Indicación de amigdalectomía en pacientes pediátricos por sospecha de trastorno linfoproliferativo postrasplante / Indication for tonsillectomy in pediatric patients with suspected post-transplant lymphoproliferative disorder

Un sitio común de hiperplasia linfoidea en los trastornos linfoproliferativos postrasplante (TLPT) son las amígdalas palatinas. Sin embargo, la hipertrofia amigdalina es extremadamente común en niños, lo que dificulta la sospecha de estos trastornos. Se realizó un estudio de una serie de casos de pacientes trasplantados intervenidos de amigdalectomía por sospecha de TLPT en un hospital pediátrico de alta complejidad en Argentina desde enero de 2014 hasta diciembre de 2021. El objetivo de este trabajo es exponer las características clínicas de los pacientes trasplantados a los que se les indicó amigdalectomía con fin diagnóstico de TLPT.

A common site of lymphoid hyperplasia in post-transplant lymphoproliferative disorders (PTLD) is the palatine tonsils. However, tonsillar hypertrophy is extremely common in children, which hinders the suspicion of PTLD. A case series of transplanted patients undergoing tonsillectomy for suspected PTLD was conducted at a tertiary care children's hospital in Argentina between January 2014 and December 2021. The objective of this study is to expose the clinical characteristics of transplanted patients who underwent a tonsillectomy to diagnose PTLD

Lymphoproliferative disorders in the lacrimal caruncle: report of three cases and review of literature.

This report included three cases of lymphoproliferative disorders developing from the lacrimal caruncle. The first case was an 11-year-old boy with reactive lymphoid hyperplasia in the left lacrimal caruncle. The second case was an 80-year-old woman with reactive lymphoid hyperplasia in the right lacrimal caruncle. The third case was a 77-year-old man with follicular lymphoma in the left lacrimal caruncle. Our literature review of cases with lacrimal caruncular lesions showed 11 reported cases with reactive lymphoid hyperplasia and 17 with malignant lymphoma. There had been no previous report on follicular lymphoma in the lacrimal caruncle.

Indication for tonsillectomy in pediatric patients with suspected post-transplant lymphoproliferative disorder. / Indicación de amigdalectomía en pacientes pediátricos por sospecha de trastorno linfoproliferativo postrasplante.

A common site of lymphoid hyperplasia in post-transplant lymphoproliferative disorders (PTLD) is the palatine tonsils. However, tonsillar hypertrophy is extremely common in children, which hinders the suspicion of PTLD. A case series of transplanted patients undergoing tonsillectomy for suspected PTLD was conducted at a tertiary care children's hospital in Argentina between January 2014 and December 2021. The objective of this study is to expose the clinical characteristics of transplanted patients who underwent a tonsillectomy to diagnose PTLD.

Un sitio común de hiperplasia linfoidea en los trastornos linfoproliferativos postrasplante (TLPT) son las amígdalas palatinas. Sin embargo, la hipertrofia amigdalina es extremadamente común en niños, lo que dificulta la sospecha de estos trastornos. Se realizó un estudio de una serie de casos de pacientes trasplantados intervenidos de amigdalectomía por sospecha de TLPT en un hospital pediátrico de alta complejidad en Argentina desde enero de 2014 hasta diciembre de 2021. El objetivo de este trabajo es exponer las características clínicas de los pacientes trasplantados a los que se les indicó amigdalectomía con fin diagnóstico de TLPT.

Surgical, Dermatological and Morphopathological Considerations in the Diagnosis and Treatment of Cutaneous Lymphoproliferative Tumors, Primary CD4.

