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Peripheral T-cell Lymphoma (peripheral t-cell + lymphoma)
Selected AbstractsUNSPECIFIED SOLITARY PERIPHERAL T-CELL LYMPHOMA OF THE BREAST IN A TEENAGED GIRLPEDIATRIC DERMATOLOGY, Issue 2 2006Evren Sarifakioglu M.D. No abstract is available for this article. [source] Peripheral T-cell lymphoma associated consecutively with hemophagocytic lymphohistiocytosis and hypereosinophilic syndromeEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 4 2003Antonio Gutiérrez Abstract:, Both hemophagocytic lymphohistiocytosis and hypereosinophilic syndrome have been associated with hematologic neoplasms and are respectively related to an overproduction of the cytokines Thelper 1 (Th1) and Th2 by tumor cells or reactive cells. To our knowledge, this is the first time a case of a peripheral T-cell lymphoma consecutively associated with both paraneoplastic conditions has been reported. Importantly, in this case when the lymphoma exclusively involved the bone marrow, severe paraneoplastic systemic damage, a CD8+ suppressor/cytotoxic phenotype and a hypereosinophilia not related to high levels of interleukin (IL)-5 was found. Interestingly, progression of the lymphoma coincided with an increase in the serum levels of several Th2 cytokines and IL-2, a decrease in tumor necrosis factor and granulocyte-macrophage colony-stimulating factor levels and the onset of a hypereosinophilic syndrome. [source] Peripheral T-cell lymphoma in a five year oldPEDIATRIC BLOOD & CANCER, Issue 1 2008M.W. Beresford PhD Abstract We report a case presenting with persistent pyrexia that led to the diagnosis of peripheral T-cell lymphoma, a rare malignancy in childhood. The case illustrates diagnostic conundrums in a patient who is not responding as expected to treatment. Pediatr Blood Cancer 2008;50:145,147. © 2006 Wiley-Liss, Inc. [source] Peripheral T-cell lymphoma with diffuse pulmonary infiltration and an increase in serum KL-6 levelRESPIROLOGY, Issue 3 2007Tomoyuki FUJISAWA Abstract: Peripheral T-cell lymphoma is a subtype of non-Hodgkin's lymphoma. A case of peripheral T-cell lymphoma showing diffuse pulmonary involvement together with a marked increase in the level of serum KL-6 is presented. CXR and CT revealed reticular and ground-glass opacities, which mimicked interstitial pneumonia. Immunopathological findings and an analysis of T-cell receptor gene rearrangements of the lung biopsy specimen led to a definite diagnosis of peripheral T-cell lymphoma. In addition, the extensive proliferation of type II pneumocytes, which stained strongly positive for anti-KL-6 antibody suggested that the pneumocytes were the source of serum KL-6. [source] Peripheral T-cell lymphomas, unspecified (or not otherwise specified): a reviewHEMATOLOGICAL ONCOLOGY, Issue 1 2008Delvys Rodriguez-Abreu Abstract Peripheral T-cell lymphomas (PTCL) comprises a heterogeneous group of haematological tumours, which originate from mature T-cells, and constitute less than 15% of all non-Hodgkin's lymphomas (NHLs) in adults. The current WHO classification recognizes nine distinct clinicopathologic peripheral T-cell NHLs, being the ,unspecified variant' (PTCL-U) the most common subtype. These neoplasms often present in advanced stage at diagnosis, and most commonly have an aggressive clinical course requiring prompt treatment. The rarity of these tumours requires additional studies to better understand their biology and search for new therapies which may hopefully improve the dismal outcome of most patients. This review aims to describe the pathobiological aspects as well the clinical characteristics and current therapeutic strategies of the PTCLs, with special attention to the group of PTCL-U Copyright © 2007 John Wiley & Sons, Ltd. [source] Peripheral T-cell lymphoma gene expression profilesHEMATOLOGICAL ONCOLOGY, Issue 3 2006B. Martinez-Delgado Abstract Expression profiling using DNA microarrays has been very helpful to improve our knowledge of the pathobiology of many tumour types, including lymphomas. Peripheral T-cell lymphomas (PTCL) constitute an heterogeneous group of tumours with different morphologic, immunophenotypic, and clinical characteristics. Their complexity and their low frequency in the western countries have made difficult the