The Experts below are selected from a list of 1314 Experts worldwide ranked by ideXlab platform

L. Jeffrey Medeiros - One of the best experts on this subject based on the ideXlab platform.

  • Hepatosplenic T-Cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors.
    Human pathology, 2018
    Co-Authors: Mariko Yabe, Roberto N. Miranda, L. Jeffrey Medeiros
    Abstract:

    Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare and clinically aggressive type of T-Cell Lymphoma that arises most often in adolescents and young adults. Patients with HSTCL commonly present with B-symptoms and cytopenias, which may suggest a diagnosis of acute leukemia initially. Patients present with extranodal disease involving the spleen, liver and bone marrow; lymphadenopathy is usually absent. The Lymphoma cells can show a spectrum of cell sizes and are of T-Cell lineage, often negative for CD4 and CD8 and positive for T-Cell receptor γδ or, less often, αβ. Recent studies have identified gene mutations in oncogenic pathways that are likely involved in pathogenesis and may be targets for therapy. Mutations in STAT3 or STAT5B lead to activation of the JAK/STAT pathway, and mutations involving SETD2, IN080 and ARID1 are involved in chromatin modification. Currently, there is no consensus standard of care for HSTCL patients, although several studies support a role for allogeneic hematopoietic stem cell transplant. Although patients with HSTCL are best treated in the context of clinical trials, the rarity of these neoplasms likely necessitates a multi-institutional approach. In this review, we focus on the clinicopathologic and genetic characteristics of HSTCL. We also discuss the differential diagnosis and therapeutic approaches.

  • Distinguishing Between Hepatosplenic T-Cell Lymphoma and γδ T-Cell Large Granular Lymphocytic Leukemia: A Clinicopathologic, Immunophenotypic, and Molecular Analysis.
    The American journal of surgical pathology, 2017
    Co-Authors: Mariko Yabe, L. Jeffrey Medeiros, Guilin Tang, A. Wang, Govind Bhagat, Carlos E. Bueso-ramos, Jeffrey L. Jorgensen, Weina Chen, Ken H. Young
    Abstract:

    Abstract Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare, aggressive T-Cell Lymphoma that can be challenging to diagnose. In particular, distinguishing HSTCL from T-Cell large granular lymphocytic (T-LGL) leukemia of γδ T-Cell receptor (TCR) type is difficult without examination of a splenectomy specimen. In this study, we systematically assessed a series of HSTCL cases for findings reported in the literature as supporting or not supporting the diagnosis of HSTCL. We also compared HSTCL with a group of cases of T-LGL of γδ TCR type. Criteria assessed in this study included: B-symptoms, massive splenomegaly, lymphadenopathy, extranodal involvement, peripheral lymphocytosis, Lymphoma cells that expand bone marrow sinuses, lymphocyte azurophilic granules, immunophenotype, evidence of infection by Epstein-Barr virus, human immunodeficiency virus, or human T-Cell leukemia virus type 1, isochromosome 7q, trisomy 8, and TCR gene rearrangement status. On the basis of the data of this study, we conclude that massive splenomegaly, bone marrow sinusoidal expansion by Lymphoma cells, and lymphocytes devoid of azurophilic granules were significantly more common in HSTCL patients than in γδ T-LGL patients (P

  • Prognostic Factors of Hepatosplenic T-Cell Lymphoma: Clinicopathologic Study of 28 Cases.
    The American journal of surgical pathology, 2016
    Co-Authors: Mariko Yabe, L. Jeffrey Medeiros, Guilin Tang, A. Wang, Sairah Ahmed, Yago Nieto, Govind Bhagat, Yasuhiro Oki, Keyur P. Patel
    Abstract:

    Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare type of Lymphoma. Patients have a poor prognosis, and there is no standard of care. We evaluated 28 HSTCL patients to determine factors that may be associated with outcome. There were 19 men and 9 women with a median age of 32.5 years. Most patients had massive splenomegaly, and bone marrow showed sinusoidal involvement by Lymphoma. The HSTCL cells expressed γδ T-Cell receptor (TCR) in 20 (74%), αβ TCR in 5 (19%), and neither in 2 (7%) patients (1 case not assessed). Conventional cytogenetics and/or fluorescence in situ hybridization analysis in 24 patients at diagnosis showed isochromosome 7q (i7q) in 10 (42%) and trisomy 8 in 8 (33%) patients. Median overall survival (OS) and event-free survival (EFS) were each 28.3 months. Serum bilirubin level ≥1.5 mg/dL, αβ TCR expression, and trisomy 8 each correlated significantly with shorter OS and EFS. Patients with HSTCL received a variety of chemotherapy regimens with no regimen better than any other. However, patients who underwent stem cell transplant showed longer survival (OS: hazard ratio 0.3, P=0.09; EFS: hazard ratio 0.2, P=0.034). In conclusion, although HSTCL patients have a poor prognosis overall, the data presented support the novel suggestions that HSTCL patients can be stratified into 2 prognostic groups, with an elevated serum bilirubin level, αβ TCR expression, and trisomy 8 identifying a poorer prognostic group. In addition, the outcomes of this patient cohort suggest that stem cell transplantation has value for the treatment of patients with HSTCL.

