Trasplante Renal en un paciente con Síndrome de Down, Seudo Prune Belly y un trastorno linfoproliferativo post-trasplante: Primer reporte de un caso en Argentina / Kidney trasplantation in a female infant with Down syndrome, Pseudo Prune Belly Syndrome, and a post-transplant lymphoproliferative disorder. First case report in Argentina

RESUMEN Introducción: Los pacientes con trisomía 21 tienen un mayor riesgo de anomalías congénitas, incluidas las anomalías renales y de vías urinarias. La ocurrencia del Síndrome de Prune Belly y el Síndrome de Down ha sido descrita, pero representaría una coincidencia. Existen escasos reportes que describan estos pacientes y que fueran trasplantados. Caso Clínico: Paciente de 5 años con diagnóstico prenatal de trisomía 21. La ecografía postnatal reveló megavejiga y riñones displásicos. Comenzó con diálisis peritoneal a los 5 meses de vida. Se realizó el trasplante renal a los 3 años de edad, complicando con trombosis aguda de la arteria y pérdida del injerto. Cuatro meses después del segundo trasplante, la paciente fue diagnosticada con un trastorno linfoproliferativo post-trasplante.Conclusión: Se describe la evolución y el manejo de un único paciente que presentó múltiples complicaciones, con la esperanza de contribuir al conocimiento existente en relación con los pacientes trasplantados renales con síndrome de Down.

ABSTRACT Introduction: Patients with trisomy 21 have a higher risk for congenital anomalies including congenital anomalies of the kidney and urinary tract. The association between Prune Belly Syndrome and Down Syndrome has been described but the occurrence of both conditions would likely represent a coincidence. There are few published reports on renal transplantation in patients with this syndromes. Clinical Case: We reporte a 5-year-old female patient with antenal diagnosis of down syndrome. Post-natal abdominal ultrasound revealed megabladder and dysplastic kidneys. At five months of age, she was commenced on peritoneal dialysis. The patient underwent renal transplantation at age of 3. Acute thrombosis of transplanted renal artery was diagnosed, resulting in graft loss. Four months after second transplantation, the patient presented bilateral tonsillar enlargement and a post-transplant lymphoproliferative disorder was diagnosed. Conclusion: We describe the existence of these conditions in a single patient who underwent kidney transplantation, it's clinical manement and follow up. This case has been reported with the hope of contributing to the existing knowledge which pertains to kidney transplantation patients with Down syndrome.

Central nervous system post-transplant lymphoproliferative disorder: a case report / 中华神经科杂志

Post-transplant lymphoproliferative disorder (PTLD) is a solid organ or hematopoietic stem cells transplant associated syndrome, and central nervous system PTLD(CNS-PTLD) is extremely rare. A case of CNS-PTLD occurring after 24 years of kidney transplant was reported, and pathological examination proved it to be diffuse large B cell lymphoma. Cerebrospinal fluid next generation sequencing and pathological examination supported that Epstein-Barr virus infection was associated with it.

Significance of Epstein-Barr virus-infected lymphocyte cell types in peripheral blood mononuclear cells in post-transplant lymphoproliferative disorder after pediatric liver transplantation / 中华器官移植杂志

Objective:To explore the characteristics and significance of Epstein-Barr virus-infected lymphocyte cell types in peripheral blood mononuclear cells(PBMC)in post-transplant lymphoproliferative disorder(PTLD)after pediatric liver transplantation.Methods:From June 2013 to March 2021, retrospective data analysis was performed for 14 pediatric liver transplant recipients with PTLD.The determination of EBV-DNA in PBMC, plasma and TBNK lymphocyte cells was analyzed.Results:EBV-DNA in PBMC showed a high viral load(>10 4 copies/ml)and plasma EBV-DNA was >10 3 copies/ml( n=8). There were dominant B-cell-type infection( n=12)and T/NK-cell-type infection( n=2). After treatment, EBV-DNA in PBMC and plasma turned negative in 7 patients with a decline( n=6)and an increase( n=1). EBV-DNA in B lymphocyte became negative( n=10)with a decline( n=3). In one case, EBV-DNA increased in T, B and NK cells with a high viral load.The remainders recovered after treatment.One case of hemophagocytic syndrome died from a progression of PTLD. Conclusions:A large majority of EBV-related PTLD are dominated by B-cell-type infection and a few belong to T or NK-cell-type infection.Patients with T/NK-cell-type infection have a worse response to therapy and poorer prognosis than those with B-cell-type infection.Determination of EBV-infected lymphocyte cell types is of vital research value for treatment and prognosis.

