2022
DOI: 10.3389/fimmu.2022.915986
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Case report: Cellular therapy for hydroa vacciniforme-like lymphoproliferative disorder in pediatric common variable immunodeficiency with chronic active Epstein-Barr virus infection

Abstract: Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at higher risk of developing a severe course of infections especially viral and malignancies than the general population. The aim of the study was to present complex diagnostic and therapeutic management of HV-LPD. The… Show more

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Cited by 6 publications
(4 citation statements)
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“…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%
“…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%
“…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%
“…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%