identification of molecular events responsible of the development of these tumours. The first studies on expression profiling of PTCL have also revealed heterogeneity at this level, mainly regarding the PTCL NOS subgroup. Different molecular subgroups within PTCL unspecified have been identified associated to different expression profiles. However, the clinical significance of this molecular sub-classification remains to be probed in studies involving larger number of samples. In addition, the expression level of NF-kB pathway genes allowed to differentiate two PTCL subgroups, and this difference could have clinical interest. In general, PTCL expression profiles are difficult to interpret due to the significant proportion of other infiltrating cells accompanying the tumour. However, microarrays are being a helpful tool in the initial task of dissecting the PTCL expression profile. Copyright © 2006 John Wiley & Sons, Ltd. [source] Non-mycosis fungoides cutaneous T-cell lymphoma: reclassification according to the WHO-EORTC classificationJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2010Joshua Weaver Background: Non-mycosis fungoides (non-MF) primary cutaneous T-cell lymphomas (PCTCL) are heterogeneous and divided into subgroups by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification of cutaneous lymphomas. We report the first North American series to examine the applicability of the classification, compare our findings with the predominant European literature and confirm the significance of separation into the indolent and aggressive groups. Methods: Forty-four non-MF PCTCL cases with available tissue for phenotyping, adequate clinical staging information and follow-up were reclassified according to the WHO-EORTC classification. Results: Non-MF PCTCL had a longer overall survival (OS) (13.8 years) compared with secondary cutaneous T-cell lymphoma (SC-TCL) (2.5 years). Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) had the most favorable outcome (OS 14.1 years), whereas secondary and primary peripheral T-cell lymphoma, unspecified had the shortest OS (2.5 and 2.4 years, respectively). Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (CTLCD4) appeared to have a favorable course. Conclusions: Most non-MF PCTCL can be classified according to the WHO-EORTC classification. The relative frequencies are similar to European experience. Non-MF PCTCL is a heterogeneous group with a favorable outcome compared to SC-TCL, especially PC-ALCL and CTLCD4. Separation of non-MF PCTCL into indolent and aggressive groups appears clinically significant and may provide direction for therapeutic decisions. Weaver J, Mahindra AK, Pohlman B, Jin T, His ED. Non-mycosis fungoides cutaneous T-cell lymphoma: reclassification according to the WHO-EORTC classification. [source] Unusual manifestation of histiocyte-rich peripheral T-cell lymphomaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 10 2009Stephen E. Mercer MD [source] CNS lymphomatoid granulomatosis with lymph node and bone marrow involvementsNEUROPATHOLOGY, Issue 6 2008Akihiro Takiyama Lymphomatoid granulomatosis (LYG) in the CNS is an uncommon lymphoproliferative disease with characteristic angiocentric lymphoreticular proliferative and granulomatous lesions exhibiting low-grade malignant potential. Here we report a rare case of CNS-LYG, which disseminated to the lymph node and bone marrow. A 50-year-old man was diagnosed with CNS-LYG based on brain biopsy showing perivascular infiltration of CD3-positive small T-lymphocytes without overt nuclear atypism. Eight months after the initial neurological symptoms, inguinal lymph node swelling was found and histopathologically diagnosed as peripheral T-cell lymphoma. TCR,-gene rearrangement study using both paraffin-embedded specimens of brain and inguinal lymph node demonstrated an identical clonal band. Considering the clinical course, we concluded lymph node involvement of CNS-LYG, suggesting the malignant potential of CNS-LYG. [source] Comparative analysis of NK/T-cell lymphoma and peripheral T-cell lymphoma in Korea: Clinicopathological correlations and analysis of EBV strain type and 30-bp deletion variant LMP1PATHOLOGY INTERNATIONAL, Issue 11 2003Ji Eun Kim Natural killer/T-cell lymphoma (NKTL) and peripheral T-cell lymphomas (PTCL) are prevalent in the Asian population and exhibit a high association with the Epstein,Barr virus (EBV). Moreover, differentiation of these two groups is often difficult and problematic. We investigated 35 cases of NKTL (22 nasal cases and 13 extranasal cases) and 30 cases of PTCL in terms of their clinical features, immunohistology, EBV positivity, EBV strain-type polymorphism and latent membrane protein 1 (LMP1) deletion variant distribution. Eighteen cases (82%) of nasal NKTL and seven (54%) of extranasal NKTL showed EBV positivity by EBV in situ hybridization. Fifteen cases (50%) of PTCL revealed EBV positivity. EBV strain type A was predominant in NKTL (18:5), and EBV strain types A and B were distributed evenly in PTCL (6:6). EBV-positive patients had significantly shorter survival than EBV-negative patients (P < 0.05), and EBV positivity correlated with advanced clinical stage (P < 0.05). Patients harboring type A EBV showed slightly poorer prognoses than those having type B, though it was not obviously statistically different (P = 0.07). The LMP1 deletion variant was prevalent in both NKTL (three wild-type LMP1, 15 deletion variants) and PTCL (three wild-type LMP1, eight deletion variants, two coexistent forms) patients, but did not have prognostic impact. Our results indicate that EBV acts as a negative prognostic factor in NKTL and PTCL, and that the intrinsic properties of a specific viral strain might influence the clinical behavior of these diseases. [source] The World Health Organization classification of malignant lymphoma: Incidence and clinical prognosis in HTLV-1-endemic area of FukuokaPATHOLOGY INTERNATIONAL, Issue 1 2002Koichi Ohshima New insights into the pathogenesis of lymphoid malignancies have been gained through novel genetic, molecular and immunological techniques. A new classification system for lymphoid malignancies, known as the new World Health Organization (WHO) classification, has been proposed recently based on these findings. The relative incidence of the subtypes of malignant lymphoma is known to differ according to geographic location. Adult T-cell leukemia/lymphoma (ATLL) is a human malignancy associated with human T-cell leukemia virus type 1 (HTLV-1), and the Kyushu islands are an HTLV-1 endemic area. To clarify the relationship between the histological classification and prognosis of lymphoid malignancies, we reclassified previous cases in our department and summarized our previous reports using the WHO classification. Of 933 cases of lymphoid malignancies, 471 (50%) were B-cell lymphoma, 396 (42%) T/natural killer (NK)-cell lymphoma and 41 (4%) Hodgkin lymphoma (HL). Analysis of clinical outcome showed favorable prognosis for HL, intermediate for B-cell lymphoma and poor prognosis for T-cell lymphoma. Among B-cell lymphomas, the commonest type was diffuse large B-cell lymphoma (n = 281; 60%). Marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) was diagnosed in 82 cases (17%), follicular lymphoma in 52 (11%) and mantle cell lymphoma in 24 (5%). Other less common lymphomas were Burkitt lymphoma (n= 9; 2%) and lymphoblastic lymphoma (n = 5; 1%). Using overall survival rates, the various B-cell lymphoma types could be divided into three broad groups for prognostic purposes: (i) low-risk group comprising follicular lymphoma and MALT; (ii) intermediate-risk group comprising diffuse large B-cell lymphoma and Burkitt lymphoma; and (iii) high-risk group comprising mantle cell lymphoma and lymphoblastic lymphoma. Among the T/NK-cell lymphomas, the commonest type was ATLL (n = 191; 48%), followed by peripheral T-cell lymphoma, unspecified (n = 83; 21%), angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) (n = 38; 10%), anaplastic large cell lymphoma (ALCL) (n = 22; 6%). Less common types were lymphoblastic lymphoma (n = 17; 4%), nasal and nasal-type NK/T-cell lymphoma (n = 17; 4%), mycosis fungoides (MF) (n = 9; 2%) and other rare types. With respect to clinical prognosis, T/NK-cell lymphomas fell into three groups: (i) relative low-risk group comprising ALCL, AILD, MF and lymphoblastic lymphoma; (ii) relative intermediate-risk group comprising NK/T-cell lymphoma and unspecified lymphoma; and (iii) extremely high-risk group comprising ATLL. Among the lymphoblastic lymphomas, B-cell