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 ◽  
...  

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.

2002 ◽  
Vol 74 (3) ◽  
pp. 367-372 ◽  
Author(s):  
Ronald D. Holmes ◽  
Kathy Orban-Eller ◽  
Frederick R. Karrer ◽  
David T. Rowe ◽  
Michael R. Narkewicz ◽  
...  

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
M. Filippini ◽  
S. Cartella ◽  
O. Bonzanini ◽  
E. Morello ◽  
A. Tincani

A 38-year-old woman was referred to our hospital for rheumatologic manifestations (migrant arthritis and tenosynovitis), without psoriasis or family history of psoriasis, gastroenteric manifestations, or recent genitourinary infections. The instrumental and laboratory tests have suggested a diagnosis of undifferentiated seronegative HLA-B27-positive spondyloarthritis with predominantly peripheral involvement. The symptoms were very severe and resistant to anti-inflammatory drugs and steroids. She had a history of hyaline-vascular unicentric Castleman’s disease (HBV, HIV, and HHV-8 negative) treated with surgery resection. After a first pharmacological attempt with sulfasalazine (suspended for urticarial rash), we managed the patient with monotherapy tocilizumab 8 mg/kg, with full response of rheumatologic manifestations. The efficacy of tocilizumab was confirmed even after a follow-up of three years. Our experience seems to describe a new late-onset autoimmune disease (only 21 cases described in literature) potentially related to Castleman’s disease. The patient experienced marked improvement from IL-6-based therapy (tocilizumab).


2013 ◽  
Vol 34 (3) ◽  
pp. 262-264 ◽  
Author(s):  
Heather Gomes ◽  
Phillip Huyett ◽  
Nora Laver ◽  
Richard O. Wein

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Talayeh Rezayat ◽  
Matthew B. Carroll ◽  
Bryan C. Ramsey ◽  
Andria Smith

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.


2011 ◽  
Vol 140 (11) ◽  
pp. 2089-2095 ◽  
Author(s):  
A. E. HWANG ◽  
A. S. HAMILTON ◽  
M. G. COCKBURN ◽  
R. AMBINDER ◽  
J. ZADNICK ◽  
...  

SUMMARYInfectious mononucleosis is a clinical manifestation of primary Epstein–Barr virus infection. It is unknown whether genetic factors contribute to risk. To assess heritability, we compared disease concordance in monozygotic to dizygotic twin pairs from the population-based California Twin Program and assessed the risk to initially unaffected co-twins. One member of 611 and both members of 58 twin pairs reported a history of infectious mononucleosis. Pairwise concordance in monozygotic and dizygotic pairs was respectively 12·1% [standard error (s.e.)=1·9%] and 6·1% (s.e.=1·2%). The relative risk (hazard ratio) of monozygotic compared to dizygotic unaffected co-twins of cases was 1·9 [95% confidence interval (CI) 1·1–3·4, P=0·03], over the follow-up period. When the analysis was restricted to same-sex twin pairs, that estimate was 2·5 (95% CI 1·2–5·3, P=0·02). The results are compatible with a heritable contribution to the risk of infectious mononucleosis.


2014 ◽  
Vol 15 (3) ◽  
pp. 110-111
Author(s):  
Prashilla Soma ◽  
Sita Kara

HIV is not indicated in the aetiology of Castleman’s disease. However, it impacts on the prevalence and natural history of this disease and significantly on the disease progression. Castleman’s disease is a uni- or multicentric disease of the lymph node with or without polyclonal proliferation of B-cells. It is a morphologically distinct form of lymph node hyperplasia and is characterised by significant architectural changes in all lymphatic compartments. Histopathologically, the disease is classified into two major subtypes: the hyaline-vascular type and the plasma-cell type. A mixed type is also identified, as there are frequent transitions between the types. The diagnosis of Castleman’s disease needs to be made histologically. Treatment modalities include surgery, which is curative for unicentric disease, and systemic therapy, which is needed for multicentric disease. This case highlights the diagnostic value of lymph node excision biopsy in HIV-infected patients. 


