Posttransplant Lymphoproliferative Disorder Presenting Research Articles

#99 Clinical characteristics and outcomes of PTLD after kidney transplant: a single center experience

Abstract Background and Aims Post-Transplant Lymphoproliferative Disorder (PTLD) is a serious and potentially fatal complication of immunosuppression in kidney transplantation. Given the rarity of this entity, a high index of suspicion is necessary. We aimed to review the incidence, clinical presentation, histological subtypes, treatment, patient and graft survival of PTLD in kidney transplant patients in our unit. Methods Observational retrospective study, including adults diagnosed with PTLD between 1998 and 2023 in a Kidney Transplantation Center. Results Of 1390 kidney transplant recipients, 4 (0.3%) developed PTLD with a mean age of 52 ± 12.5 years. All cases were late-onset, occurring one year after transplantation with a median time from transplantation to diagnosis of 16.7 ± 7.3 years (range: 5-22 years). Of these, none had EBV donor/recipient mismatch. Two patients had prior rejection episodes (one patient, acute T cell-mediated rejection and the other patient, acute T cell-mediated rejection and active antibody mediated rejection). Clinical presentation was variable. Non-specific constitutional symptoms such as fever, fatigue and night sweats were present in all patients. Organ-specific symptoms were also present: lumbar pain in one patient with bone involvement, and delirium and lethargy in two patients with central nervous system involvement. Laboratory analyses revealed an increase in serum LDH in all patients. The primary sites of involvement were central nervous system (50%), lymph nodes (25%) and bone (25%). At PTLD diagnosis, immunosuppression included: tacrolimus (100%), mycophenolate mofetil (75%), low-dose of steroids (50%) and everolimus (25%). Regarding histologic classification, 75% were monomorphic and 25% polymorphic. In the monomorphic group, all patients had Diffuse Large B Cell Lymphoma (CD20 positive). In the polymorphic group, the patient had a Mixed Cellularity Classic Hodgkin Lymphoma (CD20 negative). Immunosuppression therapy was reduced in all patients with discontinuation of mycophenolate mofetil and tacrolimus. In the patient who was taking everolimus, the dose was increased due to the potential clinical antitumor effects of mTOR inhibition. Additional therapies included, rituximab in monotherapy or in combination with chemotherapy (R-CHOP) in the monomorphic group. Palliative measures were selected for one patient of this group due to advanced disease. The patient with Mixed Cellularity Classic Hodgkin Lymphoma (CD20 negative) did chemotherapy with ABVD. None of the patients underwent surgery or radiation therapy. Two patients needed dialysis. Three patients died in the first four weeks after PTLD diagnosis, all due to infectious complications. Patient survival was 25% one year after diagnosis. Conclusions Although the incidence of PTLD was low following kidney transplantation, its impact was significant in kidney graft and overall patient survival. Given the poor prognosis, new therapies combining high efficacy and low toxicity are an urgent unmet medical need in this field.

S3477 Uncommon Presentation of Post-Transplant Lymphoproliferative Disorder in a Liver Transplant Recipient

S3477 Uncommon Presentation of Post-Transplant Lymphoproliferative Disorder in a Liver Transplant Recipient

S3805 EBV-Negative Post-Transplant Lymphoproliferative Disorder Presenting as Multiple LI- RADS M Lesions on MRI

S3805 EBV-Negative Post-Transplant Lymphoproliferative Disorder Presenting as Multiple LI- RADS M Lesions on MRI

S2763 Post-Transplant Lymphoproliferative Disorder Presenting as an Isolated Pre-Pouch Ileal Ulcer in a Liver Transplant Patient With Crohn’s Disease

