scholarly journals SEROLOGICAL AND MOLECULAR MARKERS OF HERPESVIRUS INFECTIONS IN THE DEBUT OF ANGIOIMMUNNOBLASTIC T-CELL LYMPHOMA

2018 ◽  
Vol 23 (2) ◽  
pp. 77-84
Author(s):  
Dmitriy S. Tikhomirov ◽  
N. G. Chernova ◽  
M. N. Sinitsyna ◽  
Yu. V. Sidorova ◽  
N. G. Yaroslavtseva ◽  
...  
Keyword(s):  
Molecular Markers ◽  
Lymph Node ◽  
T Cell ◽  
Primary Infection ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Herpes Viruses ◽  
High Detection Rate ◽  
Ebv Infection

Background. Angioimmunoblastic T-cell lymphoma (AITL) is the rare lymphoproliferative disorder traditionally thought to be associated with EBV. This original study gives data about serological and molecular markers of herpes viruses in primary AITL patients. Materials and methods. The review includes analysis of clinical and laboratory features of 40 primary AITL patients. Peripheral blood, lymph node, bone marrow and bronco-alveolar aspirate samples were tested by ELISA, PCR and in situ hybridization. Results. Laboratory markers of the acute herpetic infection were detected in 29 (72.0%) out of 40 patients. Primary infection was detected in 8 cases: 6 - primary EBV, 1 - HCMV and 1-EBV and HCMV simultaneously. Anti-HSV 1,2 IgM were observed in 15 (37.5%) patients. EBV small non-coding RNAs (EBER) was positive in 27 (71.1%) out of 38 cases. The comparison of the detection of EBER and markers of acute EBV infection showed good correlation (p<0.001). Patients with EBER-negative lymph node samples (n=11) didn’t have any markers of acute EBV infection. Conversely, 24 of 27 (88.9%) EBER-positive cases accompanied by markers of acute EBV infection: 7 (25.9%) of them held markers of primary infection and 17 (63.0%) - reactivation. A pattern of markers of latent EBV was observed in the rest 3 (11.1%) EBER-positive cases. Conclusion. In primary AITL patients markers of herpetic infections are detected with a high frequency. EBV infection is the most frequent. The high detection rate of IgM HSM 1.2 in primary AITL patients seems to be a characteristic feature of the tumor. Obtained data proved the necessity of laboratory testing for markers of acute herpes viruses, especially in EBER-positive cases.

Peripheral T-cell Lymphoma, NOS With Epstein-Barr Virus Positivity in an Elderly Patient With Myelodysplastic Syndrome: An Autopsy Case Report

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S81-S81
Author(s):  
J Lanceta ◽  
W Xue ◽  
M Hurford ◽  
H Wu
Keyword(s):  
Bone Marrow ◽  
T Cells ◽  
Lymph Node ◽  
T Cell ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Barr Virus ◽  
Epstein Barr

Abstract Casestudy Epstein-Barr virus (EBV)-associated peripheral T-cell lymphomas are a group of aggressive neoplasms with a geographic predilection for South America and Asia, but are very rare in Western populations. Results We report a case of a 74-year-old Caucasian female who presented with pancytopenia and B symptoms with EBV-IgG detected on admission. Past medical history included: ITP, chronic urticaria, and recently diagnosed myelodysplastic syndrome (MDS) on bone marrow biopsy one month prior to admission. Excisional biopsies of an enlarged right neck lymph node (repeated within 6 months) and right axillary lymph node five years ago were negative for a lymphoproliferative disorder at the time. Repeated bone marrow biopsy, performed during the current admission, confirmed the diagnosis of MDS, with scattered T-cells without aberrant immunophenotype. Despite aggressive treatment from multiple specialties, the patient deteriorated and expired four weeks later from complications of MDS. At autopsy, there was diffuse lymphadenopathy involving the mediastinum, axilla, pelvis and peripancreatic fat. Lymph node sections demonstrated nodal architecture effacement by diffuse, vaguely nodular lymphoid infiltrates. Histologically, the infiltrates were composed of medium to large lymphocytes with round to slight irregular nuclei, rare Reed-Sternberg-like multinucleated cells, clumped chromatin, and indistinct nucleoli. Individual cell necrosis was abundant with mitotic figures readily identifiable. Immunohistochemistry revealed CD2+ CD3+ neoplastic T-cells that co-express MUM1 and a subset of CD30, while negative for CD4, CD5, CD8, CD56, ALK1, and TDT. EBV-encoded RNA in-situ hybridization was focally positive. The final postmortem diagnosis was peripheral T-cell lymphoma, not otherwise specified (NOS), with focal EBV positivity. Conclusion Co-existence of a de-novo MDS and non-Hodgkin lymphoma without any prior chemotherapeutic exposure is a highly unusual finding, although MDS-like presentations can occur with EBV-associated lymphomas. Peripheral T-cell lymphoma, NOS is an aggressive lymphoma and EBV positivity has been found correlated with a poor prognosis. This case demonstrates how postmortem examination remains an important tool in clinical- pathological correlation and highlights the potential pathogenetic role EBV plays in MDS and T-cell lymphoma.

