Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection

Grześk, Elżbieta; Kołtan, Sylwia; Dąbrowska, Anna; Urbańczyk, Anna; Małdyk, Jadwiga; Małkowski, Bogdan; Bogiel, Tomasz; Dębski, Robert; Czyżewski, K; Wysocki, Mariusz; Styczyński, Jan

doi:10.3389/fimmu.2022.915986

Cited by 6 publications

(4 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The viremia of EBV was constantly increasing over the months. Finally, he was diagnosed with hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD), as was described previously [4]. The patient was treated with rituximab, bortezomib and gancyclovir.…”

Section: Patientmentioning

confidence: 77%

Sclerodermatous manifestation of chronic graft versus host disease: therapy challenges

Richert-Przygońska,

Milewska,

Dębski

et al. 2023

Acta Haematol Pol.

View full text Add to dashboard Cite

Chronic graft-versus-host disease (cGvHD) is one of the most serious complications after allogeneic hematopoietic cell transplantation (allo-HCT). It varies between patients, often leading to systemic and functional limitations. It can be challenging considering the heterogeneity of patients. The aim of this paper was to present clinical aspects of sclerodermatous manifestation of cGvHD as a complication of allo-HCT in pediatric patients. We diagnosed three patients with different sclerodermatous skin involvement and cGvHD features. The treatment applied varied among the patients, and was based on currently available standards of care. We analyzed the effectiveness of systemic steroid therapy, extracorporeal photopheresis, and ruxolitinib.All patients achieved an improvement in skin lesions and quality of life, based on an individualized treatment approach.

show abstract

Section: Patientmentioning

confidence: 77%

Sclerodermatous manifestation of chronic graft versus host disease: therapy challenges

Richert-Przygońska,

Milewska,

Dębski

et al. 2023

Acta Haematol Pol.

View full text Add to dashboard Cite

show abstract

“…As in the majority of studies published over the last decade, 20-30% of EBV-PTLD patients are not cured with rituximab in the HCT setting, it strongly underlines the emerging need for the use of newer therapies in refractory PTLD ( 14 ). EBV-CTLs are at the highest hope for standard use ( 39 – 43 ), although other therapies have been reported in clinical practice both in hematopoietic and solid organ transplant settings ( 44 – 48 ).…”

Section: Discussionmentioning

confidence: 99%

Impact of prophylaxis with rituximab on EBV-related complications after allogeneic hematopoietic cell transplantation in children

Marjańska,

Pogorzała,

Dziedzic

et al. 2024

Front. Immunol.

Self Cite

View full text Add to dashboard Cite

BackgroundChildren undergoing allo-HCT are at high risk of EBV-related complications. The objective of the study was to analyze the impact of prophylactic post-transplant rituximab on EBV infection and EBV-PTLD in children after allo-HCT, to determine the risk factors for the development of EBV infection and EBV-PTLD and to determine their outcomes. Additionally, the impact of EBV-driven complications on transplant outcomes was analyzed.MethodsSingle center retrospective analysis of EBV-related complications in pediatric population undergoing allo-HCT, based on strategy of prophylaxis with rituximab. Overall 276 consecutive children, including 122 on prophylaxis, were analyzed for EBV-driven complications and transplant outcomes.ResultsProphylaxis with rituximab resulted in significant reduction of EBV infection (from 35.1% to 20.5%; HR=2.7; p<0.0001), and EBV-PTLD (from 13.0% to 3.3%; HR=0.23; p=0.0045). A trend for improved survival was also observed (HR=0.66; p=0.068), while non-relapse mortality was comparable in both cohorts. The peak value of viral load was a risk factor in the development of EBV-PTLD: 10-fold higher peak viral load in comparison to the baseline 104 copies/mL, caused a 3-fold (HR=3.36; p<0.001) increase in the risk of EBV-PTLD. Rituximab treatment was effective as a preemptive therapy in 91.1%, and in 70.9% in EBV-PTLD. Patients who developed PTLD had dismal 5-year overall survival (29% vs 60%; p<0.001), and an increased risk of relapse (72% vs 35%; p=0.024).ConclusionsRituximab for prophylaxis of EBV infection and EBV-PTLD was highly effective in pediatric population. Treatment of EBV-PTLD was successful in 70%, however the occurrence of EBV-PTLD was associated with an increased risk of relapse of primary malignant disease.

show abstract

“…The current literature also reflects EBV-associated LPD involving the skin in patients with HIV and primary immunodeficiency, but the morphology of these lesions is not wellreported. [17][18][19] As the cases herein are presently best classified within the WHO framework of lymphoid proliferations and lymphomas associated with immune deficiency and dysregulation (Table 3), they emphasize the importance of proper workup and histopathological assessment to avoid misdiagnosing patients with LyP. 2,[20][21][22] This case series reveals that EBV-positive LPD with a presentation resembling LyP could be a cardinal sign of insidious immunodeficiency in patients without a known history concerning for immune dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Epstein–Barr Virus–Associated Lymphomatoid Papules: A Sign of Immunosuppression Resembling Lymphomatoid Papulosis

Hooper,

Lee,

LeWitt

et al. 2023

The American Journal of Dermatopathology

View full text Add to dashboard Cite

Epstein–Barr virus (EBV)–positive lymphoproliferative disorders associated with immunodeficiency constitute a spectrum of lymphoid and plasma cell proliferations that vary in cytomorphology, immunophenotype, and clinical behavior. CD30-positive cutaneous lymphocytic infiltrates with EBV expression and lymphomatoid papulosis–like presentations have been rarely reported. This retrospective study assessed the clinical and histopathological characteristics of EBV-positive cases with papulonodular morphologies and CD30 positivity seen by Northwestern Medicine Dermatopathology. Twelve patients (7M:5F, mean age 69 years) were presented with papular cutaneous lesions without antecedent patch/plaque disease. Nine cases were associated with known immunosuppression in the setting of transplant-related therapies (n = 4), hematopoietic malignancy (n = 2), post-transplant hematopoietic malignancy (n = 1), and autoimmune disease treatment (n = 2). Two patients had age-related immunosenescence. Four patients demonstrated EBV viremia; for 2 patients, this finding comprised the first sign of immunosuppression. Workup was negative for systemic lymphoma in all patients. Various treatment strategies were used, including observation (n = 3), discontinuation/reduction of immunosuppression (n = 3), rituximab (n = 4), and steroids (n = 4). At mean 30-month follow-up, 4 patients (33.3%) were alive, 3 with and 1 without disease. Eight patients (67.6%) had died, 3 after lesional resolution and 5 with recurrent disease. Biopsies revealed mixed lymphoid infiltrates composed of atypical CD30-positive T cells (n = 5) or B cells (n = 7) with variable EBV-encoded small RNA expression. These cases suggest clinicopathologic presentations resembling lymphomatoid papulosis with atypical, large CD30-positive, EBV-positive cells could comprise first sign of potentially serious immunodeficiency and should prompt evaluation for EBV viremia. These cases also broaden the current picture of immunodeficiency-associated lymphoproliferative disorders to include lymphomatoid papulosis–like clinical presentations.

show abstract

Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection

Cited by 6 publications

References 25 publications

Sclerodermatous manifestation of chronic graft versus host disease: therapy challenges

Sclerodermatous manifestation of chronic graft versus host disease: therapy challenges

Impact of prophylaxis with rituximab on EBV-related complications after allogeneic hematopoietic cell transplantation in children

Epstein–Barr Virus–Associated Lymphomatoid Papules: A Sign of Immunosuppression Resembling Lymphomatoid Papulosis

Contact Info

Product

Resources

About