type and T-cell type lymphomas exhibited different clinical outcomes. We conclude that the histological, phenotypic and genotypic classification of the new WHO system should be beneficial for the clinical approach to these tumors. [source] Peripheral T-cell lymphoma in a five year oldPEDIATRIC BLOOD & CANCER, Issue 1 2008M.W. Beresford PhD Abstract We report a case presenting with persistent pyrexia that led to the diagnosis of peripheral T-cell lymphoma, a rare malignancy in childhood. The case illustrates diagnostic conundrums in a patient who is not responding as expected to treatment. Pediatr Blood Cancer 2008;50:145,147. © 2006 Wiley-Liss, Inc. [source] Cytogenetic and molecular characterization of a patient with simultaneous B-cell chronic lymphocytic leukemia and peripheral T-cell lymphomaAMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2001J. Ignacio Martin-Subero Abstract A patient is described who developed a peripheral T-cell lymphoma (PTCL) after a 6-year history of B-cell chronic lymphocytic leukemia (B-CLL). The progression of the T-cell disease spreading to pleura and skin terminated the course of the disease. A cytogenetic analysis performed six years after the first onset of the B-CLL showed the presence of two clones, one with trisomy 12 and another with inv(14)(q11q32.1) and trisomy 8. Combined immunophenotyping and fluorescence in situ hybridization demonstrated that only CD19+ cells contained a trisomy 12, whereas CD3+ cells contained a trisomy 8. Analyses of IgH and TCR rearrangements in single micromanipulated B- and T-cells lacked evidence for a clonal relation between B-CLL and PTCL cells. Based on our findings, we discuss the different hypotheses which might explain the development of simultaneous PTCL and B-CLL. Am. J. Hematol. 68:276,279, 2001. © 2001 Wiley-Liss, Inc. [source] Peripheral T-cell lymphoma with diffuse pulmonary infiltration and an increase in serum KL-6 levelRESPIROLOGY, Issue 3 2007Tomoyuki FUJISAWA Abstract: Peripheral T-cell lymphoma is a subtype of non-Hodgkin's lymphoma. A case of peripheral T-cell lymphoma showing diffuse pulmonary involvement together with a marked increase in the level of serum KL-6 is presented. CXR and CT revealed reticular and ground-glass opacities, which mimicked interstitial pneumonia. Immunopathological findings and an analysis of T-cell receptor gene rearrangements of the lung biopsy specimen led to a definite diagnosis of peripheral T-cell lymphoma. In addition, the extensive proliferation of type II pneumocytes, which stained strongly positive for anti-KL-6 antibody suggested that the pneumocytes were the source of serum KL-6. [source] Epstein-Barr virus-related lymph node lesion resembling autoimmune disease-like clinicopathological findings in elderly patients Report of three casesAPMIS, Issue 12 2003MASARU KOJIMA Three cases of Epstein-Barr virus (EBV)-related lymphoproliferative disorders in elderly patients showing autoimmune disease-associated lymphadenopathy-like clinicopathological findings have been reported. Clinically, they were characterized by systemic lymphadenopathy, "B" symptoms, polyclonal hypergammaglobulinemia, elevated serum LDH and transient presence of various autoantibodies, and absence of atypical lymphocytosis in peripheral blood. One case was associated with idiopathic thrombocytopenic purpura. The clinical course was self-limiting. Histologically, they exhibited numerous lymphoid follicles with hyperplastic germinal centers and atypical interfollicular widening with prominent vascular proliferation. In the paracortical area, there was a mixed infiltrate comprising small to medium-sized lymphocytes and plasma cells, and variable numbers of eosinophils and T- and B-immunoblasts. In situ hybridization demonstrated a varying number of EBV-infected lymphocytes in the germinal center as well as in the interfollicular area. Polymerase chain reaction demonstrated that neither clonal rearrangement of T-cell receptor ,-gene nor immunoglobulin heavy-chain rearrangement was detected in two of the cases examined. Although acute EBV infection rarely occurs in older adults, EBV related to reactive lymphoproliferative disorder should be added to the differential diagnosis of autoimmune disease-associated lymphadenopathy and node-based