Introduction: Post-transplant lymphoproliferative disorder (PTLD) can occur in solid organ and hematopoietic cell transplant recipients in the setting of chronic immunosuppression. It often results from Epstein-Barr virus (EBV) affected B-cell propagation. We present a case of PTLD in a liver transplant (LT) patient with Crohn’s disease (CD) on biologic therapy. Case Description/Methods: A 67-year-old male with primary sclerosing cholangitis (PSC) cirrhosis status post living donor LT in 2001 on tacrolimus, multiple skin cancers, recurrent PSC after transplant and pan-ulcerative colitis on sulfasalazine underwent total proctocolectomy with ileo-anal anastomosis in 2013 to 2014 for colonic dysplasia. In 2019, he was diagnosed with moderate-to-severe CD of the J-pouch and pre-pouch ileum, up to 50cm from the entry site. Vedolizumab (VDZ), an α4β7 integrin monoclonal antibody, was initiated with steroid-free clinical response on every four week dosing. Repeat pouchoscopy on VDZ showed pre-pouch ileitis up to 30cm and a large, isolated ulcer in the pre-pouch ileum, 10cm proximal to the ileitis (Figure). Biopsies revealed B-cell lymphoma, consistent with PTLD. Negative EBV-encoded RNA (EBER) in-situ hybridization suggested that this diagnosis was unrelated to EBV exposure. Positron emission tomography (PET) scan did not identify distant disease activity, and bone marrow biopsy was unremarkable. VDZ was stopped due to loss of response, budesonide resumed and R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) was subsequently initiated. Follow-up pouchoscopy showed lymphoma regression but persistent CD activity. Discussion: PTLD typically presents as extra-nodal masses involving the central nervous system, lungs, gastrointestinal tract, liver, skin or allograft within one year of transplant. Immunosuppression reduction is the cornerstone of management; however, more aggressive cases, as in our patient, require systemic therapy. This case highlights a unique presentation of PTLD as an isolated ileal ulcer in a CD patient 19 years post-LT. Interestingly, the ulcer did not appear endoscopically distinct from CD-related ulceration. Furthermore, while malignancy is a potential risk of certain treatments for inflammatory bowel disease (IBD), our patient had never received thiopurines or anti-tumor necrosis factor (TNF) agents, and VDZ has not been associated with an increased risk of B-cell lymphoma. He is currently stable on low dose tacrolimus and budesonide.Figure 1.: Isolated pre-pouch ileal ulcer.

S2519 Post-Transplant Lymphoproliferative Disorder Presenting as Iron Deficiency Anemia: A Case Report

S2519 Post-Transplant Lymphoproliferative Disorder Presenting as Iron Deficiency Anemia: A Case Report

Fatal Case of EBV-negative Posttransplant Lymphoproliferative Disorder With Hemophagocytic Lymphohistiocytosis in an Adult Kidney Transplant Recipient.

Fatal Case of EBV-negative Posttransplant Lymphoproliferative Disorder With Hemophagocytic Lymphohistiocytosis in an Adult Kidney Transplant Recipient.

Early Complication of Monomorphic B-cell Lymphoma Post Pediatric Cardiac Transplantation

<h3>Introduction</h3> Post-transplant lymphoproliferative disorder (PTLD) occurs in 5% to 10% of all pediatric transplant recipients and is an important cause of post-transplant morbidity and mortality. Subtypes include reactive plasmocytic, monomorphic (e.g. B-cell lymphoma), and polymorphic forms. The most common type is monomorphic and is most often associated with Epstein Barr Virus. <h3>Case Report</h3> We describe a case of severe monomorphic B-cell lymphoma PTLD presenting 5 months post-transplant in a 15-year-old male with dilated cardiomyopathy. Both donor and recipient were seropositive for EBV. Peak EBV log post-transplant was 4.08. He received standard induction therapy with anti-thymocyte globulin and routine post-transplant immunosuppression with standard dosing of tacrolimus, mycophenolic acid, and steroid. His post-operative course was complicated by sternal osteomyelitis treated with 3 months of Ertapenem. Early biopsies revealed ACR 1R, but no significant rejection episodes or hemodynamic compromise. He presented with fever, abdominal pain, sore throat, and bilateral ear pain 5 months post-transplant. PET/MRI scan confirmed PTLD within tonsils, adenoids, and a focal hepatic lesion. A monomorphic high grade B-cell lymphoma was identified. Given these findings, his tonsils and adenoids were removed and immunosuppression regimen reduced. He received 4 weekly doses of Rituximab. Repeat PET scan demonstrated increased interval size of the hepatic lesion and new pulmonary nodules. Due to disease persistence, he was treated with four cycles of DA-EPOCH chemotherapy (etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone). PET scan 7 months post initial presentation demonstrated disease recurrence of the adenoid/tonsillar tissue. Biopsy of the nasal sinus by endoscopy revealed a rare aberrant B-cell population including CD19, CD20, CD22, and CD23. He required 2 additional cycles of chemotherapy and continues to undergo treatment of his PTLD. <h3>Summary</h3> PTLD is a known complication post-cardiac transplantation. Risk factors include EBV-naivety, degree of immunosuppression, and host genetic variations. Treatment is typically reduction in immunosuppression. We describe an extreme case of PTLD not responsive to immunosuppression reduction or rituximab alone and highlight the need for alternative treatment strategies for more advanced disease.

