicocca, Monza, Italy.
Jazyk:	angličtina
Zdroj:	Frontiers in pediatrics [Front Pediatr] 2022 Nov 07; Vol. 10, pp. 1031687. Date of Electronic Publication: 2022 Nov 07 (Print Publication: 2022).
DOI:	10.3389/fped.2022.1031687
Abstrakt:	Myelofibrosis is a rare myeloproliferative disorder. The detailed descriptions of myelofibrosis in children and adolescents is limited to a few case series and case reports describing fewer than 100 patients, thus suggesting the extreme rarity of this condition prior to adulthood. Though pediatric patients rarely present the typical features and outcomes usually observed in older people, pediatric myelofibrosis is not considered an independent entity. Here we aim to describe patients with pediatric myelofibrosis, showing different clinical and pathological features when compared to the World Health Organization 2016 Primary Myelofibrosis classification. We retrospectively collected and analyzed 14 consecutive pediatric myelofibrosis diagnosed in our Pediatric hematology outpatient clinic over a six-year period. According to clinical data and bone marrow biopsy findings, patients were classified into three subgroups: adult-like myelofibrosis, pediatric immune myelofibrosis, idiopathic myelofibrosis. Pediatric Immune Myelofibrosis was the predominant subgroup in our cohort (7/14). Pediatric Immune Myelofibrosis is characterized by peculiar bone marrow features (i.e., T lymphocyte infiltration) and a milder course compared to the other patients Pediatric Immune Myelofibrosis is a novel and distinct pathological entity. We suggest to carefully consider Pediatric Immune Myelofibrosis in case of bone marrow biopsies showing myelofibrosis that do not fulfill WHO criteria. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (© 2022 Guerra, L'Imperio, Bonanomi, Spinelli, Coliva, Dell'acqua, Ferrari, Corti, Balduzzi, Biondi, Pagni and Saettini.)
Databáze:	MEDLINE
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