Expression of programmed death-1 and programmed death-ligand 1 in post-transplant lymphoproliferative disorders after allogeneic hematopoietic stem cell transplantation in children / 中华实用儿科临床杂志

Objective:To explore the expressions of programmed death-1 (PD-1) and programmed death-ligand 1 (PD-L1) and clinicopathological characteristics in post-transplant lymphoproliferative disorder (PTLD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children, with the aim of clarifying whether checkpoint inhibition of PD-1/PD-L1 inhibitors may serve as a therapy option.Methods:The clinical data of 13 cases of PTLD after allo-HSCT pathologically confirmed in Shenzhen Children′s Hospital from January 1, 2012 to December 30, 2019 were retrospectively analyzed.The detection was performed by immunohistochemical staining by MaxVision? method, Epstein-Barr virus(EBV) in situ hybridization and lymphoma gene rearrangement.The relationship between the expression of PD-1 and PD-L1 and the clinicopathological characteristics of PTLD were analyzed.Results:The expression of PD-1 was not correlated with gender, age, primary diseases, histopathological types, transplantation mode and the expression of EBV in situ hybridization (all P>0.05). The expression of PD-L1 was correlated with histopathological types ( P<0.05). Furthermore, the expression rate of PD-L1 on severe β-thalassemia was significantly higher than that of severe aplastic anemia [90.0%(9/10 cases) vs. 66.7%(2/3 cases)] and monomorphic PTLD was higher than that of polymorphic PTLD [100.0%(2/2 cases) vs. 83.3%(5/6 cases)]. Moreover, the positive PTLD in EBV was higher than the negative PTLD in EBV [90.9%(10/11 cases) vs. 50.0%(1/2 cases)]. The positive rates of PD-1 and PD-L1 in 13 cases with PTLD were 46.2%(6/13 cases) and 61.5%(8/13 cases) in tumor cells, 92.3% (12/13 cases) and 76.9% (10/13 cases) in microenvironmental cells, and 84.6%(11/13 cases) in EBV, respectively. Conclusions:PD-L1 has a higher positive rate in tumor cells with monomorphic PTLD; and routine staining for PD-1 and PD-L1 can be performed in all types of PTLD when standard immunotherapy and chemotherapy are ineffective.

Two cases of rare HHV8-driven intravascular lymphoma with synchronous Kaposi sarcoma, both diagnosed at autopsy in renal transplant recipients

We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 infection causes Kaposi sarcoma (KS). More recently, it is recognized that HHV8 is also related to several lymphoproliferative conditions. These are poorly characterized and often difficult to diagnose. In both cases described herein, the diagnoses of multifocal hepatic KS and intravascular HHV8 positive (EBV negative) systemic diffuse large B-cell lymphoma, NOS were made at autopsy. Given the findings we describe in cases with fatal outcomes, we discuss the implications of HHV8 screening in solid allograft recipients.

Linfoma anaplásico de células T grandes primario cutáneo CD30+. Serie de nueve casos / Primary cutaneous CD30+ anaplastic large T-cell lymphoma. Series of nine cases

