Posttransplant Lymphoproliferative Disorder in Pediatric Patients

Abstract

Transplantation of solid organs and hematopoietic stem cells is accompanied by profound disturbance of immune function mediated by immunosuppressive drugs or delayed immune reconstitution. Disturbed T cell control of Epstein-Barr virus (EBV)-infected B cells leads to posttransplant lymphoproliferative disorder (PTLD) in up to 10% of patients. Children are at a higher risk because they are more often EBV-naïve before transplantation. Patients with PTLD often present with unspecific symptoms (pain and organ/graft dysfunction). Depending on the onset of PTLD, manifestations vary between mainly nodal (late PTLD) and extranodal sites (early PTLD). Histology, immunohistology, EBER in situ hybridization and molecular pathology are required for diagnosis and subclassification of PTLD. The three major types are early lesions (resembling reactive proliferations in immunocompetent patients), polymorphic PTLD (proliferation of B and T cells with effacement of histoarchitecture) and monomorphic PTLD (presenting as malignant lymphomas, mainly high-grade B cell lymphomas). In a subfraction of cases, including monomorphic PTLD, reduction of immunosuppressive medication alone is sufficient to induce remission. Surgical debulking of tumor mass and anti-CD20-antibody treatment with or without chemotherapy (usually at lower dosages than in immunocompetent patients) constitute the basis of additional therapy.

Epstein-Barr Virus DNAemia in Iranian Liver Transplant Recipients and Assessment of Its Variation in Posttransplant Lymphproliferative Disorder Patients by Quantitative Polymerase Chain Reaction Assay

“…[22][23][24][25][26] In 8 PTLD patients who had serial viral loads available, large variation in maximum load (4370 to 286 844 copies/mL) and time of maximum load (13-360 d after transplant) were observed, which is consistent with other studies. 27,28 Infection with EBV causes a wide range of clinical manifestations including a nonspecific viral syndrome, infectious mononucleosis, and PTLD. 27,29 The PTLD, which is the most important EBVassociated disorder, can be prevented by modulation of the immune system, autologous expanded T-cell infusion, and/or use of anti-EBV drugs such as rituximab.…”

“…27,28 Infection with EBV causes a wide range of clinical manifestations including a nonspecific viral syndrome, infectious mononucleosis, and PTLD. 27,29 The PTLD, which is the most important EBVassociated disorder, can be prevented by modulation of the immune system, autologous expanded T-cell infusion, and/or use of anti-EBV drugs such as rituximab. 12,30 The incidence of PTLD in liver recipients in this study was 4.9% (34 of 696 patients); this is in agreement with other studies (1.6%-15%).…”

Posttransplant Lymphoproliferative Diseases During Childhood: Review

“…[11][12][13][14] Özellikle allojeneik HKHT'de alıcıda HLA uyumsuzluğu varsa, üründe T-hücre deplesyonu yapıldıysa veya hazırlama rejiminde antitimosit globulin kullanılmışsa PTLH sıklığı artmaktadır. [8][9][10] HKHT sonrası PTLH, SOT sonrası PTLH ile karşılaştırıldığında daha az görüldüğü, ancak mortalitesinin daha yüksek olduğu dikkat çekmektedir.…”

“…37 Hastanın transplantasyon yaşı ve transplantasyon öncesinde EBV ile enfekte olma durumu, transplante edilen organın tipi, kullanılan immünsupresif rejim PTLH gelişimini etkilemektedir. [11][12][13][14]37,38,48,49 Transplante edilen greft tipine göre uygulanan immünsupresyon rejimi değişmektedir. Kalp, akciğer ve ince barsak transplantasyonu sonrası daha yoğun immünsupresyon uygulanmakta ve bu grup hastalar PTLH gelişmesi için daha yüksek risk taşı-maktadır.…”

“…Kalp, akciğer ve ince barsak transplantasyonu sonrası daha yoğun immünsupresyon uygulanmakta ve bu grup hastalar PTLH gelişmesi için daha yüksek risk taşı-maktadır. [11][12][13][48][49][50][51][52][53][54][55][56][57][58][59] İmmünsupresyonun derecesi önemli bir faktör olmakla birlikte olası PTLH riski transplantasyonda kullanılacak immünsupresif rejim tercihini etkilememelidir.…”