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Hepatosplenic lymphoma

Mariko Yabe, L Jeffrey Medeiros, Sa A Wang, Guilin Tang, Carlos E Bueso-Ramos, Jeffrey L Jorgensen, Govind Bhagat, Weina Chen, Shaoying Li, Ken H Young, Roberto N Miranda
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...
October 13, 2016: American Journal of Surgical Pathology
Arti Karpate, Carmen Barcena, Daniel Hohl, Bettina Bisig, Laurence de Leval
No abstract text is available yet for this article.
August 25, 2016: Virchows Archiv: An International Journal of Pathology
Florian Brinkert, Philomena Arrenberg, Till Krech, Enke Grabhorn, Ansgar Lohse, Christoph Schramm
We report the first 2 cases of hepatosplenic T-cell lymphoma in adolescents diagnosed with autoimmune hepatitis under long-term treatment with azathioprine. Patients presented with fatigue, hepatosplenomegaly, and pancytopenia. The diagnosis could be confirmed performing biopsies of liver and spleen or bone marrow, which demonstrated infiltration of neoplastic T lymphocytes with the typical phenotype with both markers, CD56(+) and TCRγδ(+) Thus, these cases emphasize the need to constantly reevaluate the administered dose and duration of thiopurines for autoimmune hepatitis, especially in adolescents...
September 2016: Pediatrics
Joo Y Song, Liping Song, Alex F Herrera, Girish Venkataraman, Joyce L Murata-Collins, Victoria H Bedell, Yuan Yuan Chen, Young S Kim, Reda Tadros, Bharat N Nathwani, Dennis D Weisenburger, Andrew L Feldman
Cyclin D1 is an important regulator of the cell cycle and overexpression of this protein by immunohistochemistry is characteristically seen in mantle cell lymphoma and other B-cell neoplasms. However, little is known about the expression of this protein in T-cell lymphomas. Cyclin-dependent kinase pathway inhibitors are in development, therefore identifying cyclin D1-positive T-cell lymphomas may provide a therapeutic target in a disease where novel treatments are urgently needed. We collected 200 peripheral T-cell lymphomas from three institutions including the following types of cases: 34 anaplastic large cell lymphoma, ALK+, 44 anaplastic large cell lymphoma, ALK negative, 68 peripheral T-cell lymphomas, not otherwise specified, 24 angioimmunoblastic T-cell lymphomas, 7 extranodal NK/T-cell lymphomas, 4 enteropathy associated T-cell lymphomas, 3 hepatosplenic T-cell lymphomas, 12 cutaneous T-cell lymphomas, and 4 large granular lymphocytic leukemias...
July 29, 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Anupriya Bansal, Suchi Mittal, Jasmita Dass, Nitin Gupta, P K Agarwal, Jyoti Kotwal
De novo CD5+ Diffuse large B cell lymphoma (DLBCL) is a rare and aggressive subtype of DLBCL. It is a distinct clinicopathologic entity with complex molecular profile and poor prognosis. A 59 year old female presented with pyrexia of unknown origin since 1 month. On examination, there was severe pallor, hepatosplenomegaly and no palpable lymphadenopathy. Complete blood count revealed bicytopenia with normal total leucocyte count. Liver and renal function tests were normal. Ultrasonography abdomen revealed splenic enlargement with two focal lesions attributed to either splenic abscess or infarcts...
June 2016: Indian Journal of Hematology & Blood Transfusion
K Mellgren, A Attarbaschi, O Abla, S Alexander, S Bomken, E Bubanska, A Chiang, M Csóka, A Fedorova, E Kabickova, L Kapuscinska-Kemblowska, R Kobayashi, Z Krenova, F Meyer-Wentrup, N Miakova, M Pillon, G Plat, A Uyttebroeck, D Williams, G Wróbel, U Kontny
Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL)...
