scholarly journals A Case of Relapsing Kikuchi-Fujimoto Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Talayeh Rezayat ◽  
Matthew B. Carroll ◽  
Bryan C. Ramsey ◽  
Andria Smith
Keyword(s):  
Lupus Erythematosus ◽  
Symptomatic Relief ◽  
Specific Treatment ◽  
Cervical Lymphadenopathy ◽  
Severe Case ◽  
Lymph Node Biopsy ◽  
Barr Virus ◽  
Cell Mediated Immune Response ◽  
Night Sweats ◽  
Epstein Barr

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis was first described in Japan in 1972. It is described as a benign syndrome most commonly involving cervical lymphadenopathy, fever, and night sweats. The etiology of KFD is unknown but it is thought to be triggered by an autoimmune or viral process with an exaggerated T-cell-mediated immune response. KFD can mimic other serious conditions such as lymphoma, systemic lupus erythematosus (SLE), herpes simplex, and Epstein Barr virus. Diagnosis is confirmed histopathologically. Kikuchi’s disease is typically reported to have a self-limiting course, resolving within several months and with a low recurrence rate between 3% and 4%. There is no specific treatment for KFD but any treatment is generally directed towards symptomatic relief with antipyretics and anti-inflammatory medications. In severe cases corticosteroids have been used. Here we describe a case of a previously healthy 26-year-old female that presented with fever and cervical lymphadenopathy. Malignancy and infections were ruled, and she was diagnosed with KFD histopathologically by lymph node biopsy. Her case is a severe case of KFD that despite treatment with multiple courses of corticosteroids and an immune modulating agent, relapsed.

scholarly journals Case Report: Kikuchi-Fujimoto disease: a diagnostic and therapeutic dilemma following pretransplant nephrectomy for a 2.35 Kg kidney

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 1407
Author(s):  
Arvind P. Ganpule ◽  
Jaspreet Singh Chabra ◽  
Abhishek G. Singh ◽  
Gopal R. Tak ◽  
Shailesh Soni ◽  
...  
Keyword(s):  
Lymph Node ◽  
Lupus Erythematosus ◽  
Cervical Lymphadenopathy ◽  
Lymph Node Biopsy ◽  
Adult Polycystic Kidney Disease ◽  
Worldwide Distribution ◽  
Night Sweats ◽  
Necrotizing Lymphadenitis ◽  
Stage Renal Disease ◽  
End Stage

Kikuchi-Fujimoto disease (KFD) is an extremely rare disease with a worldwide distribution and higher prevalence in Asians. It is a benign and self-limiting disorder, characterized by regional cervical lymphadenopathy accompanied with mild fever and night sweats. Lymph node histopathology is diagnostic and treating physicians should be aware of this entity as it may mimic other systemic diseases like systemic lupus erythematosus, tuberculosis, malignant lymphoma, and more rarely adenocarcinoma. Key features on lymph node biopsy are fragmentation, necrosis and karyorrhexis. Treatment includes symptomatic care, analgesics-antipyretics, corticosteroids and spontaneous recovery occurs in 1 to 4 months. We report a case of adult polycystic kidney disease (ADPKD) with end stage renal disease and episodes of fever and cervical lymphadenopathy. The infectious screen was negative and on extensive workup, the patient was found to have histiocytic-necrotizing lymphadenitis, which clinched the diagnosis of KFD.

scholarly journals Castleman’s Disease and Posttransplant Lymphoproliferative Disorder after Liver Transplant: 3-Year Follow-Up

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Lokesh K. Jha ◽  
Laura L. Ulmer ◽  
Marco A. Olivera-Martinez ◽  
Timothy M. McCashland ◽  
Kai Fu ◽  
...  
Keyword(s):  
Liver Transplant ◽  
Castleman’S Disease ◽  
Lymph Node Biopsy ◽  
Castleman's Disease ◽  
Human Herpes Virus 8 ◽  
Barr Virus ◽  
Night Sweats ◽  
History Of ◽  
Epstein Barr

A 59-year-old male with a history of hepatitis C cirrhosis and history of hepatitis B exposure presented 8 months after orthotopic liver transplant (LT) with fever, fatigue, myalgia, night sweats, nonproductive cough, and shortness of breath. Bone marrow biopsy for pancytopenia was positive for Epstein-Barr virus (EBV) DNA. Lymph node biopsy for lymphadenopathy on imaging showed human herpes virus 8 (HHV8) associated Castleman’s disease. Treatment included valganciclovir, rituximab, and prednisone taper with eventual discontinuation. Quantitative HHV8 DNA was initially 611,000 DNA copies/mL and was later undetectable at 6 months following treatment and remained undetectable at 3-year follow-up.

