Novel findings of splenic extramedullary hematopoiesis during primary myelofibrosis, post-essential thrombocythemia, and post-polycythemia vera myelofibrosis.
Adult
Aged
Aged, 80 and over
Bone Marrow
/ pathology
Disease Progression
Female
France
/ epidemiology
Hematopoiesis
Hematopoiesis, Extramedullary
/ genetics
Humans
Male
Middle Aged
Myeloproliferative Disorders
/ epidemiology
Polycythemia Vera
/ pathology
Primary Myelofibrosis
/ epidemiology
Spleen
/ pathology
Thrombocythemia, Essential
/ pathology
Immunohistochemistry
Molecular biology
Myelofibrosis
Spleen pathology
Splenectomy
Journal
Virchows Archiv : an international journal of pathology
ISSN: 1432-2307
Titre abrégé: Virchows Arch
Pays: Germany
ID NLM: 9423843
Informations de publication
Date de publication:
Oct 2021
Oct 2021
Historique:
received:
26
01
2021
accepted:
22
04
2021
revised:
17
04
2021
pubmed:
3
5
2021
medline:
29
10
2021
entrez:
2
5
2021
Statut:
ppublish
Résumé
BCR-ABL-fusion-negative myeloproliferative neoplasms (MPNs) with myelofibrosis (MF) include primary MF, post-polycythemia vera MF and post-essential thrombocythemia MF. Clonal extramedullary hematopoiesis (EMH) can occur during MPN pathogenesis. Although histopathological bone-marrow (BM) features during clonal EMH have been investigated, those of the spleen have been poorly described. We analyzed splenectomy samples from 28 patients with MF and BM samples from 20 of them. Slides were stained with hematoxylin and eosin, reticulin, and trichrome, with immunohistochemical labeling of glycophorin A, myeloperoxidase, CD61, CD34, and CD117. We also subjected splenectomy and BM samples from six patients and spleen samples from seven patients to next-generation sequencing (NGS). Megakaryocyte-rich spleen nodules (MRSNs), seen in seven of the 28 patients, were significantly associated with megakaryocyte proliferation in the spleen (p = 0.04). We devised a grading system for spleen fibrosis (SF) and found that SF was increased in 20 of 28 patients. Notably, patients with SF were more likely to have MRSNs, suggesting that megakaryocytes might participate in SF, as previously described in BM. Comparisons of spleen and BM NGS findings of six patients' specimens revealed identical mutational status in the two organs for half of the patients. We observed additional mutations in the spleen of two patients. However, the meaning of this finding remains unknown since there was a long interval between BM and spleen samplings (68 and 82 months, respectively).
Identifiants
pubmed: 33934231
doi: 10.1007/s00428-021-03110-9
pii: 10.1007/s00428-021-03110-9
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
755-764Informations de copyright
© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
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