August 2016: Annals of Hematology
Kittiyod Poovorawan, Aung Myint Thu, Maleerat Sutherat, Weerapong Phumratanaprapin, Naruemon Wisedopas, Viravarn Luvira, Wirongrong Chierakul
We report here a case of hepatic lymphoma and splenic aspergillosis in an elderly patient with diabetes mellitus, exhibiting hepatosplenic abscesses mimicking melioidosis. Immunohistochemistry confirmed the diagnosis of a diffuse hepatic large B-cell lymphoma. Biopsy of the spleen revealed a clump of fungus with a slender shape and dichotomous branching, morphologically consistent with aspergillosis. Hepatosplenic abscesses are a common presentation in melioidosis, but this case reveals this assumption can lead to misdiagnosis...
March 2016: Southeast Asian Journal of Tropical Medicine and Public Health
C-C Wang, L-Y Chen, K Liu, X-Q Liu, D-J Li
OBJECTIVE: This study was to determine whether Image-Guided Percutaneous Liver Biopsy (IGPLB) is a safe and accurate procedure in patients with Hepatosplenic Gamma-Delta T-Cell Lymphoma (HSTCL) characterized by hepatosplenomegaly, and provide a rapid and safe diagnostic approach in this rare group of patients with a generally poor outcome. PATIENTS AND METHODS: We report five patients who underwent IGPLB for an initial diagnosis of HSTCL, in whom diagnosis by bone marrow biopsy and blood smear had failed...
May 2016: European Review for Medical and Pharmacological Sciences
Isabelle Arnoux, Marie Loosveld
No abstract text is available yet for this article.
January 14, 2016: Blood
Shano Naseem, Maninderbir Kaur, Manupdesh Singh Sachdeva, Jasmina Ahluwalia, Reena Das, Neelam Varma, Subhash Varma
BACKGROUND: Mature T/ NK-cell neoplasms are a rare group of disorders and their presentation as leukemia is even rarer. Most of the previous studies have focused on mature B-cell lineage leukemias and there is a paucity of data on mature T/NK-cell lineage leukemias. We, therefore, planned this study to analyze their spectrum, frequency, morphology and immunophenotypic features. SUBJECTS AND METHODS: All cases of lymphomas presenting as leukemia over a period of two and a half years were evaluated...
January 1, 2016: International Journal of Hematology-oncology and Stem Cell Research
Maja Petrova, Manuel Mendes Gomes, Jose Pedro Nascimento Carda, José Pereira de Moura
Hepatosplenic T-cell lymphoma (HSTCL) is a rare, aggressive type of peripheral T-cell lymphoma that is characterised by extranodal disease, with infiltration and proliferation of malignant T-cells within the liver, spleen and bone marrow. The authors report the case of a young immunocompetent man, who was admitted to the hospital with a history of prolonged, unexplained fever, fatigue and weight loss. Initial blood work showed mild pancytopaenia and imaging studies revealed hepatosplenomegaly. The diagnosis was challenging, initially mimicking infectious disease, and it required an extensive investigation that ultimately revealed the characteristic clinical, histopathological and cytogenetic features of HSTCL...
2016: BMJ Case Reports
Mariko Yabe, L Jeffrey Medeiros, Guilin Tang, Sa A Wang, Keyur P Patel, Mark Routbort, Govind Bhagat, Carlos E Bueso-Ramos, Jeffrey L Jorgensen, Rajyalakshmi Luthra, Weina Chen, Tariq Muzzafar, Rashmi Kanagal-Shamanna, Joseph D Khoury, Yahya Daneshbod, Masoud Davanlou, Shaoying Li, Ken H Young, Roberto N Miranda
Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell lymphoma commonly associated with cytopenias. The pathogenesis of cytopenias in patients with HSTCL is not well defined, although the presence of dyspoietic hematopoietic cells and the common association with trisomy 8 raise the possibility of an associated myelodysplastic syndrome (MDS). In 25 bone marrow specimens involved by HSTCL, we systematically assessed for morphologic features of dyspoiesis and correlated the findings with peripheral cytopenia(s), cytogenetic findings, and detection of chromosome 8 by fluorescence in situ hybridization...
