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J Diebold - One of the best experts on this subject based on the ideXlab platform.

  • sea blue Histiocyte syndrome in bone marrow secondary to total parenteral nutrition
    Leukemia & Lymphoma, 1998
    Co-Authors: C Bigorgne, Le A Tourneau, B Messing, B Rio, T Molina, J Audouin, K Vahedi, J Diebold
    Abstract:

    Clinical and hematological abnormalities can occur in patients receiving intravenous fat emulsions as part of a long-term parenteral nutrition; they consist of hepatosplenomegaly and peripheral blood cytopenia(s). These abnormalities lead to bone marrow examination which revealed numerous macrophages laden with blue staining pigment granules and separate lipid vacuoles, presenting the typical histochemical characteristics of sea-blue Histiocytes. Thus, long-term parenteral nutrition including fat-emulsion sources may represent a further condition in addition to the wide variety of disorders which can be associated with sea-blue histiocytosis. Moreover, in view of its clinical and morphological presentation, this storage pathological state could be compared with the so-called sea-blue Histiocyte syndrome described by Silverstein and colleagues.

  • sea blue Histiocyte syndrome in bone marrow secondary to total parenteral nutrition including fat emulsion sources a clinicopathologic study of seven cases
    British Journal of Haematology, 1996
    Co-Authors: C Bigorgne, Le A Tourneau, B Messing, B Rio, V Giraud, T Molina, J Audouin, J Diebold
    Abstract:

    Bone marrow examination revealed a lipid-laden histiocytosis in seven patients undergoing long-term total parenteral nutrition necessitated by extensive short-bowel surgical resection. Clinical abnormalities occurred during this treatment which required bone marrow examination. These included hepatosplenomegaly and peripheral blood cytopenia; the median time to the detection of these abnormalities was 64 months. The most striking change within the bone marrow was the presence of many pigment-laden Histiocytes which had the typical morphology of sea-blue Histiocytes seen in the so-called idiopathic sea-blue Histiocyte syndrome. The occurrence of sea-blue histiocytosis in the bone marrow in association with long-term parenteral nutrition for short-bowel syndrome has not, to our knowledge, been reported previously and should now be considered in the differential diagnosis of bone marrow sea-blue histiocytosis.

Thomas Tousseyn - One of the best experts on this subject based on the ideXlab platform.

  • junb dusp2 sgk1 socs1 and crebbp are frequently mutated in t cell Histiocyte rich large b cell lymphoma
    Haematologica, 2019
    Co-Authors: Bianca Schuhmacher, Thomas Tousseyn, Maurilio Ponzoni, Randy D Gascoyne, Julia Bein, Tobias Rausch, Vladimir Benes, Martine Vornanen, Lorenz Thurner, Christian Steidl
    Abstract:

    T-cell/Histiocyte-rich large B-cell lymphoma is a rare aggressive lymphoma showing histopathological overlap with nodular lymphocyte-predominant Hodgkin lymphoma. Despite differences in tumor microenvironment and clinical behavior, the tumor cells of both entities show remarkable similarities, suggesting that both lymphomas might represent a spectrum of the same disease. To address this issue, we investigated whether these entities share mutations. Ultra-deep targeted resequencing of six typical and 11 histopathological variants of nodular lymphocyte-predominant Hodgkin lymphoma, and nine cases of T-cell/Histiocyte-rich large B-cell lymphoma revealed that genes recurrently mutated in nodular lymphocyte-predominant Hodgkin lymphoma are affected by mutations at similar frequencies in T-cell/Histiocyte-rich large B-cell lymphoma. The most recurrently mutated genes were JUNB, DUSP2, SGK1, SOCS1 and CREBBP, which harbored mutations more frequently in T-cell/Histiocyte-rich large B-cell lymphoma and the histopathological variants of nodular lymphocyte-predominant Hodgkin lymphoma than in its typical form. Mutations in JUNB, DUSP2, SGK1 and SOCS1 were highly enriched for somatic hypermutation hotspot sites, suggesting an important role of aberrant somatic hypermutation in the generation of these somatic mutations and thus in the pathogenesis of both lymphoma entities. Mutations in JUNB are generally rarely observed in malignant lymphomas and thus are relatively specific for nodular lymphocyte-predominant Hodgkin lymphoma and T-cell/Histiocyte-rich large B-cell lymphoma at such high frequencies (5/17 and 5/9 cases with JUNB mutations, respectively). Taken together, the findings of the present study further support a close relationship between T-cell/Histiocyte-rich large B-cell lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma by showing that they share highly recurrent genetic lesions.