peripheral T-cell lymphoma in elderly patients. [source] Acute viral lymphadenitis mimicking low-grade peripheral T-cell lymphoma A clinicopathological study of nine cases,APMIS, Issue 6 2001Masaru Kojima Acute viral lymphadenitis, especially infectious mononucleosis (IM), often shows the presence of Reed-Sternberg-like cells, resulting in confusion with Hodgkin's disease. However, acute viral lymphadenitis requiring differential diagnosis from non-Hodgkin's lymphoma is not widely recognized. We describe the clinicopathological and immunohistochemical features of lymph node lesions from nine such patients which pose serious problems of differential diagnosis from low-grade peripheral T-cell lymphoma. There were three males and six females with ages ranging from 21 to 44 years (median 25 years). All patients had "B" symptoms and multicentric lymphadenopathy. The clinical course was also self-limiting. Each lymph node specimen showed an obvious expansion of an interfollicular area by pleomorphic and polymorphous infiltration with an increased number of arborizing postcapillary venules. The infiltrate was composed of variable numbers of small and medium-sized lymphocytes, immunoblasts, plasma cells in various stage of maturation and occasional granulocytes. The small lymphocytes usually had regular round nuclei, whereas the medium-sized lymphocytes occasionally showed nuclear pleomorphism. Hyperreactivity of B-lymphocytes, including hyperplastic germinal centers and/or foci of monocytoid B-cells, was seen in parts of the lesion. The majority of the interfollicular T-lymphocytes, including T-immunoblasts, expressed CD8 antigen. Various numbers of TIA-1-positive small and medium-sized T-cells were observed in the paracortical area. Despite these findings, the overall histological picture of this series posed serious difficulties when differentially diagnosing this condition from low-grade peripheral T-cell lymphomas such as angioimmunoblastic T-cell (AILD) and T-zone types, indicating that viral lymphadenitis occasionally presents with histological features of AILD and T-zone lymphomas. To avoid overdiagnosis and overtreatment, we emphasize the need to pay careful attention to the clinical and laboratory findings as well as the morphological features. [source] Toxic epidermal necrolysis secondary to angioimmunoblastic T-cell lymphomaAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 3 2005Brad Jones SUMMARY A 67-year-old man presented with a history of lymphadenopathy, fevers and separate skin eruptions of erythrodermic spongiotic dermatitis initially and subsequent toxic epidermal necrolysis. Initial lymph node biopsies showed non-specific granulomatous changes, and skin biopsies and bone marrow aspirate were not diagnostic. His toxic epidermal necrolysis responded well to 3 days of intravenous immunoglobulin. The patient was discharged from hospital and reviewed regularly as an outpatient. Due to persisting lymphadenopathy, further lymph node biopsy led to the diagnosis of angioimmunoblastic T-cell lymphoma, a rare form of peripheral T-cell lymphoma with a poor prognosis. At the time of diagnosis his condition deteriorated rapidly and he died soon after. [source] Laboratory correlates for a phase II trial of romidepsin in cutaneous and peripheral T-cell lymphomaBRITISH JOURNAL OF HAEMATOLOGY, Issue 2 2010Susan E. Bates Summary Romidepsin has shown promise in the treatment of T-cell lymphomas, and so we evaluated molecular endpoints gathered from 61 patients enrolled on a phase II trial of romidepsin in cutaneous and peripheral T-cell lymphoma at the National Institutes of Health. The endpoints included histone H3 acetylation and ABCB1 gene expression in peripheral blood mononuclear cells (PBMCs); ABCB1 gene expression in tumour biopsy samples; and blood fetal haemoglobin levels (HbF), all of which were increased following romidepsin treatment. The fold increase in histone acetylation in PBMCs at 24 h was weakly to moderately well correlated with the pharmacokinetic parameters Cmax and area under the curve (AUC)last (, = 0·37, P = 0·03 and , = 0·36, P = 0·03 respectively) and inversely associated with clearance (, = ,0·44; P = 0·03). Histone acetylation in PBMCs at 24 h was associated with response (P = 0·026) as was the increase in