A Case Report of Posttransplant Lymphoproliferative Disorder After AstraZeneca Coronavirus Disease 2019 Vaccine in a Heart Transplant Recipient

We report a case of a heart transplant recipient who presented with a rapidly growing Epstein-Barr virus (EBV)–positive, diffuse large B-cell lymphoma 7 days after receiving the first dose of the ChAdOx1 nCoV-19 vaccine. Because of the atypical radiologic presentation, the initial tentative diagnosis was a mediastinal abscess. This observation indicates a potential risk of EBV reactivation after coronavirus disease 2019 (COVID-19) vaccination, which might lead to or aggravate the presentation of posttransplant lymphoproliferative disorder in transplantation patients. Transplant surgeons should be aware of the potential immunomodulatory effects of the COVID-19 vaccination.

Unusual presentation of post-transplant lymphoproliferative disorder in renal transplant

Unusual presentation of post-transplant lymphoproliferative disorder in renal transplant

S1590 Post-Transplant Lymphoproliferative Disorder of a Pancreas Allograft Presenting as Pancreatic Necrosis

INTRODUCTION: Post-transplant lymphoproliferative disorder (PTLD) is a serious and often fatal complication of solid-organ transplants, with incidence up to 13%. PTLD can be seen in pancreas transplant recipients, but allograft involvement is rare. Epstein Barr virus (EBV) infection and high immunosuppression regimen increases the risk of PTLD. This is a case of PTLD of a pancreas allograft presenting as pancreatic necrosis. CASE DESCRIPTION/METHODS: A 41-year-old female with end-stage renal disease secondary to diabetes who received a simultaneous pancreas-kidney transplant 6 months ago presented with abdominal pain and pancytopenia concerning for PTLD. Routine testing revealed recent development of EBV viremia (greater than 30,000 copies/mL). Immunosuppressants included tacrolimus, mycophenolate, and low dose prednisone. White blood cell count and lipase values were normal. Imaging of the transplanted pancreas revealed inflammation and a 1.0 × 1.5cm peripancreatic fluid collection in the right lower quadrant, suggestive of necrosis. Based on cross-sectional imaging, Interventional Radiology deemed that the inflammation precluded adequate sampling of the allograft. Endoscopic ultrasound (EUS) with biopsy of the transplanted pancreas was requested. The transplanted pancreas was endosonographically visualized from the cecum, showing a 0.6 × 0.5cm hypoechoic heterogenous pancreatic head lesion consistent with necrosis (Figures A and B). Trans-colonic FNA was deferred due to risk of infecting the sterile necrotic cavity. Pancreatectomy was performed 1 week later due to fevers, persistent abdominal pain, and enlarging peripancreatic fluid collection. Pathology revealed extensive necrosis infiltrated by lymphocytes and plasma cells consistent with PTLD (Figure C). DISCUSSION: Our patient had a unique presentation of PTLD with necrosis of the transplanted pancreas. Early diagnosis and treatment of rejection are critical in preventing failure of the transplanted organ. Minimally invasive diagnostic methods like EUS are gaining popularity. EUS of pancreas allografts is usually performed via a trans-duodenal approach. Due to our patient's duodenojejunostomy anastomosis, EUS was performed via the colon, which is unique. Expert review of cross-sectional imaging and knowing the institutional pancreas transplant technique will help determine an effective EUS approach. Early tissue diagnosis using EUS guided sampling must be strongly considered in pancreas allografts to detect and treat post-transplant complications.Figure 1.: Endosonographic Imaging of Transplanted Pancreas.Figure 2.: Endosonographic Imaging of Transplanted Pancreas with Hypoechoic Pancreatic Head Lesion.Figure 3.: Transplanted Pancreas with Lymphocytic and Plasma Cell Infiltration Consistent with PTLD and Extensive Necrosis.

