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Hepato splenic lymphoma

Alexandra Spiegel, Catherine Paillard, Stephane Ducassou, Yves Perel, Dominique Plantaz, Marion Strullu, Alice Eischen, Patrick Lutz, Laurence Lamant, Marie-Cécile Le Deley, Laurence Brugières
This study aimed to describe the clinical features and outcome of anaplastic large cell lymphoma (ALCL) with leukaemic presentation in children. Among 267 patients included in the French paediatric ALCL database between 1989 and 2012, nine (3%) were described as having cytologically detectable circulating tumour cells. Clinical features combined fever (8/9), nodal and extra-nodal disease (9/9), including hepato-splenic (9/9) and lung involvement (7/9). The level of hyperleucocytosis ranged from 30 to 120 × 10(9) /l, with 12-90% of tumour cells...
May 2014: British Journal of Haematology
Imad Absah, Michael Stephens
PURPOSE OF REVIEW: The use of antitumor necrosis factor (anti-TNF) agents to treat Crohn's disease in children has become quite common over the past decade. There are incomplete data to guide the clinician in choosing whether adjunctive therapy should be added to optimize response to these drugs. RECENT FINDINGS: Addition of immunomodulators such as thiopurines or possibly methotrexate can increase anti-TNF drug levels, reduce the risk of antidrug antibodies, and improve response...
October 2013: Current Opinion in Pediatrics
Fernando Magro, Laurent Peyrin-Biroulet, Harry Sokol, Xavier Aldeger, Antonia Costa, Peter D Higgins, Joel C Joyce, Konstantinos H Katsanos, Anthony Lopez, Teresa Mas de Xaxars, Elena Toader, Laurent Beaugerie
The incidence of lymphoproliferative disorders (LD) is increasing in developed countries. Patients with inflammatory bowel disease (IBD) exposed to thiopurines are at additional risk of three specific forms of LD: Epstein-Barr-Virus-related post-transplant like LD, hepato-splenic T-cell lymphoma and post-mononucleosis lymphoproliferation. The risk of the two latter forms of LD can be reduced when considering specific immunosuppressive strategies in young males. It is still unclear whether the risk of uterine cervix abnormalities is increased in IBD women, irrespective of the use of immunosuppressants...
January 2014: Journal of Crohn's & Colitis
S M Keller, W Vernau, J Hodges, P H Kass, J G Vilches-Moure, V McElliot, P F Moore
The clinical, clinicopathologic, and pathological findings of 9 dogs with T-cell lymphoma that involved the liver in the absence of peripheral lymphadenopathy were assessed. Seven dogs had hepatosplenic T-cell lymphoma (HS-TCL). Dogs with HS-TCL presented with hepato- and/or splenomegaly, regenerative anemia, thrombocytopenia, and hypoproteinemia. The clinical course was rapidly progressive with all dogs but 1 dead within 24 days of initial presentation. Neoplastic lymphocytes were centered on hepatic and splenic sinusoids and had a CD3+ (5/7), TCRαβ- (5/5), TCRγδ+ (3/5), CD11d+ (6/7), granzyme B+ (5/7) immunophenotype...
March 2013: Veterinary Pathology
Jens Kelsen, Anders Dige, Heinrich Schwindt, Francesco D'Amore, Finn S Pedersen, Jørgen Agnholt, Lisbet A Christensen, Jens F Dahlerup, Christian L Hvas
BACKGROUND: Concominant with the widespread use of combined immunotherapy in the management of Crohn's disease (CD), the incidence of hepato-splenic gamma-delta (γδ)-T cell lymphoma has increased sharply in CD patients. Malignant transformation of lymphocytes is believed to be a multistep process resulting in the selection of malignant γδ-T cell clones. We hypothesised that repeated infusion of anti-TNF-α agents may induce clonal selection and that concurrent treatment with immunomodulators further predisposes patients to γδ-T cell expansion...
2011: PloS One
Philip R Roelandt, Johan Maertens, Peter Vandenberghe, Chris Verslype, Tania Roskams, Raymond Aerts, Frederik Nevens, Daan Dierickx
Hepatosplenic gammadelta T-cell lymphoma is a rare lymphoproliferative disorder originating from natural killer-like Vdelta1-lymphocytes. This subtype has been described after different types of solid organ transplants. In this article, we describe the first 2 cases after liver transplantation. Both patients had thrombocytopenia with (hepato)splenomegaly but without peripheral lymphadenopathies and sinusoidal infiltration of the liver and spleen by monomorphic gammadelta-lymphocytes on pathological examination...