fetal haemoglobin (P = 0·014); this latter association may be due to the longer on-study duration for patients with disease response. Together, these results suggest that pharmacokinetics may be an important determinant of response to histone deacetylase inhibitors (HDIs) , the association with histone acetylation in PBMCs at 24 h is consistent with a hypothesis that potent HDIs are needed for a critical threshold of drug exposure and durable activity. [source] The International Prognostic Index determines the outcome of patients with nodal mature T-cell lymphomasBRITISH JOURNAL OF HAEMATOLOGY, Issue 3 2005Ruth Sonnen Summary The World Health Organization (WHO) lymphoma classification recognises anaplastic large cell lymphoma (ALCL), angioimmunoblastic lymphoma (AIL) and peripheral T-cell lymphoma, unspecified (PTCU) as nodal mature T-cell lymphomas. Little is known about long-term outcome and prognostic factors of these diseases. A retrospective analysis on 125 patients with ALCL, AIL or PTCU was performed to evaluate outcome parameters, taking into account histological subtype and the International Prognostic Index (IPI). Median age was 54 years (range 17,90 years). Complete remission (CR) was achieved in 51% of patients. Five-year overall survival (OS) was 43%, and 5-year relapse-free survival was 69%. Five-year OS was 61% for ALCL, 45% for PTCU and 28% for AIL. With regard to the IPI, 5-year OS was 74%, 49%, 21% and 6% for the low, low-intermediate, high-intermediate and high risk groups, respectively. In the multivariate analysis, the IPI but not the histological subtype significantly predicted survival. To a large extent, the IPI score explains the differences in survival between histological subtypes of nodal mature T-cell lymphomas. The IPI may therefore be used for risk stratification in clinical trials to identify patients who would benefit most from new treatment strategies, such as high-dose chemotherapy followed by stem cell or bone marrow transplantation. [source] Activity of interferon-, and isotretinoin in patients with advanced, refractory lymphoid malignanciesCANCER, Issue 3 2004Apostolia-Maria Tsimberidou M.D., Ph.D. Abstract BACKGROUND Interferon-, (IFN-,) and retinoids have shown nonoverlapping toxicity and each has shown antitumor activity in patients with lymphoma. The aim of the current study was to assess the toxicity, safety, and efficacy of IFN-, combined with isotretinoin in patients with advanced, refractory lymphoid malignancies. METHODS Adults with biopsy-proven advanced lymphoid malignancy were treated. Patients with compromised bone marrow function (platelet counts as low as 30 × 109/L) were eligible. Treatment was comprised of IFN-, at a starting daily dose of 3 mega units subcutaneously and isotretinoin orally starting at a dose of 1 mg/kg daily in 2 divided doses. RESULTS Forty-four patients were evaluable. Their median age was 57 years (range, 18,82 years). Eighteen patients had advanced cutaneous T-cell lymphoma, 6 patients had peripheral T-cell lymphoma, 14 patients had Hodgkin disease, and 6 patients had a variety of other lymphoid malignancies. Patients with Hodgkin disease had received a median of 6 previous therapies (range, 3,12 therapies) and patients with other lymphoid malignancies had received a median of 4 previous therapies (range, 1,9 therapies). The median duration of treatment was 4 months (range, 0.25,38 months). The overall response rate was 38.6% (complete response in 5 patients [11.3%] and partial response in 12 patients [27.3%]). The median response duration was 3 months (range, 1,95+ months). The most common toxicities were low-grade fever, flu-like symptoms, and fatigue (IFN-, effects); dry mouth and skin and hypertriglyceridemia (cis-retinoic acid effects); and thrombocytopenia (which generally occurred in patients with low baseline platelet counts). CONCLUSIONS IFN-, and isotretinoin combination therapy had antitumor activity and was well tolerated in heavily pretreated patients with lymphoid malignancies. Cancer 2004. © 2003 American Cancer Society. [source] Phase II study of pentostatin in advanced T-cell lymphoid malignanciesCANCER, Issue 2 2004Update of an M. D. Anderson Cancer Center series Abstract BACKGROUND The goal of the current study was to assess the