Mariko Yabe - One of the best experts on this subject based on the ideXlab platform.

  • Hepatosplenic T-Cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors.
    Human pathology, 2018
    Co-Authors: Mariko Yabe, Roberto N. Miranda, L. Jeffrey Medeiros
    Abstract:

    Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare and clinically aggressive type of T-Cell Lymphoma that arises most often in adolescents and young adults. Patients with HSTCL commonly present with B-symptoms and cytopenias, which may suggest a diagnosis of acute leukemia initially. Patients present with extranodal disease involving the spleen, liver and bone marrow; lymphadenopathy is usually absent. The Lymphoma cells can show a spectrum of cell sizes and are of T-Cell lineage, often negative for CD4 and CD8 and positive for T-Cell receptor γδ or, less often, αβ. Recent studies have identified gene mutations in oncogenic pathways that are likely involved in pathogenesis and may be targets for therapy. Mutations in STAT3 or STAT5B lead to activation of the JAK/STAT pathway, and mutations involving SETD2, IN080 and ARID1 are involved in chromatin modification. Currently, there is no consensus standard of care for HSTCL patients, although several studies support a role for allogeneic hematopoietic stem cell transplant. Although patients with HSTCL are best treated in the context of clinical trials, the rarity of these neoplasms likely necessitates a multi-institutional approach. In this review, we focus on the clinicopathologic and genetic characteristics of HSTCL. We also discuss the differential diagnosis and therapeutic approaches.

  • Distinguishing Between Hepatosplenic T-Cell Lymphoma and γδ T-Cell Large Granular Lymphocytic Leukemia: A Clinicopathologic, Immunophenotypic, and Molecular Analysis.
    The American journal of surgical pathology, 2017
    Co-Authors: Mariko Yabe, L. Jeffrey Medeiros, Guilin Tang, A. Wang, Govind Bhagat, Carlos E. Bueso-ramos, Jeffrey L. Jorgensen, Weina Chen, Ken H. Young
    Abstract:

    Abstract Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare, aggressive T-Cell Lymphoma that can be challenging to diagnose. In particular, distinguishing HSTCL from T-Cell large granular lymphocytic (T-LGL) leukemia of γδ T-Cell receptor (TCR) type is difficult without examination of a splenectomy specimen. In this study, we systematically assessed a series of HSTCL cases for findings reported in the literature as supporting or not supporting the diagnosis of HSTCL. We also compared HSTCL with a group of cases of T-LGL of γδ TCR type. Criteria assessed in this study included: B-symptoms, massive splenomegaly, lymphadenopathy, extranodal involvement, peripheral lymphocytosis, Lymphoma cells that expand bone marrow sinuses, lymphocyte azurophilic granules, immunophenotype, evidence of infection by Epstein-Barr virus, human immunodeficiency virus, or human T-Cell leukemia virus type 1, isochromosome 7q, trisomy 8, and TCR gene rearrangement status. On the basis of the data of this study, we conclude that massive splenomegaly, bone marrow sinusoidal expansion by Lymphoma cells, and lymphocytes devoid of azurophilic granules were significantly more common in HSTCL patients than in γδ T-LGL patients (P

  • Prognostic Factors of Hepatosplenic T-Cell Lymphoma: Clinicopathologic Study of 28 Cases.
    The American journal of surgical pathology, 2016
    Co-Authors: Mariko Yabe, L. Jeffrey Medeiros, Guilin Tang, A. Wang, Sairah Ahmed, Yago Nieto, Govind Bhagat, Yasuhiro Oki, Keyur P. Patel
    Abstract:

    Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare type of Lymphoma. Patients have a poor prognosis, and there is no standard of care. We evaluated 28 HSTCL patients to determine factors that may be associated with outcome. There were 19 men and 9 women with a median age of 32.5 years. Most patients had massive splenomegaly, and bone marrow showed sinusoidal involvement by Lymphoma. The HSTCL cells expressed γδ T-Cell receptor (TCR) in 20 (74%), αβ TCR in 5 (19%), and neither in 2 (7%) patients (1 case not assessed). Conventional cytogenetics and/or fluorescence in situ hybridization analysis in 24 patients at diagnosis showed isochromosome 7q (i7q) in 10 (42%) and trisomy 8 in 8 (33%) patients. Median overall survival (OS) and event-free survival (EFS) were each 28.3 months. Serum bilirubin level ≥1.5 mg/dL, αβ TCR expression, and trisomy 8 each correlated significantly with shorter OS and EFS. Patients with HSTCL received a variety of chemotherapy regimens with no regimen better than any other. However, patients who underwent stem cell transplant showed longer survival (OS: hazard ratio 0.3, P=0.09; EFS: hazard ratio 0.2, P=0.034). In conclusion, although HSTCL patients have a poor prognosis overall, the data presented support the novel suggestions that HSTCL patients can be stratified into 2 prognostic groups, with an elevated serum bilirubin level, αβ TCR expression, and trisomy 8 identifying a poorer prognostic group. In addition, the outcomes of this patient cohort suggest that stem cell transplantation has value for the treatment of patients with HSTCL.