Resumen Introducción: El linfoma anaplásico de células T grandes CD30+ es un linfoma primario cutáneo en el cual no hay evidencia de enfermedad sistémica; para su diagnóstico es necesario el estudio histopatológico. Objetivo: Presentar los casos diagnosticados en el Departamento de Dermatología del Hospital General "Dr. Manuel Gea González" con linfomas anaplásicos de células T grandes primarios cutáneos CD30+ durante un periodo de 24 años. Método: Estudio retrospectivo en el que realizó estadística descriptiva. Se recopiló información de sexo, edad, características clínicas, resultados de pruebas complementarias, tratamientos previos y actuales, reportes de los estudios histopatológicos y de inmunohistoquímica. Resultados: Entre 29 309 expedientes, se encontraron nueve casos (0.000034 %) con diagnóstico de linfoma anaplásico de células T CD30+. Se hizo la confirmación del diagnóstico histopatológico e inmunohistoquímico por dos dermatopatólogos certificados. La edad promedio fue de 61.2 años, hubo predominio del sexo femenino y de lesión papular o nodular y topografía variada como presentación clínica inicial. Conclusiones: El pronóstico del linfoma anaplásico de células T grandes CD30+ en la población estudiada fue dependiente del estadio clínico. El tratamiento en etapas tempranas tiene resultados favorables.

Abstract Introduction: CD30+ anaplastic large T cell lymphoma is a cutaneous primary lymphoma in which there is no evidence of systemic disease; histopathological study is required for its diagnosis. Objective: To present the cases diagnosed with primary cutaneous CD30+ anaplastic large T-cell lymphoma over a 24-year period in Hospital General "Dr. Manuel Gea González" Department of Dermatology. Method: Retrospective study. Descriptive statistics was carried out. Information was collected on gender, age, clinical characteristics, complementary test results, previous and current treatments, histopathological studies reports and immunohistochemistry test results. Results: Of 29 309 records, nine patients (0.000034%) with a diagnosis of CD30+ anaplastic T cell lymphoma were found. Histopathological and immunohistochemical diagnosis was confirmed by two certified dermatopathologists. Average age was 61.2 years, and there was a predominance of the female gender, with initial clinical presentation as a papular or nodular lesion and varied topography. Conclusions: The prognosis of CD30+ anaplastic large T cell lymphoma in the studied population was dependent on clinical stage. The treatment at early stages has favorable results.

Linfoma leucemizado em felino coinfectado com os vírus da imunodeficiência felina e da leucemia felina: relato de caso / Lymphoma in leukemic phase in feline coinfected with feline immunodeficiency virus and feline leukemia virus: case report

O presente trabalho teve por objetivo relatar um caso de linfoma leucemizado em um felino coinfectado com os vírus da imunodeficiência felina (FIV) e o da leucemia felina (FeLV). Foram realizados exames de hemograma, contagem de reticulócitos, mielograma, bioquímica, teste de imunocromatografia para FIV e FeLV, imunofluorescência indireta (IFA) para FeLV, radiografia torácica e citologia renal. Esse último exame revelou um linfoma extranodal. Foi determinante para a conclusão diagnóstica a associação dos sinais clínicos corroborados com a infiltração de elevada quantidade de células linfoblásticas na medula óssea, exibindo critérios citomorfológicos de malignidade, como mitoses atípicas, relacionadas à presença de corpúsculos linfoglandulares e material hematopoiético inter-relacionado. O linfoma é uma neoplasia relativamente comum em felinos, entretanto, a apresentação leucemizada é rara, podendo representar um desafio diagnóstico clínico, o que torna fundamental a inclusão da citologia medular na prática clínica dessa espécie.(AU)

The present study aimed to report a case of lymphoma in leukemic phase in feline coinfected with feline immunodeficiency virus (FIV) and feline leukemia virus (FeLV). Blood counts, reticulocyte counts, bone marrow avaluation, biochemistry, immunochromatography assay for FIV and FeLV, indirect immunofluorescence (IFA) for FeLV, thoracic radiography and renal citology were performed. This last examination revealed extranodal lymphoma. The association of the clinical signs with the infiltration of a high number of lymphoblastic cells in the bone marrow with cytomorphological criteria of malignancy, atypical mitoses, lymphoglandular corpuscles and hematopoietic material were determinant for the diagnostic conclusion. Lymphoma is a relatively common neoplasm in felines, however the leukemic phase is rare and may represent a clinical diagnostic challenge, making it essential to include bone marrow cytology in the clinical practice of this species.(AU)