  • Endpoints of a Spectrum of One Disease?
    2016
    Co-Authors: Rich Large Cell B Lymphoma, Thomas Tousseyn, Sylvia Hartmann, Maurilio Ponzoni, Fabio Facchetti, Christina Jakobus, Benjamin Rengstl, Sebastian Newrzela, Xavier Sagaert, Chris De Wolf-peeters
    Abstract:

    In contrast to the commonly indolent clinical behavior of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL), T cell/Histiocyte rich large B cell lymphoma (THRLBCL) is frequently diagnosed in advanced clinical stages and has a poor prognosis. Besides the different clinical presentations of these lymphoma entities, there are variants of NLPHL with considerable histopathologic overlap compared to THRLBCL. Especially THRLBCL-like NLPHL, a diffuse form of NLPHL, often presents a histopathologic pattern similar to THRLBCL, suggesting a close relationship between both lymphoma entities. To corroborate this hypothesis, we performed gene expression profiling of microdissected tumor cells of NLPHL, THRLBCL-like NLPHL and THRLBCL. In unsupervised analyses, the lymphomas did not cluster according to their entity. Moreover, even in supervised analyses, very few consistently differentially expressed transcripts were found, and for these genes the extent of differential expression was only moderate. Hence, there are no clear and consistent differences in the gene expression of the tumor cells of NLPHL, THRLBCL-like NLPHL and THRLBCL. Based on the gene expression studies, we identified BAT3/ BAG6, HIGD1A, and FAT10/UBD as immunohistochemical markers expressed in the tumor cells of all three lymphomas. Characterization of the tumor microenvironment for infiltrating T cells and Histiocytes revealed significant differences in th

  • macrophages in t cell Histiocyte rich large b cell lymphoma strongly express metal binding proteins and show a bi activated phenotype
    International Journal of Cancer, 2013
    Co-Authors: Sylvia Hartmann, Thomas Tousseyn, Claudia Doring, Patricia Fluchter, Holger Hackstein, An Herreman, Maurilio Ponzoni, Chris De Wolfpeeters, Fabio Facchetti, Randy D Gascoyne
    Abstract:

    Abundant macrophage infiltration in tumors often correlates with a poor prognosis. T cell/Histiocyte rich large B cell lymphoma (THRLBCL) is a distinct aggressive B cell lymphoma entity showing a high macrophage content. To further elucidate the role of tumor-associated macrophages in THRLBCL, we performed gene expression profiling of microdissected Histiocyte subsets of THRLBCL, nodular lymphocyte predominant Hodgkin lymphoma (NLPHL), Piringer lymphadenitis, sarcoidosis, nonspecific lymphadenitis and monocytes from peripheral blood. In a supervised principal component analysis, Histiocytes from THRLBCL were most closely related to epithelioid cells from NLPHL, with both types of cells expressing genes related to proinflammatory and regulatory macrophage activity. Moreover, Histiocytes from THRLBCL strongly expressed metal-binding proteins like MT2A, by which Histiocytes of THRLBCL can be distinguished from the other Histiocyte subsets investigated. Interestingly, the validation at the protein level showed a strong expression of TXN, CXCL9, MT2A and SOD2 not only in macrophages of THRLBCL but also in the tumor cells of NLPHL and classical Hodgkin lymphoma (cHL). Overall, the present findings indicate that macrophages in the microenvironment of THRLBCL have acquired a distinct gene expression pattern that is characterized by a mixed M1/M2 phenotype and a strong expression of several metal binding proteins. The microenvironments in NLPHL and THRLBCL appear to have a similar influence on the macrophage phenotype. The high expression of metal binding proteins in Histiocytes of THRLBCL may be diagnostically useful, but a potential pathophysiological role remains to be identified.

  • nodular lymphocyte predominant hodgkin lymphoma and t cell Histiocyte rich large b cell lymphoma endpoints of a spectrum of one disease
    PLOS ONE, 2013
    Co-Authors: Sylvia Hartmann, Thomas Tousseyn, Claudia Doring, Maurilio Ponzoni, Fabio Facchetti, Christina Jakobus, Benjamin Rengstl, Sebastian Newrzela, Xavier Sagaert, Chris De Wolfpeeters
    Abstract:

