BACKGROUND: Epstein-Barr virus (EBV) is closely associated with African Burkitt lymphoma, B-cell lymphoproliferative disorders arising in immunocompromised individuals, and nasopharyngeal carcinoma. Whether EBV is also associated with non-Hodgkin lymphoma (NHL) in patients without pre-existing immunocompromized status is less clear. The authors examined the clinical, morphologic, immunohistochemical features of 42 sporadic NHLs in areas of head and neck and aslo analyzed the presence of EBV genome by using polymerase chain reaction (PCR). METHODS: All lymphoma cases examined were classified according to the Working Formulation and also determined the immunophenotype using paraffin-embedded sections. For PCR, DNA was extracted from formalin fixed, paraffin-embedded tissue using chelating resin with some modifications, and processed amplification with EBV genome primers. RESULTS: Morphologically, the cases consisted of 21 diffuse large cell, 7 diffuse mixed cell, 5 large immunoblastic, 4 follicular, two small cleaved cell, two small lymphocytic and one lymphoblastic lymphoma in Working Formulation classification. Immunohistochemically, 29 cases were of B-cell lineage, 11 cases were of T-cell lineage and two cases were of non-B and non-T cell immunophenotype. EBV genome was detected in 8 of 42 cases (19%) including 3 of 29 B-cell lymphomas (10%), and 5 of 11 T-cell lymphomas (45%). CONCLUSION: These findings suggest that EBV play a role in the development of B and T-cell lymphoma.
B-Lymphocytes ; Burkitt Lymphoma ; Classification ; DNA ; Formaldehyde ; Genome ; Head* ; Herpesvirus 4, Human* ; Humans ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin* ; Lymphoma, T-Cell ; Lymphoproliferative Disorders ; Neck* ; Polymerase Chain Reaction ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; T-Lymphocytes

Epstein-Barr virus (EBV) is one of eight human herpesvirus. Primary infection with EBV in childhood is generally asymptomatic or mild, however, often causes overt diseases such as infectious mononucleosis (IM) and lymphoproliferative disorder (LPD), the latter occursing in immunologically compromised individuals. Historically, EBV has been considered to be etiologically linked to human malignancies such as EBV genome positive Burkitt's lymphoma and nasopharyngeal carcinoma. Recently, however, another category of EBV-related disease, "chronic active EBV infection", has been made to describe persons without a clearly defined underlying disease. We report 4 cases of patients, presented with episodic fever of unknown origin (FUO), who were diagnosed as severe chronic active EBV infection. A combined application of serology for EBV and in-situ hybridization established the diagnosis of the EBV infection.
Burkitt Lymphoma ; Diagnosis ; Epstein-Barr Virus Infections ; Fever of Unknown Origin* ; Fever* ; Genome ; Herpesvirus 4, Human* ; Humans ; Infectious Mononucleosis ; Lymphoproliferative Disorders

Epstein-Barr virus (EBV) is one of eight human herpesvirus. Primary infection with EBV in childhood is generally asymptomatic or mild, however, often causes overt diseases such as infectious mononucleosis (IM) and lymphoproliferative disorder (LPD), the latter occursing in immunologically compromised individuals. Historically, EBV has been considered to be etiologically linked to human malignancies such as EBV genome positive Burkitt's lymphoma and nasopharyngeal carcinoma. Recently, however, another category of EBV-related disease, "chronic active EBV infection", has been made to describe persons without a clearly defined underlying disease. We report 4 cases of patients, presented with episodic fever of unknown origin (FUO), who were diagnosed as severe chronic active EBV infection. A combined application of serology for EBV and in-situ hybridization established the diagnosis of the EBV infection.
Burkitt Lymphoma ; Diagnosis ; Epstein-Barr Virus Infections ; Fever of Unknown Origin* ; Fever* ; Genome ; Herpesvirus 4, Human* ; Humans ; Infectious Mononucleosis ; Lymphoproliferative Disorders

Burkitt Lymphoma ; diagnosis ; pathology ; Female ; Hodgkin Disease ; diagnosis ; pathology ; Humans ; Lymphoma ; classification ; diagnosis ; pathology ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; pathology ; Lymphoma, Follicular ; diagnosis ; pathology ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; pathology ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; pathology