Primary cutaneous lymphomas are a heterogeneous group of T-cell (CTCL) and B-cell lymphomas (CBCL) developing in the skin and without signs of extracutaneous disease at the time of diagnosis. The term "primary small/medium CD4+ T-cell lymphoma" was changed to "primary small/medium cutaneous CD4+ lymphoproliferative disorder" due to its indolent clinical behavior and uncertain malignant potential. This paper presents a rare case of primary cutaneous lymphoma with small to medium CD4+ T-cells. A 37-year-old patient presented with a tumor in the frontal region that had occurred approximately 8-9 months earlier. The tumor had a diameter of about 8-9 mm, well demarcated macroscopically, it was round in shape, about 6-7 mm high, pink in color, firm in consistency and painless during palpation. Surgical excision of the tumor was performed with a margin of safety of 8 mm and deep to the level of the frontal muscle fascia. The histopathological examination supported the diagnosis of cutaneous lymphoproliferation with a nodular disposition in the reticular dermis and extension around the follicular epithelia and sweat glands, composed mainly of dispersed medium-large lymphocytes. Additional immunohistochemical examination was requested. Immunohistochemical examination confirmed the diagnosis of "primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder." Patient monitoring was carried out through clinical dermatological controls at 3, 6, and 12 months. After one year, a cranio-cerebral MRI was performed. For the following 5 years, an annual dermatological examination accompanied by cranio-cerebral MRI, blood count, and pulmonary X-ray were recommended. Similarly to all solitary skin lesions, the prognosis is excellent in this case, the only treatment being surgical excision.

Role of antithymocyte globulin in matched sibling donor peripheral blood stem cell transplantation for hematologic malignancies.

BACKGROUND: High incidence of chronic graft-versus-host disease (GVHD) has been a major drawback of matched sibling donor peripheral blood stem cell transplantation (MSD -PBSCT). This study aimed to investigate the safety and efficacy of antithymocyte globulin (ATG) as a standardized part of GVHD prophylaxis in patients receiving MSD -PBSCT. METHODS: A total of 72 patients with hematological malignancies receiving MSD -PBSCT who displayed similar baseline characteristics were either given rabbit ATG ( n = 42) or no ATG (n = 30), in addition to cyclosporine, methotrexate, and mycophenolate mofetil as a standard GVHD prophylaxis regimen. Either patients or donors aged ≥40 years were included in the study. Thymoglobulin was administered at a daily dose of 1.5 mg/kg on day -5 and 3.5 mg/kg on day -4 prior to transplant (the total dose was 5 mg/kg). RESULTS: After a median follow-up of 874 days, the 3-year cumulative incidence of chronic GVHD (cGVHD) was 37.3% in the ATG group and 52.1% in the non -ATG group. The 3-year overall and disease-free survival probability were 71.0% and 62.0% (ATG versus non -ATG, P = .262) and 66.7% and 58.4% (ATG versus non -ATG, P = .334). No difference was found in the 2-year cumulative incidence of nonrelapse mortality and relapse between the ATG and non -ATG groups. This significant reduction in the incidence of cGVHD without increased relapse risk and nonrelapse mortality led to a 3-year GVHD-free, relapse-free survival probability of 66.7% and 40.0% in the ATG and non-ATG groups, respectively. CONCLUSIONS: These data suggested that rabbit antithymocyte globulin in the current protocol for GVHD prophylaxis was well tolerable and efficacious.The clinical trial was registered on January 1, 2016 (ClinicalTrials.gov Identifier NCT02677181). https://clinicaltrials.gov/ct2/show/NCT02677181.

Post-transplant Lymphoproliferative Disorder in Recipient's Reconstructed Middle Hepatic Vein After Pediatric Living Donor Liver Transplant: A Case Report.

BACKGROUND: Post-transplant lymphoproliferative disorder (PTLD) is known as one of the most frequent post-transplant neoplastic diseases, which may lead to recipient and graft morbidity after liver transplant. PATIENT: A 14-year-old boy received pediatric living donor liver transplant (right living graft) 29 months ago, with etiology of biliary atresia. During 22-month follow-up after transplant, computed tomography and positron emission tomography with computed tomography scan showed a single progressive mass locating in the graft cutting surface with occlusive reconstructed middle hepatic vein (MHV). Serology was negative for Epstein-Barr virus serology. Mild fever and pneumonia did not improve after 1-week intravenous antibiotics. PTLD was considered. RESULTS: A surgical resection was scheduled and performed in compliance with the Helsinki Congress and the Istanbul Declaration. During laparotomy, a single mass located in the reconstructed MHV from segment V to the inferior vena cava was confirmed. Postoperative immunohistochemical result showed CD 3(+), CD 20(+), CD 38(+), CD 10(-), CD 56(-), Ki-67(+, 20%-30%), Epstein-Barr virus-encoded RNA(-), and Epstein-Barr virus nuclear antigen 2(-). Polymorphic PTLD was eventually diagnosed. No recurrence or new set lesions were detected after 6-month follow-up. CONCLUSIONS: This is the first case describing PTLD may originate from reconstructed MHV after pediatric living donor liver transplant. As a life-threatening complication of liver transplant, surgical resection should be considered as a safe and feasible treatment for the single resectable mass.