scholarly journals Marked paraneoplastic basophilia accompanying eosinophilia in a cat with alimentary T-cell lymphoma

2017 ◽  
Vol 3 (2) ◽  
pp. 205511691773018
Author(s):  
Maria Balan ◽  
Aimee Hope ◽  
Joseph Cassidy ◽  
Maureen McCullough ◽  
Peter J O’Brien
Keyword(s):  
Lymph Node ◽  
T Cell ◽  
Cell Lymphoma ◽  
Severe Case ◽  
Corticosteroid Treatment ◽  
Needle Aspiration ◽  
T Cell Lymphoma ◽  
Cell Marker ◽  
Mesenteric Lymph Nodes ◽  
Peripheral Lymph

Case summary A 5-year-old male neutered domestic shorthair cat was referred with a history of persistent pyrexia, pica, soft faeces, inappetence, intermittent vomiting, mild-to-moderate granulocytosis and mild hypercalcaemia. No significant improvement was noted after antibiotic and corticosteroid treatment, except that the hypercalcaemia resolved. Physical examination, including thoracic auscultation, and abdominal and peripheral lymph node palpation, were unremarkable. On admission, haematology revealed moderate leukocytosis (36.8 × 109/l) with moderate-to-marked eosinophilia (21.3 × 109/l) and marked basophilia (4.04 × 109/l), the latter identified microscopically. Lymphocytes were markedly decreased (0.37 × 109/l). Blood smear examination revealed 58% eosinophils, 28% neutrophils, 11% basophils, 2% monocytes, 1% lymphocytes and marked, diffuse platelet clumping. Biochemistry abnormalities indicated mild pancreatitis, dehydration and anorexia with mildly increased pancreatic lipase, mild hypernatraemia (157 mmol/l), a moderate decrease in urea (3.1 mmol/l) and a slight decrease in phosphate (1.32 mmol/l). Ultrasound and radiographic imaging revealed enlargement of the mesenteric lymph nodes. Fine-needle aspiration, a Tru-cut biopsy and immunohistochemistry were performed. Cytological examination revealed ~65–75% lymphocytes (~80% were larger than a neutrophil), ~25–35% eosinophils and occasional basophils. Lymphocytes had single, small (<1/3 red blood cells), prominent nucleoli and increased pale, mildly vacuolated cytoplasm. On histopathology, cells were monomorphic, large, with prominent nucleoli, and mild, multifocal, staining for T-cell marker CD3. Smaller cells were strongly CD3-positive. Cells were negative for B-cell marker CD45R. Relevance and novel information This is the most severe case of paraneoplastic basophilia reported with feline alimentary T-cell lymphoma with accompanying eosinophilia and lymph node infiltration. Feline basophil prevalence is reported for the first time.

Sinonasal T-cell Lymphoma and Wegener's Granulomatosis: Aspects in Early Differential Diagnosis

1996 ◽  
Vol 10 (4) ◽  
pp. 239-246
Author(s):  
Anders Cervin ◽  
Michael Dictor ◽  
Olof Kalm
Keyword(s):  
Differential Diagnosis ◽  
In Situ Hybridization ◽  
T Cell ◽  
Wegener’S Granulomatosis ◽  
Cell Lymphoma ◽  
Wegener's Granulomatosis ◽  
T Cell Lymphoma ◽  
Upper Airways ◽  
Gastrointestinal Complications

