hepatosplenic Ｔ cell lymphoma

Non-cutaneous extranodal NK/T cell lymphoproliferations constitute a heterogenous group of rare neoplasms, occurring primarily in the gastro-intestinal tract, nasal area, spleen, and liver. Their nomenclature refers to their usual clinical presentation and predilection for specific anatomic sites-i.e. extranodal NK/T-cell lymphoma, nasal-type, hepatosplenic T-cell lymphoma, primary intestinal T-cell lymphomas, indolent lymphoproliferative disorders of the gastrointestinal tract, and breast implant-associated anaplastic large cell lymphoma...

Primary myelofibrosis (PMF) is a disease that affects the bone marrow. It presents with cytopenias, hepatospleomegaly accompanied with extramedullary hematopoiesis, and often with constitutional symptoms. Cytotoxic gamma-delta T-cells are considered a distinct hepatosplenic lymohoma. This is a case presentation of a 43-year-old male with an initial diagnosis of PMF who underwent allogeneic stem cell transplantation complicated by primary graft failure. He subsequently underwent a partial splenic embolization; however, he quickly developed a fulminant hepatosplenic gamma-delta T-cell lymphoma which led to his death that was diagnosed posthumously...

Hepatic involvement by a T-cell neoplasm is rare and often challenging to diagnose in liver biopsies. We collected 40 cases of T-cell neoplasms diagnosed in the liver from five large academic institutions to assess the clinicopathologic features. The patients included 11 women and 29 men, with a median age of 54 (range: 2-75) years and a high mortality rate (31/37, 83.8%). Fourteen (35%) patients were diagnosed with hepatosplenic T-cell lymphoma (HSTCL), 13 (32.5%) peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), and 13 (32...

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BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of non-Hodgkin's lymphoma, which has an aggressive clinical course and an extremely poor prognosis. Chidamide is a novel, orally active, benzamide-type histone deacetylase (HDAC) inhibitor that has been used for peripheral T-cell lymphoma (PTCL) treatment. However, to date, there has been no report of the treatment and effect of the HDAC inhibitor chidamide in HSTCL, which is a special subtype of PTCL. CASE SUMMARY: A 45-year-old male patient was admitted with splenomegaly and slight bicytopenia...

Hepatosplenic T-cell lymphoma is a rare but highly aggressive form of T-cell malignancy. As cases are not routinely seen in practice, the malignancy can be confused with other hematologic conditions that have a similar presentation. Here in, we present the challenges faced in diagnosing a 27-year-old-male who initially presented with asymptomatic pancytopenia and then developed massive splenomegaly over the next three months. After an elaborate workup, including a bone marrow biopsy and extensive serological testing, which all turned out to be negative, he eventually underwent a splenectomy with biopsy results confirming hepatosplenic T-cell lymphoma...

A 64-year old Chinese male patient was admitted to our hospital because of severe jaundice that persisted for 2 months. No swollen lymph nodes or hepatosplenomegaly was detected on physical examination. His laboratory data indicated high levels of direct bilirubin, alkaline phosphatase, aspartate aminotransferase, and alanine aminotransferase. No abnormality was revealed on abdominal computed tomography (CT). However, positron emission tomography (PET)-CT revealed diffuse hypermetabolism in the liver and spleen...

Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. HSTCL is more common in adolescent and young adults and has a rapidly progressive clinical course and poor outcome due to its refractoriness to conventional chemotherapy regimens. Approximately 20% of the cases arise in the background of chronic immunosuppression or immune dysregulation. Patients commonly present with constitutional symptoms, hepatic and liver enlargement, and cytopenias; hematophagocytic syndrome can also occur...

Introduction: Human immunodeficiency virus (HIV) positive individuals with the CD4 count less than 200 cells/mm3 are at risk for opportunistic infections. Pneumocystis jirovecii , a fungal pathogen, is a common cause of opportunistic infections with predominantly pulmonary involvement. Disseminated P. jiroveciii infection presenting with hepatosplenic lesion is extremely rare. Case Summary . A 31-year-old male with HIV with and acquired immunodeficiency syndrome (AIDS) presented with diarrhea for 3 weeks...