PURPOSE: To analyze the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after liver transplantation in children. METHODS: From January 1988 to June 2009, we retrospectively reviewed the medical records of 8 PTLD cases among 148 pediatric patients underwent liver transplantation. The age at transplantation, time of presentation after transplantation, clinical manifestations, histologic diagnosis, results of EBV (Epstein-Barr virus) assessments, managements and outcomes of PTLD were investigated. RESULTS: The prevalence of PTLD in liver transplant pediatric recipients was 5.4% (8 of 148). The mean age of patients was 25.4+/-21.3 months (range 10 to 67 months). Seven of 8 patients (87.5%) underwent liver transplantation before 1 year of age. The common clinical presentations were persistent fever (8 of 8, 100%) and bloody diarrhea (6 of 8, 75%). PTLD was diagnosed with gastrointestinal endoscopic biopsies in five patients and surgical biopsies in three. Histologic findings showed early lesion in three patients, polymorphic in two, and monomorphic in three. Burkitt lymphoma and lymphoblastic lymphoma were found in two of 3 monomorphic patients. Seven of 8 patients were found with EBV-positive. Eight patients were treated with dose reduction of immunosuppressants and infusion of ganciclovir. Rituximab was added to four patients. PTLD were successfully managed in all patients except one who died of sepsis during chemotherapy. CONCLUSION: Major risk factor of PTLD was to undergo liver transplantation before 1 year of age. Continuous monitoring for EBV viral load and gastrointestinal endoscopic biopsy may be useful to early detection of PTLD.
Antibodies, Monoclonal, Murine-Derived ; Biopsy ; Burkitt Lymphoma ; Child ; Diarrhea ; Fever ; Ganciclovir ; Herpesvirus 4, Human ; Humans ; Immunosuppressive Agents ; Liver ; Liver Transplantation ; Lymphoproliferative Disorders ; Medical Records ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prevalence ; Retrospective Studies ; Risk Factors ; Sepsis ; Transplants ; Viral Load ; Rituximab

Paraneoplastic autoimmune multiorgan syndrome (PAMS) is also known as paraneoplastic pemphigus, and this is a heterogenous autoimmune syndrome involving the skin, mucosa and internal organs and it is caused by autoantibodies related to the underlying neoplasm. Painful stomatitis is the earliest and most constant feature. The cutaneous lesions of this disease are polymorphic. The majority of cases of this syndrome are associated with a lymphoproliferative disorder such as non-Hodgkin lymphoma, chronic lymphocytic leukemia and Castleman disease. A 40-year-old woman presented with a 1-month history of a pruritic erythematous papulomacular rash on the trunk and extremities. She also had a 4-month history of painful oral stomatitis that had failed to respond to steroid therapy. Despite the vigorous treatment, the skin lesions progressed to generalized polymorphic lesions with persistent oral lesions. The histopathologic findings of these lesions revealed polymorphous characteristics according to the clinical features, and they mimicked erythema multiforme, graft-versus-host disease, lichen planus and pemphigus vegetans. Finally, a mass of lymphatic tissue 7.2 cm in diameter and that was diagnosed as Castleman disease was found in her retroperitoneal pelvic cavity. With removal of the lymphatic mass, her skin lesions subsided except for the glossal lesion.
Adult ; Autoantibodies ; Erythema Multiforme ; Exanthema ; Extremities ; Female ; Giant Lymph Node Hyperplasia ; Graft vs Host Disease ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lichen Planus ; Lymphoid Tissue ; Lymphoma, Non-Hodgkin ; Lymphoproliferative Disorders ; Mucous Membrane ; Pemphigus ; Skin ; Stomatitis