The clinical course of 12 patients with sinonasal T-cell lymphoma retrospectively diagnosed using in situ hybridization for Epstein-Barr virus RNA was compared with that of 10 recently treated patients with Wegener's granulomatosis (WG) in the upper airways. In particular, we studied the presenting signs and symptoms of both diseases, which commonly offer a problem in differential diagnosis at the clinical and pathological level. A bimodal age distribution was suggested in both T-cell lymphoma and WG; five patients with T-cell lymphoma developed disease prior to 40 years of age. Four of the 12 lymphoma patients had a history of “chronic rhinitis” for several years before developing mucosal ulcerations, which were initially unilateral, as opposed to the bilateral ulcerations in early sinonasal WG. Two lymphoma patients had swelling of the nasal dorsum and cheek. In contrast to the WG patients, cases of T-cell lymphoma did not exhibit associated clinical signs of arthritis, conjunctivitis, pulmonary lesions, or nephritis in the early stage of the disease. Nine of the patients with T-cell lymphoma presenting as a sinonasal lesion developed disseminated disease, variably including infiltrates in intestine, lung, CNS, and skin. Four of these patients died from gastrointestinal complications of their disease. We conclude that unilateral ulcerative or hemorrhagic polypoid mucosal lesions in the sinonasal area are suggestive of lymphoma rather than WG, and nonspecific symptoms, at least in Western patients, may be present as early as the second or third decade of life. A biopsy specimen containing T lymphocytes positive for the EBV ribonucleoprotein EBER1 on in situ hybridization offers reliable confirmation of T-cell lymphoma and is of differential diagnostic value against WG.

scholarly journals A Clinically-Indolent Variant of Extranodal NK/T Cell Lymphoma with Unique Immunophenotypic Profile and Superior Outcome

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5278-5278
Author(s):  
Fabiola Valvert ◽  
Elizabeth Solorzano ◽  
Edward Briercheck ◽  
Marcos Mauricio Siliézar Tala ◽  
Yasodha Natkunam ◽  
...  
Keyword(s):  
T Cell ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Elevated Serum ◽  
T Cell Lymphoma ◽  
Nasal Type ◽  
Aggressive Disease ◽  
Ebv Infection ◽  
Nk T Cell ◽  
Indolent Disease

Introduction Extranodal NK/T-cell lymphoma, nasal type (ENKTL), is the most frequent NK-cell malignancy. It is typically associated with a highly aggressive course and extensive local tissue destruction. ENKTL, nasal type, is most common among East Asians and indigenous persons in Latin America, which may result from genetic predisposition, shared strains of EBV infection or other factors. We noted that a subset of patients with ENKTL in Guatemala present with more indolent disease. The clinical and histologic features of these indolent cases, including outcomes after treatment, have not been defined. Methods We reviewed clinical data from 68 patients with ENKTL at INCAN, the largest public cancer hospital in Guatemala, who underwent evaluation between 2006-2018. We confirmed the diagnosis of ENKTL using available paraffin-embedded biopsies based on immunohistochemistry and in situ hybridization for 46 markers at Stanford University (O.S., Y.N.). We defined indolent cases as those lacking macroscopic necrosis, palate perforation, distant lesions (i.e. Stage II or greater), hemophagocytic lymphohistiocytosis (HLH) and B symptoms. Aggressive cases had one or more of these characteristics. Statistical analysis on categorical data was performed using Fisher's exact test. Results Fifty-three patients were confirmed to have ENKTL. The median age at the time of diagnosis was 43 years (range: 11-83) and 36 patients were male (68%). 75.7% of patients self-identified as Mayan ancestry and 85% were born or lived in central or western Guatemala. As outlined in the Table, 14 cases were classified as indolent and 39 were aggressive. Patients with indolent NKTCL were older (mean, 51 years vs. 41.5 years in the aggressive group; p=0.04). Patients with aggressive disease more commonly had anemia, lymphocytopenia and elevated serum LDH. Both indolent and aggressive cases typically had NK cell immunophenotype, including positivity for CD56, granzyme, perforin and TIA-1. All 53 NKTCLs expressed EBER, consistent with EBV infection, with a subset in each group also expressing EBV LMP1. In contrast, greater than 40% of aggressive cases expressed CXCL13 compared to 0% of indolent cases (p=0.005). Aggressive cases were more commonly BCL2 positive (67% versus 31%, p = 0.048). A subset of aggressive cases had Ki67 >50% (6/39 versus 0/14 indolent cases) but there were also aggressive cases with Ki67 <10%. A multiple correspondence analysis using 14 clinical and 18 IHC markers was performed on 33 patients with complete data available. Variables contributing to categorization of aggressive versus indolent ENKTL included palate perforation, peripheral blood lymphocyte count < 0.8 K/uL, B symptoms, anemia, cachexia and macroscopic necrosis. Median survival was markedly better for patients with indolent disease compared to those with aggressive disease (median not reached vs. 2 years, p<0.05). Twelve of (92.9%) thirteen treated patients in the indolent group achieved a complete response compared to only 8 (40%) of 22 treated for aggressive disease (p=0.04). In fact, 9 patients with aggressive disease died before receiving treatment compared to 0 with indolent disease (23.0% vs. 0%; p=0.04). Three of the deaths in patients with indolent disease were due to toxicity from chemotherapy (infection, pancytopenia). Conclusion Approximately one-quarter of patients with extranodal NK/T cell lymphoma, nasal type, in our cohort have a unique variant associated with the absence of aggressive clinical features. These patients have a more indolent clinical course, better outcome with treatment, have less frequent expression of BCL2, and lack CXCL13 expression. Patients with the indolent variant may benefit from less aggressive therapeutic approaches to minimize unnecessary treatment-associated toxicity. Efforts to define genetic and transcriptional characteristics of these cases are underway. Table Disclosures Weinstock: Celgene: Research Funding.