Pérdida de agudeza visual progresiva en paciente con proptosis. Caso clínico y revisión de la literatura / Progressive visual impairment in a patient with proptosis. Case report and review of the literature.

Introducción: a raíz del siguiente reporte de caso clínico se pretende repensar el diagnóstico diferencial de los tumores orbitales y revisar la literatura existente al respecto. Caso: paciente de 54 años, fumadora, acude a nuestro centro por una pérdida de agudeza visual progresiva de dos años de evolución en el ojo derecho, que se acompañaba de proptosis. Las pruebas de imagen basadas en resonancia magnética y tomografía por emisión de positrones ­ tomografía computarizada (PET-TC) realizadas describían una lesión intraconal derecha de morfología indefinida, que rodeaba el nervio óptico. El estudio inmunohistoquímico y molecular anatomopatológico confirmó la sospecha de síndrome linfoproliferativo extranodal de bajo grado. Discusión: el manejo endoscópico de estas lesiones puede resultar en una menor comorbilidad en comparación con el abordaje externo tradicional. El papel de la cirugía radica en la obtención de una muestra de la lesión que permita un correcto diagnóstico. Conclusiones: el abordaje multidisciplinar con oftalmólogos, hematólogos y expertos en radioterapia permite obtener buenos resultados quirúrgicos y clínicos en la inmensa mayoría de casos.

Introduction: as result of the following clinical case report, we intend to review the differential diagnosis of orbital tumors and review the existing literature in this regard. Case report: a 54-year-old smoking patient, consulted to our department due to a progressive visual impairment over the last two years in her right eye. She presented proptosis in her clinical examination. Imaging studies based on MRI and PET-CT described a right intraconal lesion with an undefined morphology surrounding the optic nerve. Orbital tumors differential diagnosis is delicate. Nevertheless, Non-Hodgkin lymphomas followed by metastasis are the two most common found in this location. The immunohistochemistry and molecular studies, confirmed the suspected diagnosis of extranodal low-grade lymphoproliferative syndrome. Discussion: endoscopic management of these lesions may result in a lower comorbidity compared to traditional external approaches. Role of surgery lays in obtainment of a quality sample which allows a proper diagnosis. Conclusions: multidisciplinary approach with ophthalmologists, hematologists and radiotherapy experts enhance good surgical and clinical results in the vast majority of cases.

An Enteropathy-like Indolent NK-Cell Proliferation Presenting in the Female Genital Tract.

Natural killer (NK) cell enteropathy is a lymphoproliferative disorder, initially described by Mansoor and colleagues, that presents in the gastrointestinal tract, and is often mistaken for extranodal NK/T-cell lymphoma on first assessment. This population of cells in this process have an NK-cell phenotype (CD3, CD56, CD2, CD7), lacks evidence of Epstein-Barr virus infection, has germline rearrangement of the T-cell receptor, and a very indolent clinical course. Indeed, many of such patients had been originally diagnosed as having an NK/T-cell lymphoma, and subsequently received chemotherapy. We report a unique case where an indolent lymphoproliferative disorder with features that resemble NK-cell enteropathy is encountered for the first time outside the gastrointestinal tract, specifically in the female genitourinary tract. We provide morphologic, immunophenotypic, and molecular documentation of such, in association with a completely indolent clinical behavior of this type of process.

X-linked lymphoproliferative syndrome in mainland China: review of clinical, genetic, and immunological characteristic.