S2572 Post-Liver Transplantation Lymphoproliferative Disorder Presenting as Biliary Stricture

INTRODUCTION: A 63 year-old man with non-alcoholic steatohepatitis (NASH) cirrhosis who underwent orthotopic liver transplant (OLT) presented 1 year later with obstructive jaundice due to a biliary stricture. Further investigation revealed encasement of the bile duct by a tumor, later determined to be post-transplant lymphoproliferative disorder (PTLD). PTLD represents a rare cause of post-OLT biliary stricture. CASE DESCRIPTION/METHODS: Post-transplant course was complicated by Grade 2 acute rejection, necessitating high-intensity immunosuppression. He later developed laboratory derangements, intermittent fevers, weight loss, and sweating episodes which were ascribed to active cytomegalovirus (CMV) and Epstein-Barr Virus (EBV) viremia. One year post-transplantation, an acute elevation in total bilirubin prompted an MRCP which revealed a soft tissue mass arising from segment VIII of the liver, extending into the porta hepatis and encasing the common bile duct at the surgical anastomosis. Biopsy showed diffuse large B cell lymphoma, later classified as stage IV disease with diffuse lymph node and bony metastasis. His tacrolimus was discontinued and systemic chemotherapy with R-CHOP was initiated. DISCUSSION: PTLD includes a heterogenous group of lymphoproliferative disorders that occur after solid organ or allogenic hematopoietic stem-cell transplantation, generally in the setting of EBV. PTLD may occur in up to 5.5% of adults following liver transplant. The clinical presentation of PTLD is heterogenous, and may range from incidental findings to fulminant organ failure. Treatment for PTLD consists of reduction of immunotherapy and systemic chemotherapy regimens, such as R-CHOP. Biliary complications after OLT are reported in up to 40% of cases, and usually involve biliary strictures. Biliary strictures are classified as anastomotic or non-anastomotic and are usually secondary to vascular or surgical complications. Post-OLT biliary stricture resulting from PTLD is quite rare. This case demonstrates the difficulties of prevention and diagnosis of PTLD-related biliary stricture. The presence of acute rejection necessitated high-intensity immunosuppression, which predisposed the patient to both CMV and EBV. These active viral infections were used to explain persistent “B symptoms” and abnormal laboratory findings that may have actually been the direct result of PTLD. This case illustrates the need to consider PLTD-related biliary stricture in any post-OLT transplant patient presenting with obstructive jaundice.Figure 1.: This demonstrates an isointense mass on T2-weighted imaging, which involves hepatic segment 8 and extends towards the porta hepatis with resultant stricture of the post-transplant biliary anastomosis and subsequent intrahepatic biliary dilatation.Figure 2.: On diffusion weighted imaging, the mass demonstrates marked diffusion restriction, with corresponding low signal on ADC.Figure 3.: 18F-FDG PET demonstrates intense FDG uptake within the mass, with multifocal hypermetabolic metastatic disease involving several lymph node groups as well as multiple osseous structures.

Small Bowel Perforation as the Initial Manifestation of Post-Transplant Lymphoproliferative Disorder in a Kidney and Pancreas Transplant Recipient: A Case Report

Post-transplant lymphoproliferative disorder (PTLD) comprises a broad spectrum of diseases and is a rare but serious complication of solid organ transplantation. We report the case of a 45-year-old simultaneous pancreas and kidney (SPK) transplant recipient with diffuse, early-onset PTLD, manifesting as jejunal perforation at 6 months after transplantation. The patient underwent urgent small bowel resection of the affected portion of jejunum. The surgical pathology report was significant for diffuse large B-cell lymphoma. Subsequently, the patient underwent a full workup, including upper and lower endoscopy and whole-body positron emission tomography that revealed involvement of the axial skeleton and multiple abdominal organs with sparing of the grafts. He was treated with rituximab and intrathecal methotrexate for central nervous system prophylaxis. The patient experienced complete resolution of disease by positron emission tomography 8 months after initial presentation. We found no previous report in the literature of intestinal perforation as the initial presentation of PTLD in SPK transplant recipients.

