Articles

Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic syndrome (VEXAS syndrome) with multiple thromboembolism events: A Belgian case report

BJH - volume 14, issue 8, december 2023

M. Pirotte MD, A. De Voeght MD, G. Vertenoeil MD, PhD, M. Vasbien MD, H. Paridaens MD, J. Somja MD, PhD, P. Collins MD, F. Lambert MD

SUMMARY

VEXAS syndrome, an acquired autoinflammatory syndrome, is classified within the complex of autoinflammatory diseases (AID), arising from aberrant changes in the innate immune system due to acquisition of somatic mutation of the UBA1 gene in bone marrow cells. This recently identified syndrome is characterised by systemic inflammation, chondritis, neutrophilic dermatosis, pulmonary involvement, thrombosis, macrocytosis and cytopenia in mature men. We present a case study of a 67-years-old man exhibiting multiple thrombotic manifestations without any known underlying aetiology or haemopathy. This report emphasises the crucial collaboration between clinicians, cytologists and geneticists highlighting the pivotal role of UBA1 mutation screening in the diagnostic process to confirm the final diagnosis.

(BELG J HEMATOL 2023;14(8):336–42)

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Management of cutaneous T & B cell lymphomas: a comprehensive review

BJH - volume 9, issue 3, june 2018

M. de Vicq de Cumptich MD, C. Springael MD, PhD, J. Somja MD, PhD, C. Bonnet MD, PhD, P. Heimann MD, PhD, U. Sass MD, A. Janssens MD, PhD, D. Bron MD, PhD

SUMMARY

Primary cutaneous lymphomas are a heterogeneous group of diseases with indolent or aggressive behaviour, skin-limited or systemic extension, from T or B cell origin. The optimal management requires the multi-disciplinary approach with dermatologists, hemato-oncologists, pathologists and molecular biologists. The objective of this review is to harmonise the work-up and the treatment of these different entities of cutaneous T or B cell lymphoma in Belgium, according to the availability of the drugs and specialised treatment such as extracorporeal photopherisis or total skin electron beam therapy.

(BELG J HEMATOL 2018;9(3):86–100)

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PP74 Limited impact of imatinib in a murine model of sclerodermatous chronic graft-versus-host disease

BJH - volume 8, issue Abstract Book BHS, february 2017

G. Fransolet , L. Belle , J. Somja MD, PhD, M. Binsfeld , P. Delvenne , P. Drion , M. Hannon , Y. Beguin MD, PhD, G. Ehx , prof. F. Baron

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P22 Case report: Richter syndrome with plasmablastic lymphoma

BJH - volume 8, issue Abstract Book BHS, february 2017

A. Ladang , M. Simul , J. Somja MD, PhD, J. Foguenne , A. Gothot MD, PhD, A. Keutgens , F. Tassin

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PP3.1 Azacytidine prevents experimental sclerodermic chronic graft-versus-host disease

BJH - volume 7, issue Abstract Book BHS, january 2016

G. Fransolet , G. Ehx , J. Somja MD, PhD, L. Delens , M. Hannon , S. Dubois , P. Drion , J. Caers MD, PhD, S. Humblet-Baron , L. Belle , P. Delvenne , Y. Beguin MD, PhD, G. Conteduca , prof. F. Baron

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P3.17 Azacytidine prevents experimental sclerodermic chronic graft-versus-host disease

BJH - volume 6, issue Abstract Book BHS, january 2015

G. Fransolet , G. Ehx , J. Somja MD, PhD, L. Belle , P-V. Drion , S. Humblet-Baron , Y. Beguin MD, PhD, B. Baron

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P4.10 Imatinib improves survival of chronic Graft-versus-Host disease by inhibiting TGF-beta and PDGF-r pathways in mice

BJH - volume 5, issue Abstract Book BHS, january 2014

G. Fransolet , L. Belle , J. Somja MD, PhD, S. Humblet-Baron , S. Dubois , P. Delvenne , Y. Beguin MD, PhD, prof. F. Baron

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