Fatal, Rapidly Progressive EBV-Associated Warm Autoantibody Hemolytic Anemia (WAIHA): A Rare Case Study

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S158-S158
Author(s):  
C E Kanakis ◽  
K Gvozdjan ◽  
L Sereseanu ◽  
D Rodheim ◽  
P J DeChristopher
Keyword(s):  
Organ Failure ◽  
Cervical Lymphadenopathy ◽  
Altered Mental Status ◽  
Lymph Node Biopsy ◽  
Blood Bank ◽  
Rapid Progression ◽  
Barr Virus ◽  
Relative Paucity ◽  
Night Sweats

Abstract Introduction/Objective Separately WAIHA and Epstein-Barr Virus-infectious mononucleosis (EBV-IM) are well- studied clinical entities. EBV-IM cases are commonly linked with cold autoagglutinins (IgMs with anti-i specificity), but uncommonly associated with WAIHA. More rarely, EBV-IM can be associated with rapidly fatal AIHA. Prompt and appropriate diagnostic Blood Bank testing for WAIHA in EBV-infected patients should include work-ups to prevent missing the possibility of treating complicating WAIHA. Methods/Case Report A 24-year-old woman presented to the ED with a month of cough/cold symptoms, night sweats, cervical lymphadenopathy, and imaging showing diffuse thoracic lymphadenopathy. DNA/PCR testing was positive for active EBV infection and a lymph node biopsy was diagnostic for EBV lymphadenitis. Increasing respiratory distress developed, coupled with renal and liver insufficiency, hypotension, and possible DIC, and (suspected septic) shock. On hospital day (HD) 5, altered mental status worsened, cardiac arrest occurred requiring resuscitation and vasopressors, and acidosis trended rapidly higher. The patient rapidly decompensated and she was compassionately extubated, expiring on HD 6. There were significant laboratory interval changes in the final 20 hours of life. New positive DATs (with anti-IgG and - C3b/d), a pan-reactive warm autoantibody of broad specificity (in plasma & eluate), onset of acute hemolysis (nadir Hgb to 4.6 g/dL with net drop of 4.7 g/dL & LDH 23, 582 U/L), coagulopathy (peak PT 96.6 sec) and evidnece of organ failure. Blood Bank testing, received on HD 5 at 21:45, was resulted in the EMR on HD 6 at 00:30 and the patient expired at 05:15--further illustrating the rapid clinical course presented in this case. Results (if a Case Study enter NA) N/A Conclusion This patient died of complications directly associated with acute, immune-mediated intravascular hemolysis leading to high-output cardiac failure and resulting in multi-organ failure. With a relative paucity of published EBV-IM WAIHA associations, the rapidly evolving unexpected hematologic sequelae observed complicated the clinical recognition and treatment of severe WAIHA during the course of treatment for clinically suspected DIC. The rapid progression of this patients’ symptoms and laboratory values did not allow for adequate recognition of WAIHA-related pathogenesis and appropriate interventions.

EBV load is associated with cfDNA fragmentation and renal damage in SLE patients

Lupus ◽  
2021 ◽  
pp. 096120332110103
Author(s):  
Anna Truszewska ◽  
Agnieszka Wirkowska ◽  
Kamila Gala ◽  
Piotr Truszewski ◽  
Łucja Krzemień-Ojak ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Lupus Erythematosus ◽  
Epstein Barr Virus ◽  
Renal Damage ◽  
Healthy Individuals ◽  
Pcr Assay ◽  
Barr Virus ◽  
Fragmentation Index ◽  
Epstein Barr

Background For long Epstein–Barr virus (EBV) has been suspected to be involved in the pathogenesis of systemic lupus erythematosus (SLE). The aim of this study was to verify the association between EBV, cell-free DNA (cfDNA) and kidney disease in SLE. Methods Blood samples were obtained from 43 SLE patients and 50 healthy individuals. EBV load was measured via real-time PCR assay. Sizing and quantification of plasma cfDNA was performed on Bioanalyzer. We proposed that the uniformity of cfDNA fragmentation can be described using cfDNA fragmentation index. Results SLE patients with chronic kidney disease (CKD +) had higher EBV load compared to CKD(–) patients (P = 0.042). Patients with high cfDNA level had higher EBV load (P = 0.041) and higher cfDNA fragmentation index (P < 0.001) compared to patients with low cfDNA level. Among patients with high cfDNA level, EBV load was higher in CKD(+) group compared to CKD(–) group (P = 0.035). EBV load was positively correlated with the fragmentation index in all SLE patients (P = 0.028, R2 = 0.13), and the correlation was even more pronounced in CKD (+) patients (P < 0.001, R2 = 0.20). Conclusions We showed that EBV load was associated with non-uniform cfDNA fragmentation, higher cfDNA levels, and kidney disease in SLE patients. Although the causality of this relationship could not be determined with the current study, it brings rationale for further investigations on the role of EBV and cfDNA interplay in SLE pathogenesis.

scholarly journals Production and nucleotide sequence of an inhibitory human IgM autoantibody directed against platelet glycoprotein Ia/IIa