Nursing experience in the process of diagnosing and treating post-transplantation lymphoproliferative disorder / 中国实用护理杂志

Objective@#To explore the key points of nursing in the process of diagnosing and treating the common complication--- post-transplantation lymphoproliferative disorder (PTLD) after pediatric liver transplantation.@*Methods@#The clinical data of 21 children with PTLD after liver transplantation. The key point of nursing in the process of prevention, onset, diagnosis and treatment was summarized.@*Results@#With the individual and combined treatments 18 patients gained remission except for 1 patient died because of perforation and sequent intractable haemorrhage and 2 patient died for the development of lymphoma. In terms of nursing, during the prevention stage, it is critical to insist the regular follow-up to make early diagnosis. More efforts should be made to obtain the children′s cooperation to complete the examination. During the infusion of rituximab, strengthened monitoring is necessary and more attention should be paid to the special nursing when other complications such as intractable haemorrhage, intestinal obstruction or perforation occurs.@*Conclusions@#The nursing care of PTLD is complicated. Individual and careful nursing regimens should be made based on the specific situations in different stages.

Clinical, pathological and prognostic analysis of 15 children with hydroa vacciniforme-like lymphoproliferative disorder / 中华皮肤科杂志

Objective@#To analyze clinical features and prognosis of 15 children with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) .@*Methods@#Clinical features, histopathological findings, treatment outcomes in 15 patients, who were diagnosed with HVLPD in the Department of Dermatology, Children′s Hospital of Chongqing Medical University from 2014 to 2018, were retrospectively analyzed.@*Results@#Among the 15 patients, 7 were males and 8 were females. The age of onset ranged from 2 to 13 years, and the average age was 6.5 years. Main clinical manifestations included facial edema, and papules, blisters, ulcers and crusts repeatedly occurring on both the sun-exposed parts such as the face and limbs and non-sun-exposed parts such as the trunk. Of the 15 patients, 13 had intermittent fever, 13 had hepatosplenomegaly, and 15 had lymphadenectasis. Moreover, kidney failure occurred in 1 patient, hemophagocytic syndrome occurred in 2, and lymphoma occurred in 1. Laboratory examination showed that 15 patients were all positive for Epstein-Barr virus (EBV) -immunoglobulin G (IgG) antibody, and all negative for EBV-IgM antibody. Additionally, high replication of EBV DNA was detected. Histopathological study of skin lesions showed mild to dense perivascular and periadnexal infiltration of lymphocytes in the dermis and subcutaneous tissue. Immunohistochemistry study showed positive staining for CD4 and CD8 in 13 of 15 patients, for CD56 in 7 of 15 patients, and for CD3 in 12 of 13 patients. Moreover, 9 of 11 cases were detected positive for T-cell intracellular antigen-1, 8 cases were all detected positive for granzyme B, and the Ki-67 proliferation index ranged from 3% to 50% in 11 of 12 cases. The treatment protocols were similar among the 15 patients. After the treatment, 2 patients showed stable disease condition, and 10 experienced relapse. During the follow-up, 2 patients developed hemophagocytic syndrome, 1 developed lymphoma, and the 3 patients finally died of the worsening condition after chemotherapy.@*Conclusions@#HVLPD is closely associated with chronic active EBV infection. Its clinical symptoms can be relieved after immunomodulatory therapies such as glucocorticoids, gamma globulin and interferon, and the clinical manifestations, treatment outcomes and prognosis vary greatly among different patients.

