scholarly journals Cutaneous Rosai-Dorfman disease: a case report of disease limited only to skin

2021 ◽  
Author(s):  
Aureja Kontrimiene ◽  
Emilija Serpytiene ◽  
Monika Macejevska ◽  
Jonas Lauraitis ◽  
Jurate Grigaitiene

Rosai – Dorfman disease is a disorder of histiocytic proliferation that can affect almost every organ. Skin may be involved in about 10% cases, but only 3% of patients have skin lesions exclusively. Patients with CRDD usually are healthy, without leading constitutional or other symptoms. Etiology of this disease is unclear, but it might be triggered by an infectious agent such as Herpesvirus 6 and Epstein-Barr virus, there is also predisposition to autoimmune diseases, hematological malignancies. In our case patient was diagnosed with Borrelia burgdorferi infection. Any known association between this infection and Rosai – Dorfman disease was not described before. The disease may manifest in any age group. The differential diagnosis includes Langerhans cell histiocytosis, metastasis, Hodgkin’s lymphoma and other diseases. Disease usually resolves spontaneously over a period of months to years. Treatment should be administered in non-responsive or multiple relapsing cases. Also, it is very important to take into consideration the patient’s expectations and aesthetic appearance before making a treatment decision. Various treatment options are described, including surgical excision, cryotherapy, corticosteroids, antibiotics, Methotrexate, Dapsone. In our case, we used CO2 laser treatment successfully. To our knowledge, this is only the second case where CRDD lesions were treated successfully with CO2 laser.

Blood ◽  
2009 ◽  
Vol 114 (19) ◽  
pp. 4002-4008 ◽  
Author(s):  
Helen E. Heslop

Abstract Epstein-Barr virus (EBV)–associated B-cell lymphoproliferation is a life-threatening complication after hematopoietic stem cell or solid organ transplantation resulting from outgrowth of EBV-infected B cells that would normally be controlled by EBV-cytotoxic T cells. During the past decade, early detection strategies, such as serial measurement of EBV-DNA load in peripheral blood samples, have helped to identify high-risk patients and to diagnose early lymphoproliferation. Treatment options include manipulation of the balance between outgrowing EBV-infected B cells and the EBV cytotoxic T lymphocyte response and targeting the B cells with monoclonal antibodies or chemotherapy. Major challenges remain for defining indications for preemptive therapies and integrating novel and conventional therapies.


2020 ◽  
Author(s):  
Aditya Thandoni ◽  
Andrew Zloza ◽  
Devora Schiff ◽  
Malay Rao ◽  
Kwok-wai Lo ◽  
...  

AbstractNasopharyngeal carcinoma (NPC) is a malignancy endemic to East Asia and is caused by Epstein-Barr Virus (EBV)-mediated cancerous transformation of epithelial cells. The standard of care treatment for NPC involves radiation and chemotherapy. While treatment outcomes continue to improve, up to 50% of patients can be expected to recur by five years, and additional innovative treatment options are needed. We posit that a potential way to do this is by targeting the underlying cause of malignant transformation, namely EBV. One method by which EBV escapes immune surveillance is by undergoing latent phase replication, during which EBV expression of immunogenic proteins is reduced. However, chemoradiation is known to drive conversion of EBV from a latent to a lytic phase. This creates an opportunity for the targeting of EBV-infected cells utilizing anti-viral drugs. Indeed, we found that combining acyclovir with cisplatin and radiation significantly decreases the viability of the EBV-infected C666-1 cell line. Western blot quantification revealed a resultant increase of thymidine kinase (TK) and apoptosis-inducing mediators, cleaved PARP (cPARP) and phosphorylated ERK (pERK). These studies suggest that the addition of anti-viral drugs to frontline chemoradiation may improve outcomes in patients treated for EBV-related NPC and future in vivo and clinical studies are needed.


2006 ◽  
Vol 10 (3) ◽  
pp. 131-135 ◽  
Author(s):  
Daisuke Tsuruta ◽  
Yukiko Someda ◽  
Junko Sowa ◽  
Hiromi Kobayashi ◽  
Masamitsu Ishii

Background: Minocycline is a commonly prescribed drug for the treatment of acne. Its use is generally not associated with systemic side effects. Objective: To describe a case of minocycline-induced drug hypersensitivity syndrome in a 20-year-old Japanese woman. Methods and Results: Following 2 months of minocycline treatment, the patient developed skin lesions composed of exudative maculopapules, purpuratous macules, and target-like, erythema multiforme-like plaques over most of her body. In addition, she had fever, abnormal liver function tests, eosinophilia, and atypical lymphocytosis. Laboratory tests indicated no elevation of antibody titers against cytomegalovirus, Epstein-Barr virus, and human herpesvirus 6. Her ongoing exposure to minocycline was stopped, and treatment with oral prednisolone was begun. Her signs, symptoms, and laboratory abnormalities then began to resolve. Subsequently, the syndrome was observed to return briefly in response to an oral challenge with minocycline. Conclusions: Minocycline is able to elicit a drug hypersensitivity syndrome that can resemble infectious mononucleosis. This drug reaction can be treated effectively by cessation of exposure to this drug and steroid therapy.


