Pediatric myelofibrosis: WHO 2024 update on myeloproliferative neoplasms calling?
Adolescent
Adult
Amino Acid Substitution
Cell Transformation, Neoplastic
/ genetics
Child
Child, Preschool
Female
Humans
Infant
Janus Kinase 2
/ genetics
Male
Megakaryocytes
/ metabolism
Mutation, Missense
Neoplasm Proteins
/ genetics
Osteosclerosis
/ genetics
Primary Myelofibrosis
/ genetics
Retrospective Studies
Thrombopoiesis
anemia
dysmegakaryopoiesis
idiopathic myelofibrosis
pediatric myelofibrosis
Journal
Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624
Informations de publication
Date de publication:
05 2020
05 2020
Historique:
received:
10
09
2019
accepted:
08
02
2020
pubmed:
7
3
2020
medline:
14
7
2020
entrez:
6
3
2020
Statut:
ppublish
Résumé
Pediatric myelofibrosis is a rare entity with the largest reported series of 19 cases. We describe here the clinicopathological spectrum and outcomes of 15 cases of pediatric myelofibrosis. Case files of myelofibrosis of patients less than 18 years were retrieved from January 2016 to January 2019, and patients with idiopathic myelofibrosis after exhaustive work-up were studied. Their clinicopathological profiles were studied and then followed up for resolution and malignant transformation. Of the 15 cases of idiopathic myelofibrosis, transfusion-dependent anemia (14/15) was most common presentation. Only one patient showed leukoerythroblastosis with dacryocytes. Myeloid hyperplasia was seen in 13 of 15 patients and megakaryocytic hyperplasia in 10 patients. Dysmegakaryopoiesis was seen in 8 of 15 patients, and only three had small loose megakaryocytic clustering. None showed hyperchromatic megakaryocytes, intrasinusoidal hematopoiesis, or osteosclerosis. One patient with trisomy 8 tested positive for JAK2V617F. Bone marrow biopsy was hypercellular in 13, and 8 had world health organization (WHO) MF-3 fibrosis. None of the patients developed malignancy, one had spontaneous resolution, and one patient required allogenic stem cell transplant. Pediatric myelofibrosis is a distinct entity from primary myelofibrosis in adults and merits mention in the WHO manual as a distinct entity.
Substances chimiques
Neoplasm Proteins
0
JAK2 protein, human
EC 2.7.10.2
Janus Kinase 2
EC 2.7.10.2
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e28232Informations de copyright
© 2020 Wiley Periodicals, Inc.
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