July 2009: Liver Transplantation
Sana Kallel, Mejda Essid, Salah Boujelbene, Ihsen Ben Brahim, Samia Chatty, Sadok Sassi, Moussadek Azzouz
Many authors suggest the role of hepatitis C virus (HCV) infection in the pathology of B-cell non Hodgkin's lymphomas; this is based on epidemiological, physiopathological and therapeutic arguments. The frequency of the association with hepatitis C virus infection is variable in the different study (1 to 30%). We report two cases of hepatitis C virus infection in association with non Hodgkin's lymphomas. The first case presented a low grad splenic and nodal non-Hodgkin's lymphoma associated with hepatitis C virus infection and complicated by hepato-cellular carcinoma...
August 2007: La Tunisie Médicale
Andrés J M Ferreri, Giuseppina P Dognini, Elías Campo, Rein Willemze, John F Seymour, Osnat Bairey, Maurizio Martelli, Amalia O De Renz, Claudio Doglioni, Carlos Montalbán, Alberto Tedeschi, Astrid Pavlovsky, Sue Morgan, Lilj Uziel, Massimo Ferracci, Stefano Ascani, Umberto Gianelli, Carlo Patriarca, Fabio Facchetti, Alessio Dalla Libera, Barbara Pertoldi, Barbara Horváth, Arpad Szomor, Emanuele Zucca, Franco Cavalli, Maurilio Ponzoni
BACKGROUND AND OBJECTIVES: This study explored variations in the clinical manifestations of intravascular lymphoma (IVL) on the bases of the association with hemophagocytosis and the country where the diagnosis was made. DESIGN AND METHODS: The clinical features of 50 Western patients with IVL were compared with those of 123 patients with IVL diagnosed in Eastern countries (87 diagnosed in Japan and 36 in other Asian countries), previously reported in English literature, and collected by an electronic bibliographic search...
April 2007: Haematologica
Ravindra Rajakariar, Madhumita Bhattacharyya, Andrew Norton, Michael Sheaff, Jamie Cavenagh, Martin J Raftery, Muhammad M Yaqoob
Post-transplant lymphoproliferative disorders (PTLDs) occur in approximately 1% of renal graft recipients. Of these, up to 15 percent are of the T-cell type. In this study, we present four cases of T-cell lymphoma from our renal transplant population, each of whom presented with non-specific symptoms, pancytopenia and/or liver dysfunction, with no obvious lymphadenopathy. They were all diagnosed with rare subsets of T-cell PTLD that included hepato-splenic T-cell lymphoma and anaplastic large cell lymphoma (ALCL)...
September 2004: American Journal of Transplantation
Akihiko Taguchi, Mutsuko Miyazaki, Shizu Sakuragi, Kenji Shinohara, Toshiaki Kamei, Yusuke Inoue
A 54-year-old woman complained of fever and hepato-splenomegaly. The pathological findings of a liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids and that of the laparoscopically resected spleen revealed the infiltration of lymphocytes in the red pulp, which was positive for CD3, CD43, CD45RO and T-cell intracellular antigen-1 (TIA-1) and was negative for betaF1, while the white pulp was spared. Genetic analysis of the spleen cells revealed the rearrangement of T-cell receptor (TCR) Cbeta1, Jdelta1 and Jgamma...
February 2004: Internal Medicine
Federico Perfetto, Roberto Tarquini, Francesco Mancuso, Simonetta Di Lollo, Silvia Tozzini, Giampiero Bellesi, Giacomo Laffi
We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented with markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic, autoimmune and metabolic liver diseases were excluded. Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease...
June 2003: World Journal of Gastroenterology: WJG
L Plank, P Szépe, M Adamkov, L Sokol, D Durcanský
We analyzed one autopsy case and two biopsy cases of primary (hepato-)splenic lymphoma, diagnosed in numerous trephine bone marrow, spleen and liver biopsies. It is a distinctive "new" type of a rare T-cell lymphoma characterized usually by rearrangement of gamma delta chains of T-cell receptor. Morphologically, the lymphoma is composed of a cytologically monotonous proliferation of small to medium sized lymphocytes, with diagnostically characteristic intrasinusoidal spread in the bone marrow, spleen and liver...