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Ang Wei ◽  
Honghao Ma ◽  
Liping Zhang ◽  
Zhigang Li ◽  
Yitong Guan ◽  
...  

Abstract Objective To investigate the clinical characteristics, treatment, prognosis and risk factors for chronic active Epstein–Barr Virus infection (CAEBV) associated with coronary artery dilatation (CAD) in children. Methods Children with CAEBV associated with CAD hospitalized at Beijing Children’s Hospital, Capital Medical University from March 2016 to December 2019 were analyzed. Children with CAEBV without CAD were selected as the control group and matched by sex, age, treatment and admission time. The clinical manifestations, laboratory and ultrasound examinations, treatment and prognosis of the children were collected in both groups. Results There were 10 children with CAEBV combined with CAD, including 6 males and 4 females, accounting for 8.9% (10/112) of CAEBV patients in the same period, with an onset age of 6.05 (2.8–14.3) years. The median follow-up time was 20 (6–48) months. All the patients had high copies of EBV-DNA in whole blood [1.18 × 107 (1.90 × 105–3.96 × 107) copies/mL] and plasma [1.81 × 104 (1.54 × 103–1.76 × 106) copies/mL], and all biopsy samples (bone marrow, lymph nodes or liver) were all positive for Epstein–Barr virus-encoded small RNA. Among the 10 children, 8 had bilateral CAD, and 2 patients had unilateral CAD. After diagnosis, 7 children were treated with L-DEP chemotherapy in our hospital. After chemotherapy, four patients underwent allogeneic hematopoietic stem cell transplantation (HSCT). The others were waiting for HSCT. At the time of the last patients follow up record, the CAD had returned to normal in 3 patients, and the time from the diagnosis of CAD to recovery was 21 (18–68) days. LDH, serum ferritin, TNF-α and IL-10 levels were statistically significantly different between the two groups (P = 0.009, 0.008, 0.026 and 0.030). There were no significant differences in survival rate between the two groups (P = 0.416). Conclusion The incidence of CAEBV with CAD was low. CAEBV with CAD did not influence the prognosis. Patients who had high LDH, serum ferritin, TNF-α, and IL-10 levels early in their illness were more likely to develop CAD.


BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wen-Jie Chen ◽  
Wen-Na Xu ◽  
Hai-Yun Wang ◽  
Xiao-Xia Chen ◽  
Xue-Qi Li ◽  
...  

Abstract Objective Plasma Epstein-Barr virus (EBV) DNA is considered a biomarker for nasopharyngeal carcinoma (NPC). However, its long-term role in NPC development is unclear. Materials and methods A total of 1363 participants seropositive for EBV VCA-IgA and EBNA1-IgA in a community-based NPC screening program in southern China were tested for plasma EBV DNA levels by real-time qPCR between 2008 and 2015. New NPC cases were confirmed by active follow-up approach and linkage to local cancer registry through the end of 2016. Cox proportional hazards regression analysis was performed to calculate the hazard ratios (HRs) for NPC risk with plasma EBV DNA. Results Thirty patients were newly diagnosed during a median 7.5 years follow-up. NPC incidence increased with the plasma EBV DNA load ranging from 281.46 to 10,074.47 per 100,000 person-years in participants with undetectable and ≥ 1000 copies/ml levels; the corresponding cumulative incidence rates were 1.73 and 50%. Furthermore, plasma EBV DNA loads conferred an independent risk for NPC development after adjustment for other risk factors, with HRs of 7.63 for > 3–999 copies/ml and 39.79 for ≥1000 copies/ml. However, the HRs decreased gradually after excluding NPC cases detected in the first 2 to 3 years and became statistically nonsignificant by excluding cases detected during the first 4 years. Conclusion Elevated plasma EBV DNA can predict NPC risk over 3 years. Monitoring plasma EBV DNA can be used as a complementary approach to EBV serological antibody-based screening for NPC.


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