Toshiro Kurokawa - One of the best experts on this subject based on the ideXlab platform.

  • A case of Hepatosplenic T-Cell Lymphoma successfully treated by HLA haploidentical stem cell transplantation.
    Journal of clinical and experimental hematopathology : JCEH, 2020
    Co-Authors: Noriko Iwaki, Kanako Mochizuki, Jun Ozaki, Yoshinobu Maeda, Toshiro Kurokawa
    Abstract:

    We report a case of Hepatosplenic T-Cell Lymphoma (HSTL) transplanted from an HLA-haploidentical daughter. A 51-year-old man was referred due to liver function test abnormalities and fever. He was confirmed to have γδ-type HSTL by bone marrow and liver biopsies. He was treated with five cycles of a CHOP regimen. Although metabolic complete response (CR), as defined by positron emission tomography, was achieved, his bone marrow still contained tumor cells on polymerase chain reaction (PCR). He underwent transplantation using unmanipulated peripheral blood stem cells from his HLA-haploidentical daughter. The preconditioning regimen consisted of fludarabine, melphalan, busulfan and antithymocyte globulin. Graft-versus-host disease (GVHD) prophylaxis consisted of tacrolimus and short-term methotrexate. Neutrophil engraftment was achieved on day 14. His bone marrow exhibited a completely female phenotype by fluorescence in situ hybridization, and no Lymphoma cells were detected by PCR on day 30. Although he developed grade II acute GVHD on day 47, it was successfully treated by prednisolone. He has a limited type of skin chronic GVHD and still receives oral immunosuppressive therapy. He remains in CR four years after transplantation.

Jeffrey S. Hyams - One of the best experts on this subject based on the ideXlab platform.

  • Hepatosplenic T-Cell Lymphoma in adolescents and young adults with Crohn's disease: a cautionary tale?
    Inflammatory bowel diseases, 2007
    Co-Authors: Joel R. Rosh, Thomas G. Gross, Petar Mamula, Anne M. Griffiths, Jeffrey S. Hyams
    Abstract:

    Therapy for the inflammatory bowel diseases increasingly includes the use of immune-modifying and biologic therapies. Recently, in young patients with IBD, an association has been noted between the use of infliximab along with concomitant purine analogues and the development of Hepatosplenic T-Cell Lymphoma (HSTCL)-a rare and all but incurable form of non-Hodgkin's Lymphoma. This report briefly reviews the issue of Lymphoma and IBD therapy. Additionally, a description of HSTCL and a summary of the known cases of this apparent therapeutic complication are presented. Clinical options in light of this new information are explored.

Keyur P. Patel - One of the best experts on this subject based on the ideXlab platform.

  • Prognostic Factors of Hepatosplenic T-Cell Lymphoma: Clinicopathologic Study of 28 Cases.
    The American journal of surgical pathology, 2016
    Co-Authors: Mariko Yabe, L. Jeffrey Medeiros, Guilin Tang, A. Wang, Sairah Ahmed, Yago Nieto, Govind Bhagat, Yasuhiro Oki, Keyur P. Patel
    Abstract:

    Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare type of Lymphoma. Patients have a poor prognosis, and there is no standard of care. We evaluated 28 HSTCL patients to determine factors that may be associated with outcome. There were 19 men and 9 women with a median age of 32.5 years. Most patients had massive splenomegaly, and bone marrow showed sinusoidal involvement by Lymphoma. The HSTCL cells expressed γδ T-Cell receptor (TCR) in 20 (74%), αβ TCR in 5 (19%), and neither in 2 (7%) patients (1 case not assessed). Conventional cytogenetics and/or fluorescence in situ hybridization analysis in 24 patients at diagnosis showed isochromosome 7q (i7q) in 10 (42%) and trisomy 8 in 8 (33%) patients. Median overall survival (OS) and event-free survival (EFS) were each 28.3 months. Serum bilirubin level ≥1.5 mg/dL, αβ TCR expression, and trisomy 8 each correlated significantly with shorter OS and EFS. Patients with HSTCL received a variety of chemotherapy regimens with no regimen better than any other. However, patients who underwent stem cell transplant showed longer survival (OS: hazard ratio 0.3, P=0.09; EFS: hazard ratio 0.2, P=0.034). In conclusion, although HSTCL patients have a poor prognosis overall, the data presented support the novel suggestions that HSTCL patients can be stratified into 2 prognostic groups, with an elevated serum bilirubin level, αβ TCR expression, and trisomy 8 identifying a poorer prognostic group. In addition, the outcomes of this patient cohort suggest that stem cell transplantation has value for the treatment of patients with HSTCL.