toxicity, safety, and efficacy of pentostatin in patients with T-cell lymphoid malignancies. METHODS Patients were eligible if they had biopsy-proven T-cell lymphoma or leukemia and failure to respond to previous therapy or an expected complete response rate to conventional therapy of < 20%. Pentostatin was administered at an initial dose of 3.75 or 5.0 mg/m2 by intravenous bolus daily over a consecutive 3-day period every 3 weeks. RESULTS Forty-two of 44 patients enrolled in the study were evaluable. The median age of the patients was 62 years (range, 38,86 years). Patients received a median of 3 previous therapies (range, 0,10 previous therapies). Of these patients, 32 (76%) had mycosis fungoides/Sézary syndrome and 10 patients (24%) had other T-cell leukemias or lymphomas. The overall response rate was 54.8% (complete remission, 6 patients [14.3%]; partial remission, 17 patients [40.5%]). Durable responses were observed mainly in patients with Sézary syndrome or peripheral T-cell lymphoma. The median follow-up period for surviving patients was 20 months (range, 1,83+ months). The median duration of response was 4.3 months (range, 1,61 months). The most common toxicities were neutropenia, nausea, and CD4 suppression. A transient early ,flare' of disease was observed in some responders. CONCLUSIONS At these doses, pentostatin was reasonably well tolerated and is an effective drug for the treatment of T-cell lymphomas. Cancer 2004;100:342,9. © 2003 American Cancer Society. [source] Phase II study of the irinotecan (CPT-11), mitoxantrone and dexamethasone regimen in elderly patients with relapsed or refractory peripheral T-cell lymphomaCANCER SCIENCE, Issue 1 2007Nozomi Niitsu We treated elderly patients with relapsed or refractory peripheral T-cell lymphoma (PTCL) using a CMD (CPT-11, mitoxantrone [MIT], dexamethasone [DEX]) regimen and studied its safety and efficacy. The subjects were 70,79-year-old patients with relapsed or refractory PTCL. CPT-11 at 25 mg/m2 on days 1 and 2, MIT at 8 mg/m2 on day 3, and DEX at 40 mg/day on days 1,3 were administered once every 3 weeks, and this was performed for six cycles. Eleven (37%) of the 30 patients achieved complete remission and seven patients (23%) achieved partial remission. With a median follow-up period of 32 months, the 3-year survival rate was 28.2% and the 3-year progression-free survival rate was 17.5%. The main adverse drug reaction was hematological toxicity and there were no deaths related to the treatment. B-type natriuretic peptide and troponin T levels did not increase after the treatment and none of the patients showed electrocardiogram or echocardiogram abnormalities. Our results indicate that the CMD regimen is safe in elderly patients and no cardiotoxicities developed as a result of this regimen. In addition, it was effective in patients who had previously been treated with doxorubicin and good treatment results were obtained in elderly patients with relapsed PTCL. (Cancer Sci 2007; 98: 109,112) [source] Comparative analysis of NK/T-cell lymphoma and peripheral T-cell lymphoma in Korea: Clinicopathological correlations and analysis of EBV strain type and 30-bp deletion variant LMP1PATHOLOGY INTERNATIONAL, Issue 11 2003Ji Eun Kim Natural killer/T-cell lymphoma (NKTL) and peripheral T-cell lymphomas (PTCL) are prevalent in the Asian population and exhibit a high association with the Epstein,Barr virus (EBV). Moreover, differentiation of these two groups is often difficult and problematic. We investigated 35 cases of NKTL (22 nasal cases and 13 extranasal cases) and 30 cases of PTCL in terms of their clinical features, immunohistology, EBV positivity, EBV strain-type polymorphism and latent membrane protein 1 (LMP1) deletion variant distribution. Eighteen cases (82%) of nasal NKTL and seven (54%) of extranasal NKTL showed EBV positivity by EBV in situ hybridization. Fifteen cases (50%) of PTCL revealed EBV positivity. EBV strain type A was predominant in NKTL (18:5), and EBV strain types A and B were distributed evenly in PTCL (6:6). EBV-positive patients had significantly shorter survival than EBV-negative patients (P < 0.05), and EBV positivity correlated with advanced clinical stage (P < 0.05). Patients harboring type A EBV showed slightly poorer prognoses than those having