April 2016: Human Pathology
Vinodh Pillai, Michael Tallarico, Michael R Bishop, Megan S Lim
Mature T/Natural killer (NK)-cell neoplasms of children and the young adolescent population exhibit higher prevalence in Central and South American and Asian populations and many are associated with Epstein-Barr virus (EBV). They are represented in large part by extranodal T/NK cell lymphomas- nasal-type or extra nasal-type, chronic lymphoproliferative disorders of T/NK cells or chronic active EBV disease, systemic EBV-positive lymphoproliferative disorders of childhood, hydroa vacciniforme-like lymphoma, hepatosplenic T-cell lymphoma and primary cutaneous gamma/delta T-cell lymphoma among others...
May 2016: British Journal of Haematology
Mariko Yabe, L Jeffrey Medeiros, Guilin Tang, Sa A Wang, Sairah Ahmed, Yago Nieto, Shimin Hu, Govind Bhagat, Yasuhiro Oki, Keyur P Patel, Mark Routbort, Rajyalakshmi Luthra, Michelle A Fanale, Carlos E Bueso-Ramos, Jeffrey L Jorgensen, Francisco Vega, Weina Chen, Daniela Hoehn, Sergej Konoplev, Denai R Milton, Ignacio Wistuba, Shaoying Li, M James You, Ken H Young, Roberto N Miranda
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)...
May 2016: American Journal of Surgical Pathology
Hidetsugu Kawai, Hiromichi Matsushita, Ken Ohmachi, Minoru Kojima, Shinichiro Machida, Yoshiaki Ogawa, Hiroshi Kawada, Naoya Nakamura, Kiyoshi Ando
Hepatosplenic T-cell lymphoma (HSTCL), a rare type of γδ T-cell lymphoma, is characterized by hepatosplenomegaly and cytopenias. It is associated with immunodeficiency and its age of onset is reportedly between the 20s and 30s. We herein report 4 Japanese HSTCL cases. Three of them, including an elderly case that was 74 years of age, were not at adolescence. No cases had a history of immunodeficiency. All other disease phenotypes were similar to the typical HSTCL cases. These findings suggest that there are a certain proportion of HSTCL patients who presented after middle age...
2016: Leukemia Research Reports
Emmanuel Benayoun, Orianne Wagner-Ballon
No abstract text is available yet for this article.
October 22, 2015: Blood
Abhijit Saste, Javier Arias-Stella, Philip Kuriakose
A 24-year-old male presented with abdominal pain, fever, and palpable splenomegaly. His differential count revealed myelocytes, metamyelocytes, and nucleated red cells. A bone marrow biopsy confirmed a diagnosis of hepatosplenic gamma delta T-cell leukemia/lymphoma. We describe here our center's diagnostic and treatment approach for this rare leukemia.
January 2016: Clinical Case Reports
Marco Pizzi, Shannon Covey, Susan Mathew, Yen-Chun Liu, Jia Ruan, John P Leonard, Amy Chadburn
No abstract text is available yet for this article.
March 2016: Clinical Lymphoma, Myeloma & Leukemia
Feryal A Ibrahim, Vignesh Shanmugam, Aliaa Amer, Halima El-Omri, Ahmad Al-Sabbagh, Ruba Y Taha, Dina S Soliman
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδ T-cell receptor (TCR), but recently, a few cases have been shown to express the αβ TCR. Comparison of these two subtypes (αβ and γδ) shows similar clinicopathologic and cytogenetic features; however, due to the paucity of reported cases, it is not clear whether they are prognostically distinct entities. We report a case of αβ HSTCL with a rather unusual presentation of Coombs'-negative hemolytic anemia...
2015: Clinical Medicine Insights. Oncology
Neha Mehta-Shah, Steven Horwitz
Although all the peripheral T-cell lymphomas are uncommon, there are some entities that are truly rare. Subtypes, such as enteropathy-associated T-cell lymphoma, hepatosplenic T-cell lymphoma, extranodal NK/T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma, have an approximate annual incidence in United States of <500 each. In these very rare subtypes, there is limited data to guide clinical decision-making. As such, our treatment decisions are often based on extrapolation, case series, personal experience, and biases...
2015: Hematology—the Education Program of the American Society of Hematology
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