Medicine ◽  
2016 ◽  
Vol 95 (5) ◽  
pp. e2676 ◽  
Author(s):  
Hai-Juan Xiao ◽  
Ji Li ◽  
Hong-Mei Song ◽  
Zheng-Hong Li ◽  
Mei Dong ◽  
...  

1994 ◽  
Vol 25 (1) ◽  
pp. 88-90 ◽  
Author(s):  
W.Y.W. TSANG ◽  
T.T.C. YIP ◽  
J.K.C. CHAN

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1631-1631
Author(s):  
Ayako Arai ◽  
Ichiro Yonese ◽  
Chizuko Sakashita ◽  
Ken-Ichi Imadome ◽  
Tohru Kobayashi ◽  
...  

Abstract Background and aims Chronic active Epstein-Barr virus infection (CAEBV) is classified into T- or NK-cell neoplasms in the new WHO classification revised in 2017. Allogeneic stem cell transplantation (allo-HSCT) has recently been reported to be an effective treatment for this disorder. Conversely, effects of chemotherapies on CAEBV have not yet been examined in a large number of patients. To clarify clinical features and the current state of chemotherapies for CAEBV under the new definition of the disease, we performed a nationwide survey in Japan. Methods Questionnaires were sent to all educational hospitals certified by the Japanese Society of Hematology and the Japanese Pediatric Society. Subjects were patients newly diagnosed with CAEBV between January 2003 and March 2016. CAEBV was diagnosed according to criteria suggested by the Research group of Measures against Intractable Diseases by Ministry of Health, Labour and Welfare of Japan in 2016: (1) elevated EBV DNA load in peripheral blood (PB) (>102.5 copies/μg DNA); (2) detection of EBV infection in T or NK cells from the affected tissues or PB; (3) systemic inflammatory symptoms (such as fever, lymphadenopathy, liver dysfunction, progressive skin lesions, vasculitis, and uveitis) persisting for >3 months; and (4) exclusion of other possible diagnoses, such as primary EBV infection, autoimmune disease, immunodeficiencies, and lymphomas. Patients who fulfilled (1)-(4) were diagnosed with CAEBV. These criteria were established based on those proposed by Okano et al.(Am J Hematol. 2005;80,p64) and Kimura et al.(Blood. 2012;119,p673) and were compatible with the definition of CAEBV described by WHO in 2017. The disease activity was defined according to the previous reports (Blood. 2012;119,p673 and BMT. 2016;51,p879) as follows: positive for fever, ALT level elevation, vasculitis, progressive skin lesions, or uveitis. Effects of treatments were evaluated as follows: partial response (PR), partial resolution of disease activity; complete response (CR), complete resolution of disease activity with EBV load in PB remaining high (>102.5 copies/μg DNA, which is the upper limit for healthy people); virological CR (vCR), CR with a significant decrease in the EBV DNA load in PB (<102.5 copies/μg DNA). Results Completed questionnaires were returned by 29 institutes and 100 patients were evaluated. They were 53 males and 47 females aged 1-78 (median, 21) years. Types of EBV-infected cells were as follows: CD4 (n = 25), CD8 (n = 13), and CD56 (n = 28). The 3-year overall survival (OS) from diagnosis was 58%. Because different outcomes have been reported between children and adults with CAEBV, we divided patients into three groups according to their onset ages: childhood onset (CO) with patients aged <9 years, adolescent- and adult-onset (AAO), and advanced age-onset (AO) with patients aged >45 years. Seventy-eight percent patients in the CO group were males. Conversely, 85% patients in the AO group were females. The 3-year OS from diagnosis in the CO, AAO, and AO groups were 83%, 57%, and 31%, respectively. The prognosis of CO of CAEBV was significantly better than that of AAO (p = 0.0151) and AO (p = 0.0034) of CAEBV. Spontaneous regression was not observed in patients with active disease. Main chemotherapies employed were a combination of cyclosporine A, steroids, and etoposide (cooling therapy) in 52 patients and CHOP in 45 patients. The rate of CR + PR was as follows: cooling therapy, 57% and CHOP, 39%. Viral CR was not observed. Three-year OS from the start of treatment was 0% in patients treated with only chemotherapy (n = 20) and 69% in those who received allo-HSCT (n = 59). Conclusions Based on the difference in OS and increased prevalence of males in the CO group, it can be concluded that CO of CAEBV may be independent from AO and AAO of CAEBV. Chemotherapy alone is currently insufficient for eradicating infected cells and resolving CAEBV. The development of an effective treatment reagent is an urgent issue. Disclosures No relevant conflicts of interest to declare.