Objective: To analyze the cell morphological features of hepatosplenic T-cell lymphoma (HSTCL) gamma-delta (γδ) type, differentiate from acute leukaemia (AL). Methods: This was a retrospective study. The clinical data of four cases of HSTCL γδ type who were treated in Hebei Yanda Ludaopei Hospital from 2009 to 2014 were collected. Their initial morphology diagnoses in other hospitals were all acute leukemia or myelodysplastic syndrome (MDS). Morphological analysis and cytochemical stains to their bone marrow (BM) aspiration and peripheral blood (PB) smears were completed when they had no response to previous chemotherapies, and the morphological reports were compared with results of immunophenotyping, chromosome, and T cell receptor (TCR) gene rearrangement...

Pediatric-type follicular (PTFL), marginal zone (MZL), and peripheral T-cell lymphoma (PTCL) account each for <2% of childhood non-Hodgkin lymphoma. We present clinical and histopathological features of PTFL, MZL, and few subtypes of PTCL and provide treatment recommendations. For localized PTFL and MZL, watchful waiting after complete resection is the therapy of choice. For PTCL, therapy is subtype-dependent and ranges from a block-like anaplastic large cell lymphoma (ALCL)-derived and, alternatively, leukemia-derived therapy in PTCL not otherwise specified and subcutaneous panniculitis-like T-cell lymphoma to a block-like mature B-NHL-derived or, preferentially, ALCL-derived treatment followed by hematopoietic stem cell transplantation in first remission in hepatosplenic and angioimmunoblastic T-cell lymphoma...

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...

INTRODUCTION: Hepatosplenic T-cell lymphoma (HSTCL) is a rare peripheral T-cell lymphoma that disproportionately affects individuals with a clinical history of immunosuppression. It carries a poor prognosis, and, owing to its rarity, there is no single or well-established treatment. PATIENTS AND METHODS: We conducted the largest-to-date individual-level meta-analysis based on literature searches to determine the best induction therapy for HSTCL. We compared response rates and survival among patients who received "non-CHOP-based" induction with regimens containing cytarabine, etoposide, and/or platinum-based treatment to those receiving treatment with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOP-like therapy...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Background: Patients with human immunodeficiency virus (HIV) are more likely to develop cancer. Malignant lymphomas are the main cancer group seen in these patients. Diffuse large B-cell lymphoma including central nervous system lymphoma and Burkitt's lymphoma account for 90% of HIV-related non-Hodgkin's lymphomas. Clinical case: A 22-year-old man with fever up to 39 ° C, malaise, excessive tiredness and night sweats, loss of 8 kg of weight, abdominal pain in the right hypochondrium, all 5 months before hospitalization...

BACKGROUND: Hepatosplenic T-cell lymphoma (HSTCL) is a rare, poorly treatable malignancy associated with therapy for IBD. Current knowledge of HSTCL risk in IBD comes from an era of step-up therapy, before earlier use of biologics or combination therapy was advocated to achieve deep mucosal healing. HSTCL risk among newer biologic classes has also not been evaluated. AIMS: To systematically characterise the association of HSTCL with biologic therapy for IBD. METHODS: We conducted a literature search and query of the Food and Drug Administration Adverse Event Reporting System to summarise HSTCL cases among IBD patients with prior biologic exposure...

Hepatosplenic γδ T-cell lymphoma (HSTCL) is a very rare peripheral T-cell lymphoma characterized by extranodal infiltration of mature malignant post-thymic T-lymphocytes into sinusoids of the liver and spleen without lymphadenopathy and significant cytopenias. It is an aggressive form of lymphoma, resistant to the conventional chemotherapy. We report a case of HSTCL in pregnancy. Because this condition is an extremely aggressive and rare entity, data from these cases can help confirm the most suitable treatment regimen and timing of initiation...

T-cell lymphoproliferative processes in the spleen are rare and it is important to study normal T cell subsets in the spleen to understand the splenic milieu in which they arise. True malignant T-cell processes including hepatosplenic T-cell lymphoma and T-cell large granular lymphocytic leukemia occur in the spleen, but other atypical reactive T-cell proliferations and those of uncertain significance also have been described. Proper distinction of florid T cell responses from malignant T-cell neoplasms has important therapeutic implications for the patient...