Clinical, pathological and prognostic analysis of 15 children with hydroa vacciniforme - like lymphoproliferative disorder / 中华皮肤科杂志

Objective To analyze clinical features and prognosis of 15 children with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD). Methods Clinical features, histopathological findings, treatment outcomes in 15 patients, who were diagnosed with HVLPD in the Department of Dermatology, Children's Hospital of Chongqing Medical University from 2014 to 2018, were retrospectively analyzed. Results Among the 15 patients, 7 were males and 8 were females. The age of onset ranged from 2 to 13 years, and the average age was 6.5 years. Main clinical manifestations included facial edema, and papules, blisters, ulcers and crusts repeatedly occurring on both the sun-exposed parts such as the face and limbs and non-sun-exposed parts such as the trunk. Of the 15 patients, 13 had intermittent fever, 13 had hepatosplenomegaly, and 15 had lymphadenectasis. Moreover, kidney failure occurred in 1 patient, hemophagocytic syndrome occurred in 2, and lymphoma occurred in 1. Laboratory examination showed that 15 patients were all positive for Epstein-Barr virus (EBV)-immunoglobulin G (IgG) antibody, and all negative for EBV-IgM antibody. Additionally, high replication of EBV DNA was detected. Histopathological study of skin lesions showed mild to dense perivascular and periadnexal infiltration of lymphocytes in the dermis and subcutaneous tissue. Immunohistochemistry study showed positive staining for CD4 and CD8 in 13 of 15 patients, for CD56 in 7 of 15 patients, and for CD3 in 12 of 13 patients. Moreover, 9 of 11 cases were detected positive for T-cell intracellular antigen-1, 8 cases were all detected positive for granzyme B, and the Ki-67 proliferation index ranged from 3％to 50％in 11 of 12 cases. The treatment protocols were similar among the 15 patients. After the treatment, 2 patients showed stable disease condition, and 10 experienced relapse. During the follow-up, 2 patients developed hemophagocytic syndrome, 1 developed lymphoma, and the 3 patients finally died of the worsening condition after chemotherapy. Conclusions HVLPD is closely associated with chronic active EBV infection. Its clinical symptoms can be relieved after immunomodulatory therapies such as glucocorticoids, gamma globulin and interferon, and the clinical manifestations, treatment outcomes and prognosis vary greatly among different patients.

Diagnosis and treatment of posttransplant lymphoproliferative disorder after pediatric liver transplantation / 中华器官移植杂志

Objective To retrospectively explore the clinical symptoms ,diagnosis ,treatment and prognosis of posttransplant lymphoproliferative disorder (PTLD) after pediatric liver transplantation .Methods The diagnosis and treatment of PTLD were reviewed for 3 children recipient with living donor liver transplantation .Their primary diseases were biliary atresia ,glycogen storage disease type III and ornithine-transcarbamylase deficiency . All of them received FK506 for immunosuppression therapy . They were diagnosed as PTLD at 7 ,8 ,6 months post-operation respectively .Their major clinical manifestations were non-specific ,including fever ,diarrhea and anemia .Positron emission tomography/computed tomography (PET/CT) and ultrasound revealed enlarged mesenteric lymph nodes with neck lymphoadenopathy (n=2) . Pathological examinations of resected enlarged lymph nodes indicated post-transplantation lymphoproliferative disorder .One case was diffuse large B cell lymphoma and two of them belonged to preliminary EBER + . Results After a definite diagnosis ,there was one cycle of R-CHOP regimen (rituximab ,cyclophosphamide , pirarubicin ,vincristine ,dexamethasone) or 2 cycles of rituximab along with a .reduction of anti-rejection drug and they stayed in remission .Three were followed up for 37 ,39 and 20 months respectively from May 31 , 2019 . Currently transplanted liver function was stable and EBV viral load remained negative persistently .Conclusions This case highlights the complexity of clinical presentations and co-morbidities of PTLD . Reducing immunosuppressive agents and using rituximab plus chemotherapy can achieve a satisfactory efficacy for Epstein-Barr virus-related PTLD patients after pediatric liver transplantation .