2019 ◽  
Author(s):  
◽  
Shirley Chu

The remarkable advancements in sequencing technologies have allowed the entire mutational landscape of hundreds of different types of human cancers to be defined. This knowledge gives patients two new categories of treatment options, small molecule inhibitors and targeted immunotherapy. Veterinary oncology has been slow to enter the field of genome-wide studies, largely due to costs. Five years ago, the first whole genome of a canine cancer patient was published. Because of NIH support and the steady decline in sequencing costs, the number of published canine tumor sequences has exploded this past year as these projects are being concluded. This dissertation contributes to this growing knowledge. The scope was diverse and included exploration in metagenomics, epigenomics and transcriptomics in canine and feline cancers. This research affirmed previous notions that papillomavirus is not a common cause of feline oral squamous cell carcinoma (FOSCC) and added to the understanding that it is not caused by any other DNA or integrated dsRNA virus. In canin DLCBL (cDLBCL) we provided more evidence that the subgroups seen in human DLBCL (hDLBCL) cannot be faithfully reproduced in the methylome of cDLCBL, likely at a greater scale than DNA mutations, and neurogenic/neuroendocrine, HOX and Wnt pathways were epigenetically targeted. In canin osteosarcoma (OSA) we built upon the genome model by adding loss of heterozygosity data and illustration of metastatic lesions. We also provided support to target the CDK4, Wnt and mTOR pathways. Finally we highlight unexpected discoveries such as the discovery of Epstein Barr virus in FOSCC, stability of the methylome in Golden Retriever DLBCL, epimutations in the TBX pathway in cDLBCL, dysregulation of human melanoma pathways in canine OSA, absence of dysregulation of HER2 in canine OSA, absence of genomic evidence of a telomere maintenance mechanism, recurrent shattering of chr26 in canine OSA and HSP90AB1, MITF and NOTCH2, as potentially actionable genes. These data will provide a significant resource for the community to generate new hypotheses and answer key questions about the molecular drivers of canine and feline malignancies.


2019 ◽  
Vol 12 (7) ◽  
pp. e229520 ◽  
Author(s):  
Sofia V Gearty ◽  
Ayman Al Jurdi ◽  
Meredith E Pittman ◽  
Renuka Gupta

Epstein-Barr virus (EBV) is implicated in the tumorigenesis of a variety of malignancies, including Burkitt’s lymphoma, Hodgkin’s disease and nasopharyngeal carcinoma (NPC). EBV+ lymphoepithelioma-like cholangiocarcinoma (LELCC) is a rare type of intrahepatic cholangiocarcinoma with a distinct pathology and poorly understood treatment options. Morphologically, this neoplasm resembles undifferentiated NPC, a commonly EBV+ tumour with a prominent lymphoid infiltrate. Almost all of the current literature regarding LELCC describes early stage tumours that are treated surgically and achieve good outcomes. In contrast, this report documents a late stage LELCC treated unsuccessfully with systemic chemotherapy.


2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Sajal K. Ghosh ◽  
Susan P. Perrine ◽  
Douglas V. Faller

Epstein-Barr virus (EBV) is the causal agent in the etiology of Burkitt’s lymphoma and nasopharyngeal carcinoma and is also associated with multiple human malignancies, including Hodgkin’s and non-Hodgkin’s lymphoma, and posttransplantation lymphoproliferative disease, as well as sporadic cancers of other tissues. A causal relationship of EBV to these latter malignancies remains controversial, although the episomic EBV genome in most of these cancers is clonal, suggesting infection very early in the development of the tumor and a possible role for EBV in the genesis of these diseases. Furthermore, the prognosis of these tumors is invariably poor when EBV is present, compared to their EBV-negative counterparts. The physical presence of EBV in these tumors represents a potential “tumor-specific” target for therapeutic approaches. While treatment options for other types of herpesvirus infections have evolved and improved over the last two decades, however, therapies directed at EBV have lagged. A major constraint to pharmacological intervention is the shift from lytic infection to a latent pattern of gene expression, which persists in those tumors associated with the virus. In this paper we provide a brief account of new virus-targeted therapeutic approaches against EBV-associated malignancies.


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