    In contrast to the commonly indolent clinical behavior of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL), T cell/Histiocyte rich large B cell lymphoma (THRLBCL) is frequently diagnosed in advanced clinical stages and has a poor prognosis. Besides the different clinical presentations of these lymphoma entities, there are variants of NLPHL with considerable histopathologic overlap compared to THRLBCL. Especially THRLBCL-like NLPHL, a diffuse form of NLPHL, often presents a histopathologic pattern similar to THRLBCL, suggesting a close relationship between both lymphoma entities. To corroborate this hypothesis, we performed gene expression profiling of microdissected tumor cells of NLPHL, THRLBCL-like NLPHL and THRLBCL. In unsupervised analyses, the lymphomas did not cluster according to their entity. Moreover, even in supervised analyses, very few consistently differentially expressed transcripts were found, and for these genes the extent of differential expression was only moderate. Hence, there are no clear and consistent differences in the gene expression of the tumor cells of NLPHL, THRLBCL-like NLPHL and THRLBCL. Based on the gene expression studies, we identified BAT3/BAG6, HIGD1A, and FAT10/UBD as immunohistochemical markers expressed in the tumor cells of all three lymphomas. Characterization of the tumor microenvironment for infiltrating T cells and Histiocytes revealed significant differences in the cellular composition between typical NLPHL and THRLBCL cases. However, THRLBCL-like NLPHL presented a histopathologic pattern more related to THRLBCL than NLPHL. In conclusion, NLPHL and THRLBCL may represent a spectrum of the same disease. The different clinical behavior of these lymphomas may be strongly influenced by differences in the lymphoma microenvironment, possibly related to the immune status of the patient at the timepoint of diagnosis.

  • t cell Histiocyte rich large b cell lymphoma an update on its biology and classification
    Virchows Archiv, 2011
    Co-Authors: Thomas Tousseyn, Christiane De Wolfpeeters
    Abstract:

    T cell/Histiocyte-rich large B-cell lymphoma (THRLBCL), originally considered an uncommon variant of Diffuse Large B-Cell Lymphoma (DLBCL), is recognized by the World Health Organisation as a separate clinicopathological entity since 2008. It predominantly affects middle aged men often presenting with advanced stage disease frequently involving spleen, liver and bone marrow at time of diagnosis. According to the WHO, this lymphoma is morphologically characterized by less than 10% of large neoplastic B cells in a background of abundant T cells and frequently Histiocytes. Differentiating THRLBCL from other lymphoproliferative disorders such as Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) and Lymphocyte-Rich classical Hodgkin lymphoma (LRcHL) is important from a clinical point of view and can be achieved in most cases, given adequate biopsy specimens, by careful morphological and immunohistochemical evaluation of both the neoplastic cells as well as the nonneoplastic stromal component. According to this WHO definition, THRLBCL is still considered a clinically heterogeneous entity, though it is noted that especially the cases containing numerous Histiocytes behave aggressively and show resistance to current therapies for DLBCL. Gene expression profiling studies of THRLBCL provided evidence for a prominent role for this histiocytic component that is important for a tolerogenic host immune response in which they may assist neoplastic cells in escaping the T cell-mediated immune surveillance. Therefore, reserving the diagnosis of THRLBCL to cases containing a large proportion of Histiocytes might be relevant, as modulating their activity could provide new therapeutic options.

C De Wolfpeeters - One of the best experts on this subject based on the ideXlab platform.

  • immunophenotypic analysis of Histiocytes involved in aids associated mycobacterium scrofulaceum infection similarities with lepromatous lepra
    Clinical and Experimental Immunology, 2008
    Co-Authors: Jan Delabie, C De Wolfpeeters, H Bobbaers, G Bilbe, Valeer Desmet
    Abstract:

    The present study reports a rare case of systemic M. scrofulaceum infection in an AIDS patient and analyses the inflammatory infiltrate in a lymph node by immunohistochemistry. Special emphasis is put on the Histiocytes. The diffuse infiltrate consists mainly of large Histiocytes that contain numerous bacilli. These cells display the phenotype of mature Histiocytes and in addition coexpress the antigens recognized by RFD7 and RFD9, both markers of different subsets of Histiocytes which have been reported to be co-expressed by the infected Histiocytes in the infiltrate of lepromatous lepra. Interdigitating reticulum cells are rare as well as T cells which are mainly of the suppressor/cytotoxic type. These findings are similar to those reported for lepromatous lepra and might indicate common deficiencies in T cell-macrophage interactions in both conditions. Superimposed on the diffuse infiltrate of large Histiocytes we observed 'monocytic granulomas', the presence of which might be related to a reactional state comparable to erythema nodosum leprosum, a reactional state of lepromatous lepra.