type B, though it was not obviously statistically different (P = 0.07). The LMP1 deletion variant was prevalent in both NKTL (three wild-type LMP1, 15 deletion variants) and PTCL (three wild-type LMP1, eight deletion variants, two coexistent forms) patients, but did not have prognostic impact. Our results indicate that EBV acts as a negative prognostic factor in NKTL and PTCL, and that the intrinsic properties of a specific viral strain might influence the clinical behavior of these diseases. [source] Differentiating germinal center-derived lymphomas through their cellular microenvironment,AMERICAN JOURNAL OF HEMATOLOGY, Issue 7 2009Antonino Carbone Recent studies on normal and malignant B-cells have provided evidence that the germinal center (GC) of lymphoid follicles exerts a role in B-cell physiology and malignancy. GC-derived lymphomas include both B-cell and T-cell lymphomas. Remarkably, tumor cells of GC-derived lymphomas proliferate in close association with cellular environment that retains key features of normal GC cellular microenvironment. Neoplastic follicles in follicular lymphoma contain, in addition to follicular dendritic cells (FDC) other non-neoplastic cells including macrophages and GC T-cells. In addition to aggregates of FDCs, the background infiltrate of nodular lymphocyte predominant Hodgkin lymphoma includes small B-cells, T-cells, and histiocytes. Typically, most of the lymphocyte predominant (LP) cells are ringed by CD3+/CD4+ T-cells expressing CD57, PD1, BCL6, and MUM1/IRF4. By contrast, Reed,Sternberg cells of classic Hodgkin lymphoma (cHL) are surrounded by CD3+/CD4+ T-cells expressing CD40L. Unlike cHL and other peripheral T-cell lymphomas, the AITL microenvironment characteristically contain a prominent proliferation of high endothelial venules and FDC. Thus, these findings shed new light on the characterization of GC-derived lymphomas and may help in the differential diagnosis and acknowledge several novel pathogenetic mechanisms on these lymphomas. Am. J. Hematol., 2009. © 2009 Wiley-Liss, Inc. [source] Acute viral lymphadenitis mimicking low-grade peripheral T-cell lymphoma A clinicopathological study of nine cases,APMIS, Issue 6 2001Masaru Kojima Acute viral lymphadenitis, especially infectious mononucleosis (IM), often shows the presence of Reed-Sternberg-like cells, resulting in confusion with Hodgkin's disease. However, acute viral lymphadenitis requiring differential diagnosis from non-Hodgkin's lymphoma is not widely recognized. We describe the clinicopathological and immunohistochemical features of lymph node lesions from nine such patients which pose serious problems of differential diagnosis from low-grade peripheral T-cell lymphoma. There were three males and six females with ages ranging from 21 to 44 years (median 25 years). All patients had "B" symptoms and multicentric lymphadenopathy. The clinical course was also self-limiting. Each lymph node specimen showed an obvious expansion of an interfollicular area by pleomorphic and polymorphous infiltration with an increased number of arborizing postcapillary venules. The infiltrate was composed of variable numbers of small and medium-sized lymphocytes, immunoblasts, plasma cells in various stage of maturation and occasional granulocytes. The small lymphocytes usually had regular round nuclei, whereas the medium-sized lymphocytes occasionally showed nuclear pleomorphism. Hyperreactivity of B-lymphocytes, including hyperplastic germinal centers and/or foci of monocytoid B-cells, was seen in parts of the lesion. The majority of the interfollicular T-lymphocytes, including T-immunoblasts, expressed CD8 antigen. Various numbers of TIA-1-positive small and medium-sized T-cells were observed in the paracortical area. Despite these findings, the overall histological picture of this series posed serious difficulties when differentially diagnosing this condition from low-grade peripheral T-cell lymphomas such as angioimmunoblastic T-cell (AILD) and T-zone types, indicating that viral lymphadenitis occasionally presents with histological features of AILD and T-zone lymphomas. To avoid overdiagnosis and overtreatment, we emphasize the need to pay careful attention to the clinical and laboratory findings as well as the morphological features. [source] |