hepatosplenic Ｔ cell lymphoma

AIMS: The causes and diagnosis of 'double-negative' (CD3+CD4-CD8-) T-cell lymphocytosis are not well studied. We aimed to define the causes of double-negative T-cell lymphocytosis in children and adults, and to identify simple clinical and laboratory features that would help to differentiate between the underlying conditions. METHODS: We collected clinical and laboratory data on 10 children and 30 adults with significantly increased peripheral-blood double-negative T-cells (>10% of total lymphocytes)...

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PURPOSE OF REVIEW: With the advent of biologic therapies for the treatment of IBD, the roles of thiopurines have continued to evolve. This review will focus on recent advances in pharmacology and the safety and efficacy of thiopurines as maintenance therapies for steroid-induced remissions, post-surgical maintenance of remission, and as combination therapies to reduce immunogenicities of biologic agents. RECENT FINDINGS: Due to pharmacogenetics of TPMT, thiopurine dosing is more effectively based on monitoring of thiopurine metabolites rather than weight-based dosing...

Hepatosplenic T-cell lymphoma (HSTL) is an uncommon, aggressive peripheral T-cell lymphoma with a dismal prognosis, usually expressing gamma-delta T-cell receptor on immunohistochemical study. We report the second instance in the literature of a solitary skin nodule heralding recurrence of HSTL. The patient was a 40-year-old man in apparent remission from HSTL, 4 years after chemotherapy and autologous bone marrow transplant. Biopsy of a flank lesion showed atypical lymphoid cells involving the dermis with a perivascular and periadnexal pattern, and fat lobules of the subcutaneous tissue...

The T Cell Project was the largest prospective trial to explore the incidence, treatment patterns, and outcomes for T cell lymphomas. The rare subtypes of T cell lymphomas, including hepatosplenic T cell lymphoma (HSTCL), enteropathy associated T cell lymphoma (EATL), and peripheral gamma delta T cell lymphomas (PGDTCLs) are poorly represented in most studies and there is little data regarding treatment patterns. We report results from 115 patients with hepatosplenic (n = 31), enteropathy associated (n = 65), and PGDTCLs (n = 19)...

F-FDG PET/CT for a 74-year-old man with elevated serum soluble interleukin 2 receptor showed multiple intense uptake in the liver, spleen, and bone. A surgical biopsy from 2 of liver tumors confirmed hepatosplenic αβ T-cell lymphoma. One and a half months after biopsy, FDG PET scan was performed again for staging before starting chemotherapy, and it showed the complete disappearance of all of the lesions. The excisional biopsy could be a possible trigger of his spontaneous regression.

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Peripheral T-cell lymphoma comprises a heterogeneous group of mature non-Hodgkin lymphomas. Their diagnosis is challenging, with up to 30% of cases remaining unclassifiable and referred to as "not otherwise specified". We developed a reverse transcriptase-multiplex ligation-dependent probe amplification gene expression profiling assay to differentiate the main T-cell lymphoma entities and to study the heterogeneity of the "not specified" category. The test evaluates the expression of 20 genes, including 17 markers relevant to T-cell immunology and lymphoma biopathology, one EBV-related transcript, and variants of RHOA (G17V) and IDH2 (R172K/T)...

Cirrhosis resulting from autoimmune hepatitis is associated with an increased risk of hepatocellular carcinoma. A common treatment for autoimmune hepatitis, azathioprine, is also associated with the development of many other cancers, predominantly lymphomas. The strongest association is seen for post-transplant lymphoma and hepatosplenic T-cell lymphoma in Crohn's disease and ulcerative colitis patients; there is also an association with a variety of cutaneous malignancies. A relationship between azathioprine and sarcoma has not been demonstrated, though there have been sporadic case reports...

A 49-year-old man was hospitalized with infectious mononucleosis-like symptoms, hepatosplenomegaly, and circulating abnormal lymphocytes. Hepatosplenic T-cell lymphoma was suspected, and bone marrow aspiration and liver biopsy were performed. The pathological diagnosis was T-cell lymphoma. When explaining malignant lymphoma to the patient, he disclosed his Men Who Have Sex with Men (MSM) status. HIV RNA test showed a markedly high viral load, and TCR gene rearrangement was negative. In view of these results, the patient was diagnosed with acute HIV infection...

