scholarly journals Histiocytic Sarcoma of Bone Marrow With Partial Cohesive Neoplastic Cells and Hemophagocytosis: a Case Report

2021 ◽  
Author(s):  
Yang Liang ◽  
Li Mao ◽  
Yihua Chen ◽  
Guangjie Wang
Keyword(s):  
Bone Marrow ◽  
Tumor Cells ◽  
Hemophagocytic Syndrome ◽  
Histiocytic Sarcoma ◽  
Neoplastic Cells ◽  
Case Presentation ◽  
Barr Virus ◽  
History Of ◽  
Epstein Barr ◽  
Aggressive Clinical Course

Abstract Background: Histiocytic sarcoma (HS) is a rare hematolymphoid neoplasms whose cells show morphologic and immunophenotypic features of mature tissue histiocytes. We herein report a HS case without nodules or lymphadenectasis confirmed with the help of immunohistochemistry (IHC) on bone marrow (BM) biopsy and smear.Case presentation: A 63-year-old female patient with a history of cerebral infarction presented with fever, retching and hypodynamic sign for three weeks. The peripheral blood examination showed aggressive pancytopenia accompanying with the positivity for Epstein-Barr virus (EBV) antibody. The computed tomography and abdomen ultrasound scan didn’t reveal any nodules or lymphadenectasis other than hypersplenotrophy. Significantly, the BM aspirate shows vast pleomorphic tumor cells atypically distributed in both forms of single diffuse and cohesive. The hemophagocyte phagocytized granulocytes on BM smear exhibited the lymphoma related hemophagocytic syndrome. Immunohistochemically, neoplastic cells were immunopositively for macrophage-associated antigen cluster of CD4, CD68, CD163, but negative for the T-cell, B-cell and myeloid lineage markers of CD15, CD20, PAX-5, CD5, CD30, CD3, CD56, CD38, CD138, ALK and MPO, confirming the hypothesis of HS in BM. Conclusion: The rare HS case of bone marrow with atypically partial cohesive pleomorphic tumor cells had the hemophagocytic syndrome, presented highly aggressive clinical course and challenged to the diagnoses.

Epstein-Barr Virus: Main Pathogenic Agent in Hodgkin Lymphoma Associated Hemophagocytic Syndrome?.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2272-2272
Author(s):  
Fanny Menard ◽  
Caroline Besson ◽  
Patricia Rince ◽  
Olivier Lambotte ◽  
Thierry Lazure ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Sex Ratio ◽  
Lymph Nodes ◽  
Hodgkin Lymphoma ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Tumour Cells ◽  
Mixed Cellularity ◽  
Barr Virus ◽  
Epstein Barr

Abstract Hemophagocytic syndrome (HPS) is characterized by an uncontrolled proliferation of macrophages displaying extensive phagocytosis of hematopoietic cells that can be associated with non Hodgkin lymphomas. However, Hodgkin lymphoma associated with hemophagocytic syndrome (HL-HPS) has rarely been published. We report here a large series of 34 patients presenting HL-HPS. Histological analysis and Epstein-Barr Virus (EBV) studies were performed. The 34 patients were enrolled from 1992 to 2006. HPS diagnosis was based on usual clinico-biological criteria. Bone marrow aspiration or biopsy were performed in all cases and confirmed HPS, showing hemophagocytic features in scattered macrophages. HL was documented on histological material in all 34 patients. The biopsies were performed on different localizations: lymph nodes (n=18), bone marrow (n=27), liver (n=2) and tonsil (n=1). All histological slides were reviewed and classified according to the World Health Organization (WHO) Lymphoma classification. The presence of EBV in the tumour cells was detected using EBER RNA in situ hybridization and the expression of viral proteins LMP-1, EBNA-2 by immunoperoxidase. Patients were 26 men and 8 women (sex ratio M/F = 3.3). The median age was 45 years (range 19–84). HIV status was negative in 26 patients and positive in 8 patients. All patients were in clinical stage IVB. HL subtypes (18 lymph nodes) were Mixed Cellularity (n=12, 67%), Nodular Sclerosis (n=2, 11%) and Lymphocyte Depleted (n=4, 22%). Extra nodal tissues were highly infiltrated by tumour cells in all cases. The presence of EBV in tumour cells was detected in 32 out of the 34 patients (94%). In all EBV positive cases, high levels of LMP-1 without EBNA2 expression were detected, defining a latency II. This study reports the largest series to date of HL-HPS. The features of HL-HPS are particular by the high proportion of mixed cellularity subtypes (67%) in contrast to the frequency of nodular sclerosis subtypes observed in the general population of non overt immunosuppressed patients, and by the striking high proportion of EBV associated HL (32 out of the 34 patients, 94%). Our findings suggest not only a pathogenic role of EBV, but also a defective immune system in the control of EBV infection in HL-HPS patients.The high predominance of male in our population of HPS-HL (sex ratio M/F = 4.3 after exclusion of HIV positive patients) and the strong association with EBV could suggest X-linked immune defect in these patients.

