Hodgkin en de classificatie van maligne lymfomen : Hoe de britse arts ook later ontdekte lymfomen aan zich wist te binden

Thomas Hodgkin beschreef als eerste een maligne lymfoom, dat later zijn naam zou krijgen. Daarna werden andere lymfoïde maligniteiten herkend die niet op Hodgkins ziekte leken. Deze werden genoemd naar de op dat moment geldende morfologische nomenclatuur van de betrokken cellen. Later werd de naamgeving mede bepaald door immunologische kenmerken. Maar nog steeds wordt de groep van lymfomen die ontdekt zijn na Hodgkins beschrijving aangeduid als zijnde ‘geen ziekte van Hodgkin’: non-Hodgkin-lymfoom. Wij vinden het ongewenst dat de man wiens lymfoom zo algemeen bekend is, zelf zo onbekend is. In dit artikel geven wij daarom een historisch overzicht van Thomas Hodgkin, ‘zijn’ lymfoom en de andere maligne lymfomen

Challenges in Diagnosing Myelodysplastic Syndromes in the Era of Genetic Testing: Proceedings of the 13th Workshop of the European Bone Marrow Working Group

Contains fulltext : 201060.pdf (publisher's version ) (Open Access

Histiocytic cell neoplasms involving the bone marrow : summary of the workshop cases submitted to the 18th Meeting of the European Association for Haematopathology (EAHP) organized by the European Bone Marrow Working Group, Basel 2016

The bone marrow is a preferential site for both reactive and neoplastic histiocytic proliferations. The differential diagnosis ranges from reactive histiocyte hyperplasia in systemic infections, vaccinations, storage diseases, post myeloablative therapy, due to increased cell turnover, and in hemophagocytic lymphohistiocytosis, through extranodal Rosai-Dorfman disease to neoplasms derived from histiocytes, including histiocytic sarcomas (HS), Langerhans cell histiocytoses (LCH), Erdheim-Chester disease (ECD), and disseminated juvenile xanthogranuloma (JXG). One of the most important recent developments in understanding the biology of histiocytic neoplasms and in contributing to diagnosis was the detection of recurrent mutations of genes of the Ras/Raf/MEK/ERK signaling pathway, in particular the BRAFV600E mutation, in LCH and ECD. Here, we summarize clinical and pathological findings of 17 histiocytic neoplasms that were presented during the bone marrow symposium and workshop of the 18th European Association for Haematopathology (EAHP) meeting held in Basel, Switzerland, in 2016. A substantial proportion of these histiocytic neoplasms was combined with clonally related lymphoid (n = 2) or myeloid diseases (n = 5, all ECD). Based on the latter observation, we suggest excluding co-existent myeloid neoplasms at initial staging of elderly ECD patients. The recurrent nature of Ras/Raf/MEK/ERK signaling pathway mutations in histiocytic neoplasms was confirmed in 6 of the 17 workshop cases, illustrating their diagnostic significance and suggesting apotential target for tailored treatments