X-linked lymphoproliferative syndrome (XLP) is a rare primary immunodeficiency disease that can be divided into two types: SAP deficiency (XLP1) and XIAP deficiency (XLP2), caused by mutations in the SH2D1A and XIAP genes, respectively. Few cases of XLP (particularly XIAP deficiency) have been reported in mainland China; hence, little is known about the characteristics of Chinese patients with XLP. We identified 13 and 7 patients with SAP and XIAP deficiency, respectively, in our center. Of our 20 patients, 19/20 (95%) presented with disease symptoms at a very early age: six in infancy and 13 in childhood. One XIAP- and three SAP-deficient patients died, while 3/7(42.9%) and 4/13(30.8%), respectively, developed hemophagocytic lymphohistiocytosis (HLH). Epstein-Barr virus (EBV) infection was significantly more common in SAP-deficient 10/13 (76.9%) than XIAP-deficient 2/7 (28.6%) patients, as was hypogammaglobulinemia (10/13 (76.9%) vs. 1/7 (14.3%)). None of the seven XIAP-deficient patients had colitis or lymphoma. Nine SAP-deficient patients and five XIAP-deficient patients showed markedly deficient SAP and XIAP expression, respectively, in lymphocytes. Significantly reduced levels of switched memory B cells were observed in six SAP-deficient patients with persistent hypogammaglobulinemia. One of 13 (7.7%) SAP-deficient patients and 1 of 7 (12.3%) XIAP-deficient patients have received HSCT treatment and are now alive and well; the other alive patients were waiting for HSCT. We also summarized clinical, genetic, and immunological characteristics of all 55 patients (including our 20 patients) reported in the literature in mainland China today.Conclusion: The overall characteristics of SAP deficiency in mainland China were consistent with those in previous reports, whereas manifestations of XIAP deficiency varied significantly. None of inflammatory bowel disease (IBD) has been reported among XIAP-deficient patients in our center; however, whether Chinese XIAP-deficient patients will develop colitis in the future warrants further investigation. HSCT is the only curative therapy for XLP and this therapy should be urgently considered.What is Known:• SAP and XIAP deficiencies share common clinical feature, HLH, whereas they also have their own specific manifestations.• IBD affects 25-30% of XIAP-deficient patients, which has been reported in other countries especially in European country and Japan.What is New:• This is the largest patient cohort study of XLP in China.• We firstly summarized the clinical features and outcomes of Chinese XIAP-deficient patients and found only 1 in 22 patients developed IBD and diet background may contribute to it; Asian SAP-deficient patients carrying SH2D1A R55X mutation were more prone to HLH.

Long-term outcomes of pediatric kidney transplant recipients with a pretransplant malignancy.

A childhood malignancy can rarely progress to ESRD requiring a KT. To date, few reports describe long-term outcomes of pediatric KT recipients with a pretransplant malignancy. Between 1963 and 2015, 884 pediatric (age: 0-17 years old) recipients received 1055 KTs at our institution. KT outcomes were analyzed in children with a pretransplant malignancy. We identified 14 patients who had a pretransplant malignancy prior to KT; the majority were <10 years old at the time of KT. Ten (71%) patients received their grafts from living donors, the majority of which were related to the recipient. Wilms' tumor was the dominant type of pretransplant malignancy, seen in 50% of patients. The other pretransplant malignancy types were EBV-positive lymphoproliferative disorders, non-EBV-positive lymphoma, leukemia, neuroblastoma, soft-tissue sarcoma, and ovarian cancer. Ten of the 14 patients received chemotherapy as part of their pretransplant malignancy treatment. Graft survival at 1, 3, and 5 years was 93%, 83%, and 72%, respectively. Patient survival at 1, 5, and 10 years was 100%, 91%, and 83%, respectively. Six (40%) patients suffered AR following KT; half of them had their first episode of AR within 1 month of KT. Our single-center experience demonstrates that pediatric KT recipients with a previously treated pretransplant malignancy did not exhibit worse outcomes than other pediatric KT patients.

Gastric Perforation due to Iatrogenic Immunodeficiency-associated Lymphoproliferative Disorder during the Treatment of Rheumatoid Arthritis.

A 71-year-old woman being treated with methotrexate (MTX) and tacrolimus (TAC) for rheumatoid arthritis (RA) was admitted to our hospital and underwent surgery for gastric perforation and peritonitis. An endoscopic examination six days post-surgery showed an extensive ulcer in the stomach, and a biopsy revealed diffused large B-cell lymphoma. We diagnosed her with immunodeficiency-associated lymphoproliferative disorder (LPD) and discontinued the MTX and TAC. She underwent gastrectomy due to stenosis approximately two months after the first operation, but the histopathological findings of lymphoma had disappeared. LPD should be considered as a potential cause of gastric perforation during RA treatment.

Radiological and clinical findings of isolated meningeal Rosai-Dorfman disease of the central nervous system.