Clinicopathological findings of virus-associated hemophagocytic syndrome in bone marrow: Association with Epstein-Barr virus and apoptosis

1999 ◽  
Vol 49 (6) ◽  
pp. 533-540 ◽  
Author(s):  
Koichi Ohshima ◽  
Kae Shimazaki ◽  
Midori Sugihara ◽  
Seiji Haraoka ◽  
Junji Suzumiya ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Barr Virus ◽  
Clinicopathological Findings ◽  
Epstein Barr

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 Epstein–Barr Virus (EBV) Viral Load in Tumor Cells Did Not Predict Tumor Extensiveness in Nasopharyngeal Cancer

2021 ◽  
Vol 12 (1) ◽  
pp. 150-156
Author(s):  
Soehartati A. Gondhowiardjo ◽  
Handoko ◽  
Marlinda Adham ◽  
Lisnawati Rachmadi ◽  
Henry Kodrat ◽  
...  
Keyword(s):  
Viral Load ◽  
Tumor Cells ◽  
Epstein Barr Virus ◽  
Tumor Volume ◽  
Nasopharyngeal Cancer ◽  
Dna Quantification ◽  
Barr Virus ◽  
Nasopharyngeal Biopsy ◽  
Ebv Dna ◽  
Epstein Barr

Background: Nasopharyngeal cancer is commonly associated with Epstein–Barr virus (EBV) infection, especially undifferentiated non-keratinized histology. EBV DNA quantification through nasopharyngeal brushing was previously reported to be not related to disease stage. This study aimed to reinvestigate the relationship of EBV viral load in tumor tissue with tumor extensiveness by more accurate EBV DNA quantification through microscopically confirmed tumor cells from nasopharyngeal biopsy. Method: The specimens for EBV DNA quantification were derived from histopathology slides which were pre-treated following the QIAsymphony® SP protocol for tissue DNA extraction. Then, the extracted DNA underwent real-time polymerase chain reaction (RT-PCR) using the artus® EBV RG PCR Kit for EBV DNA quantification. The tumor volume was determined by delineating the gross tumor based on 3D imaging of the patient’s nasopharynx. Result: Twenty-four subjects were included in this study. All subjects were stage III and above, with more males (75%) than females. EBV viral load in tumor cells was found to have no correlation to tumor volume both in local and nodal regions. The median local tumor volume was 81.3 cm3 ± 80 cm3. The median EBV viral load in tumor cells was 95,644.8 ± 224,758.4 copies/100 ng of DNA. The median nodal or regional tumor volume was 35.7 ± 73.63 cm3. Conclusion: EBV viral load from tumor cells from nasopharyngeal biopsy has no relationship with tumor extensiveness in nasopharyngeal cancer. The presence and amount of EBV in tumor cells did not translate into larger or smaller tumors. The EBV viral proteins and RNAs were perhaps more likely to confer some prognostic information due to the fact that those molecules were related to carcinogenesis.

