HHV-8-Associated T-Cell Lymphoma in a Lymph Node With Concurrent Peritoneal Effusion in an HIV-Positive Man

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.

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

Journal of Feline Medicine and Surgery Open Reports ◽

10.1177/2055116917730180 ◽

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.

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Subcutaneous Panniculitis-like T-Cell lymphoma in Two Pediatric Patients: An HIV-Positive Adolescent and a 4-Month-Old Infant With No Significant History and Review of the Literature

American Journal of Clinical Pathology ◽

10.1093/ajcp/138.suppl1.296 ◽

2012 ◽

Vol 138 (suppl 1) ◽

pp. A319-A319

Author(s):

Sara C. Acree ◽

David S. Cassarino

Keyword(s):

T Cell ◽

Pediatric Patients ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Hiv Positive ◽

Review Of The Literature

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

Epidemiology and Infectious Diseases (Russian Journal) ◽

10.18821/1560-9529-2018-23-2-77-84 ◽

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.

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Subcutaneous Panniculitis-Like T-Cell Lymphoma in Two Pediatric Patients: An HIV-Positive Adolescent and a 4-Month-Old Infant

Fetal and Pediatric Pathology ◽

10.3109/15513815.2012.701264 ◽

2012 ◽

Vol 32 (3) ◽

pp. 175-183 ◽

Cited By ~ 10

Author(s):

Sara C. Acree ◽

Jason P. Tovar ◽

Paul K. Pattengale ◽

Larry L. Wang ◽

Joseph A. Church ◽

...

Keyword(s):

T Cell ◽

Pediatric Patients ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Hiv Positive

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T-cell lymphoma and the Chediak-Higashi syndrome

Blood ◽

10.1182/blood.v60.3.672.672 ◽

1982 ◽

Vol 60 (3) ◽

pp. 672-676 ◽

Cited By ~ 17

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.

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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 ◽

10.1182/blood.v104.11.1374.1374 ◽

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.

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

Blood ◽

10.1182/blood.v108.11.2399.2399 ◽

2006 ◽

Vol 108 (11) ◽

pp. 2399-2399 ◽

Cited By ~ 1

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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Molecular diagnosis angioimmunoblastic T-cell lymphoma

Terapevticheskii arkhiv ◽

10.26442/00403660.2019.07.000330 ◽

2019 ◽

Vol 91 (7) ◽

pp. 63-69

Author(s):

N G Chernova ◽

Y V Sidorova ◽

S Y Smirnova ◽

N V Ryzhikova ◽

E E Nikulina ◽

...

Keyword(s):

Bone Marrow ◽

Lymph Node ◽

T Cell ◽

Peripheral Blood ◽

Cell Lymphoma ◽

Cell Receptor ◽

T Cell Lymphoma ◽

Tissue Samples ◽

Angioimmunoblastic T Cell Lymphoma ◽

Allele Specific

Aim: to determine molecular diagnostics routine for different tissue samples in angioimmunoblastic T-cell lymphoma. Materials and methods. Molecular studies were performed for 84 primary AITL patients. The median age was 61 year (29-81); the male to female ratio was 48/36. T-cell and B-cell clonality was assessed by GeneScan analysis of rearranged T-cell receptor (TCRG, TCRB) and immunoglobulin heavy chain genes. For the quantitative determination of cells with RHOA G17V mutation real - time polymerase chain reaction (PCR) with allele - specific LNA modified primers was used. Results. In lymph nodes rearrangements of T-cell receptor genes were determined in 76 (90.5%) of 84 patients and were absent in 8 (9.5%) cases. Identification of the same clonal products of the TCRG and TCRB genes in the lymph node and in peripheral blood and/or bone marrow indicated the prevalence of the tumor process and was observed in 64.7% of patients. Clonal products in peripheral blood and/or bone marrow different from those in the lymph node indicated reactive cytotoxic lymphocyte population and were noted in 58.8% of AITL cases. Simultaneous detection of T- and B-cell clonality in the lymph node was observed in 20 (24.7%) of 81 patients. Cells with RHOA G17V mutation were detected in lymph node in 45 (54.9%) of 82 patients. The use of allele - specific PCR with LNA modified primers revealed presence of the tumor cells in peripheral blood in 100% and in bone marrow in 93.9% of patients with G17V RHOA mutation in the lymph nodes. Conclusion. The validity of different molecular assays performed on certain tissue samples for the diagnosis of angioimmunoblastic T-cell lymphoma has been evaluated. Quantitative allele - specific PCR assay for RHOA G17V mutation based on LNA modified primers possesses sufficient sensitivity for tumor process prevalence evaluation and minimal residual disease monitoring.

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