Rosai-Dorfman disease (RDD) with isolated central nervous system (CNS) involvement is an extremely rare disease. Most RDD of the CNS present as dural-based mass mimicking meningioma and other common lesions, which makes preoperative accurate diagnosis of great difficulty. We searched the pathology database in our hospital and 3 cases of RDD with isolated CNS involvement were finally included in our study. Radiological and clinical findings of these three cases were retrospectively analyzed. The lesions of 2 cases were dura-based against the cerebral convexity, presenting as a sheet-shaped thickened dura mater, another case was located just across the cerebral falx, the dural display in the center was intact. The 3 cases showed low signal intensity on T2-weighted image, obviously enhanced, significantly surrounding edema and finger-like protuberance but no invasion of the brain parenchyma or no sign of hyperplasia or sclerosis of the surrounding cranial bones. In conclusion, when we come across a disease that mimicking meningioma, especially when it manifests as the above radiological features, we should considered it might be a kind of proliferative disease of the meninges, such as RDD.

Adolescent male fertility following reduced-intensity conditioning regimen for hematopoietic stem cell transplantation in non-malignant disorders.

INTRODUCTION: The effects of RIC for HSCT on male fertility remain unknown. We investigated spermatogenesis and gonadal hormonal status among adolescent male patients who received RIC HSCT for non-malignant diseases. PATIENTS AND METHODS: Patients with non-malignant disease who had undergone a RIC HSCT were recruited and evaluated for spermatogenesis via semen analysis and gonadal hormonal function via serum hormone levels. Those who had received prior chemotherapy or radiation were excluded from the study. We reviewed the charts to record demographic factors, conditioning regimen and complications during and after transplant. RESULTS: Five patients were enrolled. The median age at the time of transplant was 15 years (range, 11-19 years), and the median time between bone marrow transplant and semen analysis was 5 years (range, 3-11 years). Median age of patients was 20 years (range, 18-25 years) at the time of the study. Serum FSH and LH levels were elevated in four patients, and inhibin B levels were low for age in three patients. Semen analysis showed two patients had azoospermia, and the remaining three patients showed severe oligozoospermia. Normal morphology and motility were seen in only one patient. CONCLUSION: This case series suggests that RIC transplants may be associated with impaired spermatogenesis and sequential follow-up is necessary given the potential for either permanent impairment or delayed recovery. Further larger studies are needed to confirm these findings.

Characteristics, risks, and outcomes of post-transplant lymphoproliferative disease >3 years after pediatric heart transplant: A multicenter analysis.

Post-transplant lymphoproliferative disorder (PTLD) is a significant complication after pediatric heart transplantation (HT), occurring in 5%-15% of patients within 3 years. Data >3 years from HT are limited. We sought to describe the prevalence, risk factors, and outcomes of PTLD occurring late (>3 years) after pediatric HT in the Pediatric Heart Transplant Study from 1993 to 2010. Among 3844 primary HT patients, 110 (3%) developed late, nonrecurrent PTLD. The hazard rate for late PTLD was constant at 0.01 events/year out to 20 years after HT. Risk factors for late PTLD were younger age at HT (HR 1.06, P = 0.003) and Epstein-Barr virus (EBV) naivety (HR 1.65, P = 0.02). Survival after late PTLD was 86% and 68% at 1 and 5 years, with nonwhite race (HR 2.27, P = 0.03) and earlier year of HT (HR 1.03, P = 0.04) independently associated with mortality. Acute rejection and infection were both common after late PTLD, occurring in 26% and 34% of patients. The constant late hazard and contribution of EBV to late PTLD suggest that vigilance for development of PTLD, including for EBV conversion, should persist indefinitely after pediatric HT. The reasons for elevated risk of death for nonwhites after late PTLD are unclear and warrant further investigation.

Cardiac Methotrexate-Induced Lymphoproliferative Disorder.

Methotrexate has been reported as an immunosuppressive agent associated with lymphoproliferative disorders. This report describes the case of a cardiac methotrexate-induced lymphoproliferative disorder that could be differentiated from a sinus of Valsalva aneurysm rupture by cardiac magnetic resonance imaging and fluorine-18 (18F)-fluorodeoxyglucose positron emission tomography combined with computed tomography. The definitive diagnosis was made by a tissue biopsy that was concomitantly performed with sinus of Valsalva aneurysm repair. Significant regression was seen in response to methotrexate withdrawal. To the best of our knowledge, this is the first case of a cardiac methotrexate-induced lymphoproliferative disorder.