Immunotherapy for Esophageal and Gastric Cancer

2017 ◽  
pp. 292-300 ◽  
Author(s):  
Ronan J. Kelly
Keyword(s):  
Gastric Cancer ◽  
Tumor Cells ◽  
Immune Cells ◽  
Single Agent ◽  
Phase Iii ◽  
Immune Checkpoints ◽  
Barr Virus ◽  
Mutation Burden ◽  
Heavily Pretreated ◽  
Epstein Barr

PD-L1 upregulation occurs in approximately 40% of gastroesophageal cancers. However, unlike other solid tumors, there is minimal PD-L1 expressed on the cancer cells; rather, expression occurs predominantly on infiltrating myeloid cells. Preliminary clinical data involving single-agent PD-1/PD-L1 inhibitors in metastatic gastroesophageal cancer have reported response rates of 22%–27% for patients with PD-L1+ tumors and 10%–17% for unselected patients. The phase III ONO-4538-12 (ATTRACTION 2) trial has demonstrated an improved overall survival for nivolumab compared with placebo for patients with heavily pretreated gastric cancer. In the future, we will need better biomarkers to select those most likely to respond and/or identify patients who may need combination immunotherapeutics or alternate strategies. A number of subsets of gastric cancer with different immune signatures, most notably tumors positive for Epstein-Barr virus and microsatellite instability, have been identified, with approximately 50% and 94% PD-L1+ staining seen on tumor cells and immune cells in the EBV subtype and approximately 33% and 45% PD-L1+ staining seen on tumor cells and immune cells in MSI high tumors. Both subtypes demonstrate PD-L1+ immune cells with tumor-infiltrating patterns, unlike the more commonly seen PD-L1+ immune cells at the invasive margin. PD-L2 expression has been reported in 52% of esophageal adenocarcinomas but little is known about the expression of other immune checkpoints. Additional factors that suggest gastroesophageal cancers may respond to checkpoint inhibition include the high somatic mutation burden and the link with chronic inflammation. Here we provide a comprehensive review of the checkpoint inhibitor data published to date in advanced esophagogastric cancers and rationalize how the immune microenvironment in these diverse tumors can explain response or resistance to immunotherapeutics.

scholarly journals Quantitative multi-target RNA profiling in Epstein-Barr virus infected tumor cells

2017 ◽  
Vol 241 ◽  
pp. 24-33 ◽  
Author(s):  
A.E. Greijer ◽  
O. Ramayanti ◽  
S.A.W.M. Verkuijlen ◽  
Z. Novalić ◽  
H. Juwana ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Epstein Barr Virus ◽  
Rna Profiling ◽  
Barr Virus ◽  
Epstein Barr ◽  
Target Rna ◽  
Infected Tumor

Cytomegalovirus-Associated Hemophagocytic Syndrome

PEDIATRICS ◽  
1985 ◽  
Vol 75 (2) ◽  
pp. 280-283
Author(s):  
ELIZABETH H. DANISH ◽  
BEVERLY B. DAHMS ◽  
MARY L. KUMAR
Keyword(s):  
Viral Infection ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Clinical Course ◽  
Underlying Disease ◽  
Barr Virus ◽  
Pathologic Findings ◽  
Immunosuppressed Patients ◽  
Epstein Barr ◽  
Herpes Group

Virus-associated hemophagocytic syndrome, first described by Risdall and co-workers in 1979,1 is a rare histiocytic proliferative syndrome characterzed by fever, hepatosplenomegaly, pancytopenia, and erythrophagocytosis by histiocytes that appear benign by histologic criteria. The clinical course and pathologic findings may be identical with another histiocytic disorder, familial erythrophagocytic lymphohistiocytosis, which occurs predominantly in infants. Diagnosis of virus-associated hemophagocytic syndrome depends entirely on evidence of concurrent viral infection, usually of the herpes group. Epstein-Barr virus has been associated with this syndrome in the few cases reported in children without underlying disease, whereas cytomegalovirus (CMV) has been implicated in immunosuppressed patients. We report a case of fatal CMV-associated hemophagocytic syndrome which occurred in a previously healthy infant.

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