Blood ◽  
1994 ◽  
Vol 84 (6) ◽  
pp. 1968-1974 ◽  
Author(s):  
H Deckmyn ◽  
J Zhang ◽  
E Van Houtte ◽  
J Vermylen
Keyword(s):  
Platelet Aggregation ◽  
Cell Lines ◽  
Lupus Erythematosus ◽  
Gene Segment ◽  
Platelet Glycoprotein ◽  
Enzyme Linked Immunosorbent Assay ◽  
Germline Gene ◽  
Barr Virus ◽  
Epstein Barr ◽  
Human B Cell

Abstract Human B-cell lines were derived by limiting dilutions of Epstein-Barr virus (EBV) transformed peripheral B cells from a patient with an autoantibody against glycoprotein (GP)Ia/IIa, and manifesting defective collagen-induced platelet aggregation and a bleeding problem. Antibody- producing clones were selected for their reactivity with whole platelets or with affinity-purified GPIa/IIa by enzyme-linked immunosorbent assay (ELISA). One of these cell lines, selected for further evaluation, produced an IgM (E3G6) that interfered with platelet aggregation responses. Polymerase chain reaction (PCR) amplifications with two different sets of primers specific for human kappa-chains resulted in the rescue of a unique and identical sequence. The same was true for the mu-chain, from which it was concluded that the cell line was monoclonal. Further analysis showed that the kappa variable domain sequence is similar to the germline gene A30, to 2E7, an anti-GPIIb human autoantibody, and to HF2–1/17, a systemic lupus erythematosus (SLE)-associated broad-specificity human autoantibody. Thus, the specificity of our antibody, E3G6, appears to be determined by the mu-chain, the sequence of which is encoded by a VHIII gene segment strongly homologous to the germline gene DP-77, by a D gene that is not homologous to any of the germline D genes reported to date, and by JH4 gene segment that is germline. All four mutations versus DP- 77 are in CDRs, and result in amino acid substitutions, which implies that E3G6 may have been derived from an antigen-driven response.

scholarly journals Three Severe Cases of Viral Infections with Post-Kidney Transplantation Successfully Confirmed by Polymerase Chain Reaction and Flow Cytometry

2018 ◽  
Vol 8 (3) ◽  
pp. 198-206
Author(s):  
Keita Nakanishi ◽  
Hiroshi Kaito ◽  
Miki Ogi ◽  
Denshi Takai ◽  
Junya Fujimura ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Polymerase Chain Reaction ◽  
Kidney Transplantation ◽  
Viral Infections ◽  
Specific Treatment ◽  
Epstein Barr Virus Infection ◽  
Chain Reaction ◽  
Barr Virus ◽  
Polymerase Chain ◽  
Epstein Barr

Viral infections in patients with post-kidney transplantation are often difficult to diagnose as well as treat. We herein report three cases with severe viral infections after kidney transplantation. All their causative pathogens could be detected promptly by polymerase chain reaction and flow cytometry during the early stages of infection. These examinations would also be of great use to monitor therapeutic responses and disease activity. It is indeed true that no specific treatment is available for most of the viral infections, but we should be aware that some infections, such as Epstein-Barr virus infection, can be treatable with prompt and specific treatment, such as rituximab.

Epstein–Barr virus infection as a cause of cervical lymphadenopathy in children

2011 ◽  
Vol 75 (4) ◽  
pp. 564-567 ◽  
Author(s):  
Mosaad Abdel-Aziz ◽  
Hassan El-Hoshy ◽  
Mohammed Rashed ◽  
Mohamed Qotb ◽  
Seham Awad ◽  
...  
Keyword(s):  
Virus Infection ◽  
Epstein Barr Virus ◽  
Cervical Lymphadenopathy ◽  
Epstein Barr Virus Infection ◽  
Barr Virus ◽  
Epstein Barr ◽  
Barr Virus Infection

scholarly journals Angioimmunoblastic T-cell lymphoma presenting with an acute serologic Epstein-Barr virus profile

2015 ◽  
Vol 7 (2) ◽  
Author(s):  
Timothy Beer ◽  
Patrick Dorion
Keyword(s):  
T Cell ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
Lymph Node Biopsy ◽  
T Cell Lymphoma ◽  
High Endothelial Venules ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
Barr Virus ◽  
Epstein Barr ◽  
High Index Of Suspicion

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive peripheral T-cell lymphoma typically characterized by prominent lymphadenopathy and B-symptoms at the time of presentation, polyclonal hypergammaglobulinemia, autoimmune hemolysis and frequent but highly variable involvement of Epstein- Barr virus (EBV). Lymph node biopsy findings typically include effacement of nodal architecture, polymorphic infiltrate, atypical T-cells (usually CD4+/CD10+/PD1+) and prominent proliferations of high endothelial venules and follicular dendritic cells. However, this classic constellation of pathologic findings is often initially obscured by a prominence of EBV+ B-immunoblasts with or without associated peripherally circulating EBV DNA. Here we document the first reported case of an acute serologic EBV profile (VCA-IgM) in a patient with AITL, and we recommend that clinicians maintain a high index of suspicion for AITL in the appropriate clinical scenario, irrespective of Epstein-Barr related findings.

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