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

Alexandar Tzankov, Markus Kremer, Roos Leguit, Attilio Orazi, Jon van der Walt, Umberto Gianelli, Konnie M. Hebeda

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)2117-2128
Number of pages12
JournalAnnals of Hematology
Volume97
Issue number11
DOIs
Publication statusPublished - Nov 1 2018

Fingerprint

Bone Marrow Neoplasms
Erdheim-Chester Disease
Bone Marrow
Education
Neoplasms
Langerhans Cell Histiocytosis
Histiocytes
MAP Kinase Signaling System
Mutation
Juvenile Xanthogranuloma
Sinus Histiocytosis
Histiocytic Sarcoma
Hemophagocytic Lymphohistiocytosis
ras Genes
Switzerland
Hyperplasia
Catalytic Domain
Vaccination
Differential Diagnosis
Observation

Keywords

  • Bone marrow biopsy
  • EAHP workshop
  • Erdheim-Chester disease
  • Histiocytic sarcoma
  • Mutation
  • Myeloid neoplasm

ASJC Scopus subject areas

  • Hematology

Cite this

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. / Tzankov, Alexandar; Kremer, Markus; Leguit, Roos; Orazi, Attilio; van der Walt, Jon; Gianelli, Umberto; Hebeda, Konnie M.

In: Annals of Hematology, Vol. 97, No. 11, 01.11.2018, p. 2117-2128.

Research output: Contribution to journalArticle

@article{dee2fde5a9c346a18c0e2992e314196e,
title = "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",
abstract = "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.",
keywords = "Bone marrow biopsy, EAHP workshop, Erdheim-Chester disease, Histiocytic sarcoma, Mutation, Myeloid neoplasm",
author = "Alexandar Tzankov and Markus Kremer and Roos Leguit and Attilio Orazi and {van der Walt}, Jon and Umberto Gianelli and Hebeda, {Konnie M.}",
year = "2018",
month = "11",
day = "1",
doi = "10.1007/s00277-018-3436-0",
language = "English",
volume = "97",
pages = "2117--2128",
journal = "Annals of Hematology",
issn = "0939-5555",
publisher = "Springer Verlag",
number = "11",

}

TY - JOUR

T1 - Histiocytic cell neoplasms involving the bone marrow

T2 - 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

AU - Tzankov, Alexandar

AU - Kremer, Markus

AU - Leguit, Roos

AU - Orazi, Attilio

AU - van der Walt, Jon

AU - Gianelli, Umberto

AU - Hebeda, Konnie M.

PY - 2018/11/1

Y1 - 2018/11/1

N2 - 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.

AB - 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.

KW - Bone marrow biopsy

KW - EAHP workshop

KW - Erdheim-Chester disease

KW - Histiocytic sarcoma

KW - Mutation

KW - Myeloid neoplasm

UR - http://www.scopus.com/inward/record.url?scp=85051740093&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85051740093&partnerID=8YFLogxK

U2 - 10.1007/s00277-018-3436-0

DO - 10.1007/s00277-018-3436-0

M3 - Article

C2 - 30084011

AN - SCOPUS:85051740093

VL - 97

SP - 2117

EP - 2128

JO - Annals of Hematology

JF - Annals of Hematology

SN - 0939-5555

IS - 11

ER -