  • t cell Histiocyte rich large b cell lymphoma a distinct clinicopathologic entity
    Journal of Clinical Oncology, 2002
    Co-Authors: R Achten, Gregor Verhoef, L Vanuytsel, C De Wolfpeeters
    Abstract:

    PURPOSE: Although it has proven difficult to delineate diagnostically reproducible and clinically relevant subgroups, the heterogeneity of diffuse large B-cell lymphomas (DLBCL) is widely acknowledged. In 1992, we reported on six cases that suggested that large B-cell lymphoma rich in stromal Histiocytes and T cells may be identified as a distinct clinicopathologic entity within DLBCL. PATIENTS AND METHODS: An integrated clinicopathologic study of 40 cases of this DLBCL subtype is presented. RESULTS: Distinguishing a DLBCL rich in Histiocytes and reactive T cells, designated T-cell/Histiocyte–rich large B-cell lymphoma (THR-BCL), may be justified from a clinical point of view. The disease typically affects middle-aged male patients who usually present with advanced-stage disease that is not adequately managed with current therapeutic strategies. Whereas proliferation fraction and p53 overexpression, in addition to the clinical variables incorporated in the International Prognostic Index (IPI), significant...

  • Histiocyte rich t cell rich b cell lymphoma a distinct diffuse large b cell lymphoma subtype showing characteristic morphologic and immunophenotypic features
    Histopathology, 2002
    Co-Authors: R Achten, Gregor Verhoef, L Vanuytsel, C De Wolfpeeters
    Abstract:

    Histiocyte-rich, T-cell-rich B-cell lymphoma: a distinct diffuse large B-cell lymphoma subtype showing characteristic morphologic and immunophenotypic features Aims: The clinicopathological features of Histiocyte-rich, T-cell-rich B-cell lymphoma (HRTR-BCL) were first recognized in 1992. In this study, 60 cases of HRTR-BCL were analysed in order to provide a detailed morphological and immunophenotypical profile of the disorder. Methods and results: HRTR-BCL is easily distinguished from other B-cell lymphomas rich in stromal T-cells by (i) a diffuse or vaguely nodular growth pattern, (ii) the presence of a minority population of CD15−, CD20+ large neoplastic B-cells, (iii) a prominent stromal component composed of both T-cells and non-epithelioid Histiocytes, and (iv) the scarcity of small reactive B-cells. These criteria also enable a reliable distinction from lymphocyte-rich classical Hodgkin’s lymphoma (CHL), from lymphocyte-predominant Hodgkin’s lymphoma (LPHL), paragranuloma type and from peripheral T-cell lymphoma. Based on the morphology of the neoplastic cells and on their frequent bcl-6 immunoreactivity, we speculate that HRTR-BCL may be derived from a progenitor cell of germinal centre origin. Conclusions: HRTR-BCL presents characteristic clinical features, affecting predominantly middle-aged men who present with advanced stage disease and are at high risk of treatment failure. Considering these distinctive clinicopathological features, recognizing HRTR-BCL as a lymphoma entity may be justified.

  • among diffuse large b cell lymphomas t cell rich Histiocyte rich bcl and cd30 anaplastic b cell subtypes exhibit distinct clinical features
    Annals of Oncology, 2001
    Co-Authors: B Maes, R Achten, A Anastasopoulou, J C Kluinnelemans, I Teodorovic, Antonino Carbone, C De Wolfpeeters
    Abstract:

    Background: The EORTC clinical trial 20901, activated in 1990, was designed to treat non-Hodgkin's lymphomas (NHL) of intermediate/high-grade malignancy according to the Working Formulation. Established in 1994, the R.E.A.L. Classification on NHL has now replaced all former classifications. Patients and methods: We reanalysed all cases (n = 273) documented by material available for review according to the R.E.A.L. Classification. In addition, we subdivided cases recognised as diffuse large B-cell lymphoma (DLBCL) into three morphologically distinct categories, namely, large cleaved DLBCL (LC-DLBCL), T-cell-rich/Histiocyte-rich B-cell lymphoma (T-cell-rich/Histiocyte-rich BCL) and CD30+ DLBCL with anaplastic cell features (CD30+ DLBCL). Finally, T/NULL anaplastic large-cell lymphoma (ALCL) cases were subdivided into ALK+ and ALK- lymphomas. Review was performed independently by two pathologists from two different centres. Results: DLBCL (61%), T/NULL ALCL (15%) and mantle-cell lymphoma (MCL, 5%) were the main NHL categories represented in the study. Fifty-seven of one hundred sixty DLBCL cases were further subclassified as LC-DLBCL (33 cases), T-cell-rich/Histiocyte-rich BCL (13 cases) or CD30+ DLBCL (11 cases). The remaining cases were indicated as unspecified DLBCL. A clinico-pathological correlation confirmed the findings of previous studies suggesting that MCL, DLBCL and ALCL represent distinct entities with MCL being characterised by a short survival, in contrast with the longer survival and less frequent progression typical of ALK+ compared to ALK- ALCL. Within DLBCL, T-cell-rich/Histiocyte-rich BCL showed distinctive features at presentation whereas CD30+ DLBCL showed a trend towards a more favourable prognosis, that might be comparable to that of ALK+ ALCL. Conclusions: Our data further support the usefulness of the R.E.A.L. Classification and illustrate the feasibility of DLBCL subtyping. Moreover, our results demonstrate the distinct clinical characteristics of T-cell-rich/Histiocyte-rich BCL and CD30+ DLBCL with anaplastic cell features suggesting that they may represent clinico-pathologic entities.