April 1999: Ceskoslovenská Patologie
M Y Shapira, O Caspi, G Amir, A Zlotogorski, Y Naparstek
Gammadelta T cell lymphoma is usually either subcutaneous or hepato-splenic and involvement of other extranodal sites is rare. Here we report an unusual case of gammadelta T cell lymphoma involving the subcutaneous tissue, vocal cords, gastric mucosa and the central nervous system with a rapidly progressive clinical course and fatal outcome. Epstein-Barr virus (EBV) was shown to be present in the tumor cells, and is thought to play a role in the pathophysiology of this particular case of lymphoma.
October 1999: Leukemia & Lymphoma
S Roncella, G Cutrona, M Truini, I Airoldi, A Pezzolo, A Valetto, D Di Martino, P Dadati, A De Rossi, M Ulivi, I Fontana, A Nocera, U Valente, M Ferrarini, V Pistoia
BACKGROUND AND OBJECTIVE: gd T-cell lymphomas are only exceptionally observed in transplanted patients. Aim of this study was the detailed characterization of one such case. DESIGN AND METHODS: The patient developed spontaneous splenic rupture six years after kidney transplantation. The splenic red pulp was infiltrated by medium-sized and large lymphoid cells with two or more nucleoli. At autopsy, similar lymphoid cells infiltrated the hepatic sinusoids. Histologic, immunologic and molecular studies were carried out...
March 2000: Haematologica
A Nosari, P L Oreste, A Biondi, M C Costantini, L Santoleri, L Intropido, G Muti, E Pungolino, L Gargantini, E Morra
Hepatosplenic gammadelta T-cell lymphoma is a rare histologic type of the peripheral T-cell lymphomas, clinically characterized by predominant involvement of liver and spleen, no or little adenopathy, and an often aggressive course; it affects mainly adolescents and young adults, with a male predominance. Postthymic T-cell malignancies are heterogeneous in their clinical and laboratory features. Among the gammadelta postthymic T-cell lymphomas, two distinct entities (cutaneous and hepatosplenic, respectively) are reported in the literature...
January 1999: American Journal of Hematology
T Inoue, Y Hirabayashi, H Mitsui, Y Furuta, Y Suda, S Aizawa, Y Ikawa
The SV40 large T gene under the control of immunoglobulin enhancer induced hyperproliferation of multi-lineage hematopoiesis in transgenic mice. Hence the disease has been considered to be an appropriate experimental model for MDS-like myelodysplasia, sequential pathological changes in the development of the disease are introduced in the report. Huge splenomegaly was the major gross abnormality, which developed with 100% frequency; neither hepato-renal, nor other thymico-lymphatic involvement was common. During the progressive increase in splenic weight, extensive proliferation of multi-lineage hemopoiesis was prominent, although no differences were apparent in the cellular proportions of each hematopoietic element compared with normal spleens, either in flow-cytometric analysis using markers for each subset of hematopoietic elements, or in the histological findings...
April 1994: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
X Troussard, O Reman, F Galateau, C Duhamel, M Leporrier
In patients with acute leukaemia, Candida infection may affect exclusively the liver and the spleen. Two such cases were revealed by persistent fever despite correction of bone marrow aplasia, abdominal pain, anicteric cholestasis and hypodense areas at computerized tomography suggesting hepatosplenic abscesses. Surgical liver biopsy confirmed the fungal infection and showed images of granuloma, mycelial filaments and yeasts; cultures were usually negative. The severity of these infections requires an early treatment, but amphotericin B is not very effective...
October 21, 1989: La Presse Médicale
S S Bessmel'tsev, K M Abdulkadyrov
Ultrasonographic examination of the abdominal cavity and retroperitoneal space was performed in 102 patients with acute leukemia (AL) of whom 70 suffered acute non-lymphoblastic leukemia (ANLL) while the other 32--acute lymphoblastic leukemia (ALL). All the patients were divided into 3 groups: primary-active disease, complete clinical and hematological remission and patients in relapse. Group 1 revealed hepato- and splenomegaly as well as changes in the ultrasonographic structure of these organs, whatever type of leukemia...
1991: Voprosy Onkologii
M Sherif, N G El-Mawla, N El-Bolkainy, S Badawi, H Awwad
Exploratory laparotomy, splenectomy and liver biopsy were carried out as a pre-treatment staging procedure in 32 cases of pathologically proved malignant lymphoma with clinically palpable spleen. The spleen was found to be negative for lymphoma in 17 cases, of which liver pathology showed bilharzial hepatic fibrosis in eight cases, nutritional cirrhosis in two cases and non-caseating granuloma in three cases. The liver was clinically positive in 13 cases. After laparotomy, three cases showed bilharzial pathology only...
March 1975: Journal of Tropical Medicine and Hygiene
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