Pachydermoperiostosis Associated with Myelofibrosis: A Rare Case Report

Secchin, Pedro; Fernandes, Nurimar Conceição; Quintella, Danielle Carvalho; Silva, Juliano A R; Medrado, Juliana; Magalhães, Taíssa Canedo de

doi:10.4103/ijd.ijd_360_18

Cited by 2 publications

(2 citation statements)

References 11 publications

(13 reference statements)

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“…In primary hypertrophic osteoarthropathy (pachydermoperiostosis), gene-mutations of the prostaglandin degrading enzyme, 15-hydroxyprostaglandin dehydrogenase (HPGD) and the PG transporter ( SLCO2A1 ) lead to impairment of PGE2 degradation. The resultant increase of PGE 2 levels lead to fibrosis of the marrow [ 37 , 38 ].…”

Section: Introductionmentioning

confidence: 99%

“…T-helper cells having a Th2 immunophenotype tend to promote fibrosis in the tissues where they reside. These cells are characterised by a (CD3 + , CD4 + , CD119 + , CD193 + , Foxo + , CD198 + , CD365 + and IL33α +) immunophenotype and are capable of secreting cytokines (IL-3,4,5,6,10,13,25, and 31) that cause fibrosis [ 32 , 38 , 44 ].…”

Section: Introductionmentioning

confidence: 99%

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Fibrosis and bone marrow: understanding causation and pathobiology

Ghosh,

Shome,

Kulkarni

et al. 2023

J Transl Med

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Bone marrow fibrosis represents an important structural change in the marrow that interferes with some of its normal functions. The aetiopathogenesis of fibrosis is not well established except in its primary form. The present review consolidates current understanding of marrow fibrosis. We searched PubMed without time restriction using key words: bone marrow and fibrosis as the main stem against the terms: growth factors, cytokines and chemokines, morphology, megakaryocytes and platelets, myeloproliferative disorders, myelodysplastic syndrome, collagen biosynthesis, mesenchymal stem cells, vitamins and minerals and hormones, and mechanism of tissue fibrosis. Tissue marrow fibrosis-related papers were short listed and analysed for the review. It emerged that bone marrow fibrosis is the outcome of complex interactions between growth factors, cytokines, chemokines and hormones together with their facilitators and inhibitors. Fibrogenesis is initiated by mobilisation of special immunophenotypic subsets of mesenchymal stem cells in the marrow that transform into fibroblasts. Fibrogenic stimuli may arise from neoplastic haemopoietic or non-hematopoietic cells, as well as immune cells involved in infections and inflammatory conditions. Autoimmunity is involved in a small subset of patients with marrow fibrosis. Megakaryocytes and platelets are either directly involved or are important intermediaries in stimulating mesenchymal stem cells. MMPs, TIMPs, TGF-β, PDGRF, and basic FGF and CRCXL4 chemokines are involved in these processes. Genetic and epigenetic changes underlie many of these conditions.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Fibrosis and bone marrow: understanding causation and pathobiology

Ghosh,

Shome,

Kulkarni

et al. 2023

J Transl Med

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Comprehensive Surgical Strategies for the Management of Pachydermoperiostosis

Luo

Zhao

2018

Facial plast Surg

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The cosmetic and functional deformities induced by thickened folds and furrows are the main complaints of patients in pachydermoperiostosis (PDP). However, traditional drug therapy only has a limited effect for its unclear genetic pathogenesis. Surgical treatment has now become one of the optimal strategies mainly aiming for its cosmetic improvement. Here we describe a comprehensive management of PDP approached with surgical procedure thereby showing markedly improved cosmetic outcome. Experientially, the surgical treatment of PDP is effective and may be preferentially recommended for PDP patients especially of severe degree.

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Pachydermoperiostosis Associated with Myelofibrosis: A Rare Case Report

Cited by 2 publications

References 11 publications

Fibrosis and bone marrow: understanding causation and pathobiology

Fibrosis and bone marrow: understanding causation and pathobiology

Comprehensive Surgical Strategies for the Management of Pachydermoperiostosis

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