1598 Late-Onset Post-Transplant Lymphoproliferative Disorder Presenting as Diffuse Large B-Cell Lymphoma in the Ascending and Transverse Colon

INTRODUCTION: A serious complication of solid organ transplantation is post-transplant lymphoproliferative disorder (PTLD). The vast majority of PTLD was previously found to present within the first year after transplant, when immunosuppression is highest. However, newer studies suggest a bimodal peak in incidence. 1 This case describes PTLD occurring in the colon as a late complication of multi-organ transplant. CASE DESCRIPTION/METHODS: A 54-year-old male with a complicated transplant history, on tacrolimus for immunosuppression, presented with worsening abdominal pain, nausea, diarrhea, and weight loss. His transplant history includes cardiac transplants in 1980 and 1993 for idiopathic cardiomyopathy, and renal transplant in 2003 due to tacrolimus toxicity. He denied fever, night sweats, or recent sick contacts, and was hemodynamically stable. On exam, he had diffuse abdominal pain with mild guarding but no rebound. Laboratory findings revealed a tacrolimus level of 11.1. Abdominal computed tomography scan was significant for fluid-filled loops of small bowel with focal thickening of the transverse colon, concerning for colonic malignancy (Figure 1). Gastroenterology was consulted for urgent colonoscopy, which showed mucosal ulceration and inflammation throughout the colon, with sparing of the sigmoid and rectum (Figure 2). Biopsy of the cecum, ascending colon, and transverse colon revealed diffuse infiltrate of pleomorphic large cells in the lamina propria and submucosa with focal ulcerations and necrosis, consistent with PTLD of monoclonal diffuse large-B cell morphology. The biopsy was negative for EBV and CMV. Immunohistochemistry was positive CD45RB, CD79a, CD30 and BCl2. He was referred to hematology-oncology and followed-up as an outpatient to begin chemotherapy. DISCUSSION: Though PTLD is classically described as an early complication of solid organ transplant, this case presents the development of colonic PTLD after over 15 years since last transplant. This finding supports the notion that there is a bimodal peak in incidence. This case also demonstrates the insidious, non-specific gastrointestinal findings of colonic PTLD. Because of this, and as survival after organ transplant continues to increase, providers must have a high index of suspicion for PTLD in the colon as a late complication of transplant.

2422 Post-Transplant Lymphoproliferative Disorder

INTRODUCTION: Post-transplant lymphoproliferative disorder (PTLD) is a rare but severe complication in both solid organ and hematopoietic stem cell transplantation. PTLD is caused by the dysfunction of T-cells as a result of the immunosuppressive therapy commonly used in transplant patients. This dysfunction then causes abnormal lymphoid proliferation leading to malignancy. We discuss a case of PTLD presenting in the form of a small bowel obstruction in a patient with dual liver, kidney transplantation. CASE DESCRIPTION/METHODS: A 68-year-old man with previous NASH cirrhosis status post a deceased donor liver and kidney transplant presented to the hospital with nausea, vomiting, and abdominal distension. Prior to presentation he had been maintained at home on Cellcept and Tacrolimus for many years. He was doing well until 3 months prior to presentation when he noted the onset of night sweats. He had no other symptoms until two days prior to presentation when he had the abrupt onset of abdominal pain, distention, nausea and vomiting causing his presentation to the ED. On arrival his initial work up was significant for a mild leukocytosis, anemia, and AKI. He had a CT abdomen and pelvis done which was significant for a small bowel obstruction secondary to a small bowel mass along with mesenteric and retroperitoneal lymphadenopathy, raising the concern of PTLD on initial presentation. Interventional radiology was then consulted and performed a para-aortic lymph node biopsy. EBV and CMV PCR returned negative but the surgical pathology returned as Diffuse Large B Cell Lymphoma with double expression, suggesting a diagnosis of Post Transplant Lymphoproliferative Disorder. He was started on the R-CHOP chemotherapy regimen inpatient and was discharged for further outpatient treatment. DISCUSSION: This case is a rather classic presentation of a rare complication of immunosuppressive therapy, demonstrating the possible lethal side effects of these often life saving drugs. Swift diagnosis was made possible due to early clinical suspicion and rapid involvement of a multitude of different medical specialties. This case demonstrated the need for multi-disciplinary team at all transplant centers and how close communication between consultants is key to these patients success.