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PURPOSE OF REVIEW: With the advent of biologic therapies for the treatment of inflammatory bowel disease, the roles of thiopurines have continued to evolve. This review will focus on recent advances in pharmacology and the safety and efficacy of thiopurines as maintenance therapies for steroid-induced remissions and post-surgical maintenance of remission and as combination therapies to reduce immunogenicities of biologic agents. RECENT FINDINGS: Due to pharmacogenetics of thiopurine S-methyltransferase, thiopurine dosing is more effectively based on monitoring of thiopurine metabolites rather than weight-based dosing...

Hepatosplenic T-cell lymphomas (TCLs) are a rare, aggressive subset of TCLs, accounting for less than 5% of all peripheral T-cell and natural killer (NK) cell lymphomas. We report the case of a CD3 negative hepatosplenic T-cell lymphoma in a 42-year-old female, who presented with left-sided abdominal pain. She underwent a liver biopsy that showed marked abnormal sinusoidal lymphoid infiltration. PET scan revealed increased splenic and pharyngeal lymph node uptake. Immunophenotype was remarkable for negative CD3, gamma delta T-cell receptor, and alpha beta-T-cell receptor expression...

Hepatosplenic T-cell lymphoma (HSTCL) is an extremely rare and aggressive form of non-Hodgkin lymphoma associated with poor response to treatment and high mortality. There is an increased incidence among patients with inflammatory bowel disease, especially young male patients under 35 years old and on combination therapy (thiopurine and anti-TNF-α). We describe a case of HSTCL in a young male patient with stenosing ileal Crohn's disease on azathioprine monotherapy for 4.8 years admitted to our hospital with intra- abdominal sepsis...

Canada has among the highest rates of childhood-onset IBD in the world. Over 7000 children and youth under 18 years old are living with IBD in Canada, and 600 to 650 children under 16 years old are diagnosed annually. While the peak age of onset of IBD is highest in the second and third decades of life, over the past two decades incidence has risen most rapidly in children under 5 years old. The treatment of children with IBD presents important challenges including therapeutic choices, risk of adverse events to medications, psychosocial impact on the child and family, increased cost of health care and the implications of the transition from pediatric to adult care...

Ataxia-telangiectasia is a rare autosomal recessive neurodegenerative disease characterized by ataxia, radiosensitivity, telangiectases, and increased risk for hematologic malignancies. We present a case of a female individual diagnosed with T-cell acute lymphocytic leukemia at 13 years and subsequently with αβ subtype of hepatosplenic T-cell lymphoma (HSTCL) at 20 years. During her diagnostic work up for HSTCL, paired tumor-germline sequencing identified a diagnosis of ataxia-telangiectasia. We also describe a very refractory clinical course of her αβ HSTCL, including only a brief response to multiagent chemotherapy and an allogenic bone marrow transplant...

Other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs) with a T- or NK-cell phenotype are markedly rare, with only a limited number of cases having been reported thus far. Methotrexate (MTX) is the most common agent used for OIIA-LPD patients, and 43 cases of MTX-associated T-LPDs (MTX T-LPDs) and five cases of MTX-associated NK/T-LPDs (MTX NK-LPDs) have been described. In addition to MTX T-LPDs and MTX NK/T-LPDs, T-LPD and NK/T-LPDs have been reported in patients receiving other immunosuppressive agents such as thiopurines, TNF antagonists, and cyclosporine...

Hepatosplenic γ-δ T-cell lymphoma, an exceptionally uncommon subtype of peripheral T-cell lymphomas, commonly presents with advanced-stage disease manifesting with hepatosplenomegaly, cytopenias, and constitutional symptoms. Management of this subset is challenging as a result of the unique presentation and refractory nature to conventional treatment approaches. There is a lack of consensus guidelines for up-front induction strategies, and the role of consolidative autologous or allogeneic stem-cell transplantation is controversial...

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Recently, we have presented a scheme, termed "NKL-code", which describes physiological expression patterns of NKL homeobox genes in early hematopoiesis and in lymphopoiesis including main stages of T-, B- and NK-cell development. Aberrant activity of these genes underlies the generation of hematological malignancies notably T-cell leukemia. Here, we searched for deregulated NKL homeobox genes in main entities of T-cell lymphomas comprising angioimmunoblastic T-cell lymphoma (AITL), anaplastic large cell lymphoma (ALCL), adult T-cell leukemia/lymphoma (ATLL), hepatosplenic T-cell lymphoma (HSTL), NK/T-cell lymphoma (NKTL) and peripheral T-cell lymphoma (PTCL)...