C Bigorgne - One of the best experts on this subject based on the ideXlab platform.

  • sea blue Histiocyte syndrome in bone marrow secondary to total parenteral nutrition
    Leukemia & Lymphoma, 1998
    Co-Authors: C Bigorgne, Le A Tourneau, B Messing, B Rio, T Molina, J Audouin, K Vahedi, J Diebold
    Abstract:

    Clinical and hematological abnormalities can occur in patients receiving intravenous fat emulsions as part of a long-term parenteral nutrition; they consist of hepatosplenomegaly and peripheral blood cytopenia(s). These abnormalities lead to bone marrow examination which revealed numerous macrophages laden with blue staining pigment granules and separate lipid vacuoles, presenting the typical histochemical characteristics of sea-blue Histiocytes. Thus, long-term parenteral nutrition including fat-emulsion sources may represent a further condition in addition to the wide variety of disorders which can be associated with sea-blue histiocytosis. Moreover, in view of its clinical and morphological presentation, this storage pathological state could be compared with the so-called sea-blue Histiocyte syndrome described by Silverstein and colleagues.

  • sea blue Histiocyte syndrome in bone marrow secondary to total parenteral nutrition including fat emulsion sources a clinicopathologic study of seven cases
    British Journal of Haematology, 1996
    Co-Authors: C Bigorgne, Le A Tourneau, B Messing, B Rio, V Giraud, T Molina, J Audouin, J Diebold
    Abstract:

    Bone marrow examination revealed a lipid-laden histiocytosis in seven patients undergoing long-term total parenteral nutrition necessitated by extensive short-bowel surgical resection. Clinical abnormalities occurred during this treatment which required bone marrow examination. These included hepatosplenomegaly and peripheral blood cytopenia; the median time to the detection of these abnormalities was 64 months. The most striking change within the bone marrow was the presence of many pigment-laden Histiocytes which had the typical morphology of sea-blue Histiocytes seen in the so-called idiopathic sea-blue Histiocyte syndrome. The occurrence of sea-blue histiocytosis in the bone marrow in association with long-term parenteral nutrition for short-bowel syndrome has not, to our knowledge, been reported previously and should now be considered in the differential diagnosis of bone marrow sea-blue histiocytosis.

Gerard V Aranha - One of the best experts on this subject based on the ideXlab platform.

  • rosai dorfman disease sinus histiocytosis with massive lymphadenopathy of the pancreas second case report
    Journal of Gastrointestinal Surgery, 2009
    Co-Authors: Sean P Zivin, Mohammed Atieh, Michael J Mosier, Gladell P Paner, Gerard V Aranha
    Abstract:

    Rosai–Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. It formerly was thought to be a process limited to lymph nodes, yet RDD has been documented to occur in many organ systems, notably the bone, skin, soft tissue, central nervous system, eye and orbit, and upper respiratory tract. The digestive system, however, is affected only exceptionally, with this being only the second documented case involving the pancreas. In this case report, we present a case of a 63-year-old African-American female who was found to have a pancreatic head mass and right middle lobe pleural nodule during evaluation for obstructive jaundice. She underwent a Whipple procedure. Her pathology of both the pancreatic mass and RML lung wedge resection showed sinus histiocytosis with massive lymphadenopathy, along with extensive fibrosis intertwined with nodular mixed inflammatory infiltrate. The Histiocytes characteristically showed “emperipolesis,” in which lymphocytes had penetrated the cytoplasm and remained viable within the Histiocytes (lymphocytes continued to have free movement in the Histiocyte). In addition, the histiocytic cells were positive with S-100 protein and CD68, hallmarks of RDD. Although rare, Rosai–Dorfman disease should be considered in the differential diagnosis of patients presenting with pancreatic and/or lung nodules, especially when biopsy or cytology results report atypical inflammatory findings.