Post-transplant lymphoproliferative disorder presenting with multifocal aggressive osseous lesions.

Post-transplantation lymphoproliferative disorder (PTLD) encompasses a broad category of lymphoid and plasmacytic proliferations that occur following solid organ and/or allogeneic stem cell transplantation. PTLD manifests in the setting of chronic immunosuppression and is thought to be associated with the Epstein Barr Virus, although Epstein Barr Virus infection or reactivation is not required for the process to occur. Pathologic correlation is necessary for diagnosis with B-cell lymphocytes the most commonly isolated cellular etiology. There is a broad range of clinical and imaging presentations of PTLD with intestinal and nodal involvement being the most common. Imaging plays an integral part in the diagnosis and management of PTLD, as it is utilized in the initial diagnosis and staging, guiding biopsy of lesions, and gauging treatment response. Presenting symptoms of PTLD are often vague and nonspecific and depend on the organ systems affected. Musculoskeletal involvement is especially rare, with only a few cases described in the literature. We present a case with multifocal osseous manifestations of PTLD occurring years after a renal living donor transplant.

Posttransplant Lymphoproliferative Disorder Presenting as Testicular Lymphoma in a Kidney Transplant Recipient: A Case Report and Review of the Literature

Posttransplant lymphoproliferative disorder (PTLD) is a malignancy caused by the immunosuppression that occurs after transplantation. It is primarily a nodal lesion but frequently it involves extranodal masses. Treatment is usually by reducing immunosuppressive therapy. Testicular lymphoma as PTLD is notably rare in documented literature and there is limited evidence of definitive treatment guidelines. This manuscript describes a patient who developed diffuse large B-cell lymphoma of his right testis one year following kidney transplantation. A diagnosis of PTLD was made and treatment with rituximab, locoregional radiotherapy, and intrathecal methotrexate in addition to the standard reduction of immunosuppression resulted in complete remission until now. We submit this case along with literature review of similar cases in the past and a review of specific peculiarities of our case with emphasis on our treatment plan to further the understanding of this diversiform disease.

Posttransplant Lymphoproliferative Disorder Presenting as Cardiogenic Shock With Tamponade

SESSION TITLE: Transplantation SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Post-transplant lymphoproliferative disorders (PTLD) are malignancies associated with solid organ transplantation or allogeneic hematopoietic cell transplantation requiring immunosuppression. Most cases are associated with EBV positivity, but less commonly EBV negative PTLD occurs. CASE PRESENTATION: A 55-year-old Caucasian male presented with cardiogenic shock due to pericardial effusion with tamponade. His history was significant for type I diabetes mellitus leading to ESRD, two failed renal transplants on immunosuppression, and current peritoneal dialysis dependence. Echocardiogram upon admission demonstrated pericardial effusion with right ventricular collapse. Pericardiocentesis and pericardial drain placement were performed. Approximately 2.4L of fluid was drained. Cytology of the cell block was negative for malignancy. He improved after drainage and was discharged on day 16. Two days after discharge he returned complaining of weakness. A left sided pleural effusion was detected and drained via catheter. Cell block cytology was remarkable for monomorphic PTLD, plasma cell neoplasm. Five days after pleural catheter placement he developed multifactorial shock that led to a whole-body CT with contrast. This revealed dilation of the jejunum measuring 5.1cm, as well as a mass that measured 8.4 x 5.6. x 5.4 cm. The mass demonstrated atypical plasma cells consistent with the cell block. Discontinuing immunosuppression was the initial step in treatment in this case. Further work up for plasmacytoma or multiple myeloma demonstrated negative SPEP and negative lambda ratio confirming a solitary plasmacytoma. A skeletal survey was completed with no signs of lytic lesions or evidence of multiple myeloma. A bone marrow biopsy was unable to be completed as the patient expired. DISCUSSION: The lesion consisted of atypical plasma cells similar to cases of EBV-negative PTLD previously mentioned in the literature. This is currently a designation not clearly defined by the World Health Organization. These findings call for further investigation to discover the mechanism of disease and promote effective therapy. Therapy in EBV-negative PTLD does not seem to respond to the established algorithm for EBV-positive PTLD. Reducing immunosuppression often makes no difference. Some cases require high dose immunosupression to achieve therapeutic success, which is the opposite of the standard treatment for PTLD. Defining the mechanism behind these tumors is imperative for better classification as well as directed therapy. CONCLUSIONS: This case is remarkable for PTLD with CD20 negativity; additionally, PCR for EBV was negative. EBV is typically the primary driver of PTLD. The mechanism of EBV negative PTLD remains unknown. Reference #1: Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood. 2011;117(19):5019-5032. doi:10.1182/blood-2011-01-293050. DISCLOSURE: The following authors have nothing to disclose: Matthew Boulos, Anil Singh, David Aljadir, Tina Dudney No Product/Research Disclosure Information