The Emerging Role of Liquid Biopsies in Lymphoproliferative Disorders.

PURPOSE OF THE REVIEW: Lymphomas represent clinically and molecularly heterogeneous diseases with variable presentations, treatment algorithms, and outcomes. As treatment options continue to expand, more sophisticated prognostic and predictive biomarkers are needed to guide personalized treatment approaches. RECENT FINDINGS: Liquid biopsies, in which the sequencing of circulating tumor DNA (ctDNA) in peripheral blood serves as a surrogate for a tumor biopsy, are now being studied across cancer subtypes, including in lymphoid malignancies. Recent studies have demonstrated the potential of these techniques to improve prognostication and guide individualized treatment strategies, providing a significant advance in the field of precision medicine. In this review, we describe the sequencing platforms currently available for analysis of ctDNA in lymphoma and their potential applications in clinical practice, which seem poised to refine treatment paradigms across lymphoma subtypes.

Metagenomic analysis of DNA viruses from posttransplant lymphoproliferative disorders.

Posttransplant lymphoproliferative disorders (PTLDs), 50%-80% of which are strongly associated with Epstein-Barr virus (EBV), carry a high morbidity and mortality. Most clinical/epidemiological/tumor characteristics do not consistently associate with worse patient survival, so our aim was to identify if other viral genomic characteristics associated better with survival. We extracted DNA from stored paraffin-embedded PTLD tissues at our center, identified viral sequences by metagenomic shotgun sequencing (MSS), and analyzed the data in relation to clinical outcomes. Our study population comprised 69 PTLD tissue samples collected between 1991 and 2015 from 60 subjects. Nucleotide sequences from at least one virus were detected by MSS in 86% (59/69) of the tissues (EBV in 61%, anelloviruses 52%, gammapapillomaviruses 14%, CMV 7%, and HSV in 3%). No viruses were present in higher proportion in EBV-negative PTLD (compared to EBV-positive PTLD). In univariable analysis, death within 5 years of PTLD diagnosis was associated with anellovirus (P = 0.037) and gammapapillomavirus (P = 0.036) detection by MSS, higher tissue qPCR levels of the predominant human anellovirus species torque teno virus (TTV; P = 0.016), T cell type PTLD, liver, brain or bone marrow location. In multivariable analyses, T cell PTLD (P = 0.006) and TTV PCR level (P = 0.012) remained significant. In EBV-positive PTLD, EBNA-LP, EBNA1 and EBNA3C had significantly higher levels of nonsynonymous gene variants compared to the other EBV genes. Multiple viruses are detectable in PTLD tissues by MSS. Anellovirus positivity, not EBV positivity,was associated with worse patient survival in our series. Confirmation and extension of this work in larger multicenter studies is desirable.

A review of adenotonsillar hypertrophy and adenotonsillectomy in children after solid organ transplantation.

OBJECTIVE: Paediatric solid organ transplantation is an increasingly successful treatment. Improved survival is paralleled by increased secondary complications of immunosuppression, including post-transplant lymphoproliferative disease (PTLD). PTLD frequently presents in Waldeyer's lymphatic ring. Adenotonsillar hypertrophy (ATH) is common in children, however in children after transplant, ATH may indicate PTLD. We review the literature on ATH and the role of adenotonsillectomy in children after transplantation. METHODS: A comprehensive literature search was performed on the 26 th September 2017 of Ovid Medline (1996-September 2017), Embase (1996-2017) and EBM reviews (Cochrane database of systematic reviews 2005-September 20 th 2017). Results were limited to English language publications within the last 20 years. Abstracts were screened for relevance to PTLD and ATH in the paediatric solid organ transplantation population. Screening of the bibliographies identified further articles. RESULTS: 85 unique articles were screened to yield 18 relevant publications. 10 were retrospective studies and 8 were prospective studies. CONCLUSION: In children, we report a PTLD incidence of up to 15%, with up to 63% of cases presenting in the head and neck. Histological examination of adenotonsillectomy specimens found PTLD in a mean 5.7% (range 0-39%). We found a lack of prospective studies into this topic and further high quality research is needed. Clinical assessment of ATH in children after transplantation and when to perform a diagnostic adenotonsillectomy remains challenging. Children with ATH warrant prompt further investigation and support from colleagues in transplantation and oncology is required. .