BACKGROUND: Treatment outcomes of children with non-Hodgkin lymphoma (NHL) have dramatically improved in recent years. However, there are few studies on the outcomes of pediatric NHL in Korea.METHODS: We retrospectively analyzed the outcomes of 34 children diagnosed with NHL and treated at Kosin University Gospel Hospital from Jan. 1987 to Dec. 2009, according to age, lactate dehydrogenase (LDH) level, histology, stage and involved site.RESULTS: The mean age of the subjects was 9.0 years. The abdomen and head/neck regions were the most common primary sites. On histologic classification, Burkitt lymphoma was the most common, followed by lymphoblastic lymphoma, diffuse large B-cell lymphoma, anaplastic large cell lymphoma, and unclassifiable, with respective incidences of 35.3%, 23.5%, 17.6%, 17.6%, and 5.9%. Various combination chemotherapies according to the diagnosis with mean treatment duration of 14.9 months showed 5 year event free survival (EFS) and 5 year overall survival (OS) rate of 67.7+/-8.0% and 79.3+/-7.0%, respectively. Nine out of the 34 patients relapsed, and the 5 year OS rates for those who relapsed vs. 25 patients without relapse were 44.4+/-16.6%, vs. 92.0+/-5.4%, respectively (P<0.01). Although 5 year EFS rate varied according to stage, 5 year OS rate were not different according to age, sex, LDH, stage, histology, or treatment period.CONCLUSION: The outcome of children with NHL treated in our setting was comparable to those of other large centers in Korea. No factor other than stage, including LDH, histologic subtype showed significant prognostic value.
Abdomen ; Burkitt Lymphoma ; Child ; Classification ; Diagnosis ; Disease-Free Survival ; Drug Therapy, Combination ; Humans ; Incidence ; Korea ; L-Lactate Dehydrogenase ; Lymphoma, B-Cell ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Non-Hodgkin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence ; Retrospective Studies

BACKGROUND AND OBJECTIVES: The Epstein-Barr virus (EBV) is a human herpes virus which causes infectious mononucleosis and is associated with such human cancers as Burkitt's Lymphoma, Hodgkin's disease, non-Hodgkin's disease, and nasopharyngeal carcinoma. However, the role of EBV as a causative factor in other head & neck tumors is not fully elucidated. This study analyzed the detection rate of Epstein-Barr virus (EBV) in the throat washing samples from pateints with head and neck tumors and non-malignant diseases, as well as from normal subjects. MATERIALS & METHODS: The throat washing samples were collected from 49 patients of head & neck tumors, 52 patients of non-malignant diseases (pharyngitis and tonsillitis) and 24 normal subjects. The EBV DNA was detected by using the EBV (BNRF1)-specific primers & Polymerase Chain Reaction (PCR). RESULT: The EBV DNA was detected in 25 (51%) of the 49 throat washing samples from patients with tumors, as well as 3 (60%) of 5 samples from patients with nasopharyngeal carcinoma, and 22 (50%) of 44 samples from patients with head and neck tumors. The throat wasing samples from 52 patients with non-malignant diseases and 24 samples from healthy subjects were also examined. EBV was detected in 17 (32.7%) of 52 patients with non-malignant diseases and 3 (12.5%) of 24 samples from healthy subjects. CONCLUSION: These data indicate that the EBV may be related to non-malignant diseases and head and neck tumor.
Burkitt Lymphoma ; DNA* ; Head ; Herpesvirus 4, Human* ; Hodgkin Disease ; Humans ; Infectious Mononucleosis ; Neck ; Oropharynx* ; Pharynx ; Polymerase Chain Reaction*

Castleman's disease or angiofollicular lymph node hyperplasia is a rare lymphoproliferative disorder. Complete surgical resection was recommended in unicentric Castleman's disease. Radiotherapy was considered alternative therapeutic option. However, there have been consistent favorable responses to radiotherapy. We also experienced two cases of uncentric Castleman's disease salvaged successfully with radiotherapy. This paper described these cases and reviewed the literature about Castleman's disease treated with radiotherapy. Reviewed cases showed that radiotherapy is a successful treatment option in unicentric Castleman's disease. Furthermore, our report confirms the radiotherapy role in uncentric Castleman's disease.
Giant Lymph Node Hyperplasia ; Lymphoproliferative Disorders

The Reed-Sternberg like cells (RS-like cells) are by no means specific to Hodgkin lymphoma, but they have been reported in various other lymphoproliferative disorders, including infectious mononucleosis. Many studies have postulated that Epstein-Barr virus (EBV) infection may play a role in the development of RS-like cells in non-Hodgkin's lymphoma. There are many accounts in the literature about these RS-like cells in lymph node aspirates and biopsies creating diagnostic confusion with Hodgkin lymphoma, but no report in Korea. We experienced a case of a 44 year-old male patient who had multiple lymph nodes enlargement with the history of treatment of Hodgkin lymphoma 15 years ago. At this time, this patient was diagnosed as peripheral T-cell lymphoma with RS-like cells associated with EBV in lymph node biopsy. Here, we report this case with a review of the relevant literature.
Adult ; Biopsy ; Herpesvirus 4, Human* ; Hodgkin Disease ; Humans ; Infectious Mononucleosis ; Korea ; Lymph Nodes ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell, Peripheral* ; Lymphoproliferative Disorders ; Male