Various Clinical Presentation of Post-Transplant Lymphoproliferative Disorder in Head and Neck: A Case Series of 3 Patients

The outcome of solid organ and bone marrow transplantation has been dramatically improved with the development of immunosuppressive agent. However, the use of immunosuppressive agents could increase the risk of malignancies such as post-transplant lymphoproliferative disorder (PTLD). PTLD is regarded as the lymphoid malignancy of patients using immunosuppressive agents, and it could present diverse and non-specific symptoms. It involves various organs including the tonsil, adenoid, lymph node, and the brain. Because of its poor prognosis, an early suspicion of pathologic diagnosis is crucial for the treatment of PTLD. In this report, we demonstrate the case of three pediatric patients who had been treated for PTLD of various clinical presentations by early suspicion and pathologic diagnosis. Key words: Head and neck ㆍ Immunosuppressive agent ㆍ Post-transplant lymphoproliferative disorder

Posttransplant Lymphoproliferative Disorder: Otolaryngological Manifestations and Management.

Objective Posttransplant lymphoproliferative disorder (PTLD) is a unifying term for a spectrum of lymphoid expansion entities brought about by immunosuppression and is strongly associated with Epstein-Barr virus (EBV). Otolaryngological findings tend to present early in the clinical course; therefore, disease awareness among otolaryngologists is of utmost importance. This review synthesizes the body of literature pertaining to PTLD involving the head and neck, summarizes contemporary management, and highlights areas for future research. Data Sources PubMed/Medline. Review Methods Primary literature search of the Medline database was performed for all titles published in the past 10 years pertaining to PTLD. The database search included PTLD combined with a collection of otolaryngological MeSH terms. Full manuscripts were reviewed based on relevance of their title and abstract. Selection into this review was according to clinical and scientific relevance. Conclusion Adenotonsillar focus is common in children in whom adenotonsillectomy may be diagnostic and prevents potentially morbid airway obstruction. Sinonasal PTLD may mimic fungal infection. Laryngotracheal involvement predominately presents in children with symptoms of airway obstruction. PTLD limited to the esophagus is rare. Oral PTLD is rare and phenotypically varied. Cutaneous presentation of PTLD is infrequent, yet one-third of cases affects the head and neck. PTLD may present as cervical lymphadenopathy. Implications for Practice PTLD consideration is vital when evaluating posttransplant patients. Children and EBV-seronegative patients should receive otolaryngological follow-up after transplant. PTLD treatment is multidisciplinary and typically led by lymphoma specialists. Formal partnerships between otolaryngologists and transplant centers may improve patient care and research quality.

Skin Rash: A Rare Presentation of Post-Transplant Lymphoproliferative Disorder

Skin Rash: A Rare Presentation of Post-Transplant Lymphoproliferative Disorder