scholarly journals T-cell lymphoma and the Chediak-Higashi syndrome

Blood ◽  
1982 ◽  
Vol 60 (3) ◽  
pp. 672-676 ◽  
Author(s):  
JC Argyle ◽  
CR Kjeldsberg ◽  
J Marty ◽  
AO Shigeoka ◽  
HR Hill
Keyword(s):  
Lymph Node ◽  
T Cell ◽  
Lymphoproliferative Disorder ◽  
Cellular Proliferation ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Cellular Infiltrate ◽  
Chediak Higashi Syndrome ◽  
First Time ◽  
Histopathologic Features

Abstract The majority of patients with Chediak-Higashi syndrome (CHS) develop a lymphoproliferative disorder during the accelerated phase of the disease. Controversy exists regarding the benign versus malignant nature of this cellular proliferation. For the first time, we have characterized the immunologic cell markers on the cellular infiltrate in a lymph node from a patient with CHS. The infiltrate was composed almost entirely of T cells, with histopathologic features consistent with a non-Hodgkin's T-cell lymphoma.

CD4+/bcl-6+ Peripheral T-Cell Lymphoma with Follicular Involvement: A Distinct Type of Nodal Peripheral T-Cell Lymphoma with Multiple T-Cell Clones.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1374-1374
Author(s):  
Ida Munster-Ikonomou ◽  
Anne Tierens ◽  
Gunhild Troen ◽  
Hege Aamodt ◽  
Sverre Heim ◽  
...  
Keyword(s):  
T Cells ◽  
Lymph Node ◽  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Lymphoma Cells ◽  
Peripheral T Cell Lymphoma ◽  
Lymphoid Follicles ◽  
B Lymphoid ◽  
Lymphoma Type

Abstract We studied six cases of a novel type of nodal peripheral T-cell lymphoma. Three cases with this disease were recently described by de Leval et al. (de Leval L, Savilo E, Longtine J, et al. Peripheral T-cell lymphoma with follicular involvement and a CD4+/bcl-6+ phenotype. Am J Surg Pathol2001;25:395–400). The entity was named peripheral T-cell lymphoma with follicular involvement because of its distinctive histological features. We report an additional six well-characterized cases and describe the molecular and cytogenetic findings. The neoplastic T-cells of this lymphoma type express CD4 and Bcl-6, and home to the B-lymphoid follicles. This suggests an origin of the lymphoma from an as yet poorly characterized subset of Bcl-6 expressing intra-follicular T-helper cells. Of interest, the cytogenetic data and/or the study of T-cell receptor gamma gene rearrangements revealed more than one clone in each case. Cytogenetics further revealed complex karyotypes without recurrent chromosomal aberrations. We also studied the presence of somatic mutations in the 5′ untranslated region of the BCL-6 gene in four of the cases but no somatic hypermutation was detected. Clinically, the cases presented with widespread lymph node involvement at diagnosis and multiple relapses occurred after treatment. All patients received a CHOP-based chemotherapy regimen, later followed by high dose chemotherapy with stem cell rescue in five patients. One patient died with lymphoma and hemophagocytic syndrome 24 months after diagnosis, one patient is alive with disease after 27 months from diagnosis, whereas the other four patients are in complete remission 12 to 124 months after diagnosis. In conclusion, we confirm that peripheral T-cell lymphoma with follicular involvement is a distinct lymphoma type and we show that the lymphoma is oligo-clonal. The clinical findings are those of an intermediately aggressive lymphoma type. Although minimal lymph node infiltration with lymphoma cells at diagnosis and oligo-clonality is also characteristic of angio-immunoblastic T-cell lymphoma, we believe that peripheral T-cell lymphoma with follicular involvement is a distinct T-cell lymphoma type. In contrast to angio-immunoblastic T-cell lymphoma it is characterized by the typical infiltration of lymphoma cells in B-lymphoid follicles, coagulation necrosis, the absence of proliferation of high endothelial venules and follicular dendritic cells in T-cell areas, as well as the absence of EBV infection. It is likely that T-cell lymphoma with follicular involvement arises from Bcl-6+ intra-follicular T-cells. No recurrent genetic defects have been identified but the oligo-clonal nature of the lymphoma is intriguing. The latter suggests that the triggering oncogenic factors are external, such as infection.

FDG-PET Scans as a Staging Study for T-Cell Lymphomas: High Rates of Positivity Do Not Result in Frequent Changes in Stage.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2399-2399 ◽  
Author(s):  
Steven M. Horwitz ◽  
Francine Foss ◽  
Shari Goldfarb ◽  
Ana Molina ◽  
Paul A. Hamlin ◽  
...  
Keyword(s):  
Lymph Node ◽  
T Cell ◽  
Anaplastic Large Cell Lymphoma ◽  
Fdg Pet ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Large Cell Lymphoma ◽  
Pet Scans

Abstract FDG-PET is emerging as a standard staging study for diffuse large B-cell lymphoma and Hodgkin’s disease. There is little experience with PET in staging T-cell lymphomas (TCL). TCL often involve extranodal sites not well imaged by standard CT. We hypothesized that PET would add to staging by detecting additional sites of disease. To address this question we reviewed our TCL database to identify patients (pt) who had PET as part of complete staging at initial diagnosis or at relapse. Each pt was included once. Staging included physical exam, CT scan of the chest/abdomen/pelvis alone or as part of a PET/CT combination, and bone marrow (BM) biopsy. Studies such as MRI of the sinus or ultrasound of the testes were done as clinically indicated. We reviewed 107 pt who met the above criteria. Histologies were as follows: peripheral T-cell lymphoma NOS (PTCL), angioimmunoblastic T-cell lymphoma (AILT), anaplastic large cell lymphoma, ALK-1-, (ALCL−), anaplastic large cell lymphoma, ALK-1+, (ALCL+), mycosis fungoides (MF), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), adult T-cell lymphoma (ATL), NK lymphoma nasal type (NK-Nas), lymphoblastic lymphoma (LL), enteropathy associated T-cell lymphoma (EATCL), blastic NK lymphoma (BLNK), primary cutaneous ALCL (ALCL-Cut), and hepatosplenic T-cell lymphoma (HSPTCL). All pt with MF had suspicion of extracutaneous disease. Overall 95/107 (89%) had a PET interpreted as positive by visual review. Standard uptake values (SUV) varied from 1.1–20.5 g/ml. Of the 12 pt with negative PET scans, 7(58%) had no evidence of disease on CT including PTCL (n=3, stage I resected), HSPTCL (n=2, liver, spleen, BM disease only), ALCL-Cut (n=2, skin only). T-cell Lymphoma-PET Results Histology N PET + % positive SUV Range (g/ml) ALL PT 107 95 89% 1.1–20.5 PTCL 27 24 88% 2–20 AILT 19 16 84% 2–11.7 ALCL− 12 12 100% 3–19.6 ALCL+ 4 4 100% 4–12 MF 12 10 83% 1.8–17.6 SPTCL 8 8 100% 1.4–13.1 ATL 5 5 100% 2.9–19.7 NK-Nas 5 5 100% 3.4–13.1 LL 3 3 100% 5.5–20.5 EATCL 3 3 100% 3.5–9.9 ALCL-Cut 5 3 60% 1.1–1.4 BLNK 2 2 100% 1.929.5 HSPTCL 2 0 0 N/A PET detected additional sites of disease in 34/107 (32%). These sites detected by PET were skin/subcut (n=9), bone (n=7), lymph node (n=6), spleen (n=3), nasopharynx/sinus (n=2), liver (n=2), BM (n=1), bowel (n=1), muscle (n=1), kidney (n=1), tonsil (n=1), testes (n=1). Three new malignancies were incidentally detected by PET including lung cancer (n=1), metastatic renal cell to the parotid (n=1), and mantle cell lymphoma (n=1 in a pt with ALCL-cut). Despite these additional sites, stage was changed in only 10/107 (9%). We did not use negative PET to downstage. Sites resulting in higher stage included bone (n=3), lymph node (n=2), subcut/muscle (n=3), testes (n=1), liver (n=1). Skin lesions were noted on physical exam and therefore did not change stage. In conclusion, TCL are almost universally PET positive. PET often adds information by identifying extranodal disease. However, partly due to many pt being stage IV by other modalities, PET resulted in a change of stage in <10% of pt. PET may be particularly useful in assessing of skin sites. These data suggest that it may be beneficial to include PET in the response assessment of TCL, as has been proposed for DLBCL and HL in revised NHL response criteria.

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