Nursing experience in the process of diagnosing and treating post-transplantation lymphoproliferative disorder / 中国实用护理杂志

Objective To explore the key points of nursing in the process of diagnosing and treating the common complication--- post-transplantation lymphoproliferative disorder (PTLD) after pediatric liver transplantation. Methods The clinical data of 21 children with PTLD after liver transplantation. The key point of nursing in the process of prevention, onset, diagnosis and treatment was summarized. Results With the individual and combined treatments 18 patients gained remission except for 1 patient died because of perforation and sequent intractable haemorrhage and 2 patient died for the development of lymphoma. In terms of nursing, during the prevention stage, it is critical to insist the regular follow-up to make early diagnosis. More efforts should be made to obtain the children′s cooperation to complete the examination. During the infusion of rituximab, strengthened monitoring is necessary and more attention should be paid to the special nursing when other complications such as intractable haemorrhage, intestinal obstruction or perforation occurs. Conclusions The nursing care of PTLD is complicated. Individual and careful nursing regimens should be made based on the specific situations in different stages.

Methotrexate-associated Epstein–Barr virus mucocutaneous ulcer: A case report and review of literature

Epstein–Barr virus-positive mucocutaneous ulcer (EBVMCU) comprises part of the spectrum of B-cell lymphoproliferative disorders, reported in settings of immunosenescence and iatrogenic immunosuppression, affecting the oropharyngeal mucosa, skin, and gastrointestinal tract. We report a case of a 59-year-old female, known case of rheumatoid arthritis on methotrexate (MTX) for 15 years, who presented with an ulcer in the inner aspect of her cheek region for 2 years. Clinical examination revealed an infiltrative lesion involving the lower gingivobuccal sulcus of size 2 cm × 3 cm extending to the alveolus with level I lymph nodes, suspicious for carcinoma buccal mucosa. Anti-EBV-capsid antigen-immunoglobulin M and qualitative EBV polymerase chain reaction of peripheral blood were negative. Histopathological examination revealed atypical lymphoid cells with enlarged vesicular nuclei, prominent nucleoli, and moderate eosinophilic cytoplasm, few with binucleation (CD20 focally positive, CD79a focally positive, CD30+, EBV LMP-1+, MIB-I 60%) consistent with EBVMCU, MTX-associated. This is the first case report from India.

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

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.

Aspectos generales de algunas entidades dentro de los síndromes linfoproliferativos crónicos: La leucemia linfoide crónica / General aspects of some entities within chronic lymphoproliferative syndromes: chronic lymphocytic leukemia

La leucemia linfoide crónica es el tipo de leucemia más común en los países occidentales, afecta con mayor frecuencia al sexo masculino, con una edad promedio al diagnóstico de 65 años. La variedad más frecuente es la de estirpe B; comprenden un grupo de neoplasias biológicamente diferentes, caracterizadas por una proliferación y acúmulo de linfocitos pequeños de apariencia madura en sangre periférica, médula ósea y tejidos linfoides. Es el prototipo de enfermedad maligna que involucra a defectos de la muerte celular programada o apoptosis. Esta enfermedad puede presentar variaciones en sus características inmunofenotípicas, clínicas, citogenéticas y moleculares. Aproximadamente, el 80 por ciento de los pacientes con leucemia linfoide crónica B presentan anormalidades cromosómicas, principalmente: deleciones de los cromosomas 11,13, 6,14 y 17, estas tres últimas de mal pronóstico. Pueden presentar además, disfunciones inmunes responsables de inmunodeficiencia y autoinmunidad. Se desconoce la causa de esta enfermedad aunque los informes iniciales sugieren la implicación de los genes Bcl-1 y Bcl-2, es por eso que la terapia actual está dirigida a la inhibición de Bcl-2 por ser el responsable en la regulación de la apoptosis(AU)

Chronic lymphoid leukemia is the most common type of leukemia in Western countries, which most often affects males and the average age at diagnosis is 65 years. The most common form is the B-cell and is described in this article. LLC comprise a biologically distinct group of neoplasms characterized by proliferation and accumulation of small mature lymphocytes appearance in peripheral blood, bone marrow and tissues linfoides. Is the prototype of malignant disease involving defects programmed cell death or apoptosis. This disease may present variations in their immunophenotypic, clinical, cytogenetic and molecular characteristics. Approximately 80 percent of patients with B-CLL have chromosomal abnormalities, mainly: deletions of chromosomes 11, 13, 6, 14 and 17. These last three are bad prognosis. The patients with CLL may have also immune dysfunctions responsible for immunodeficiency and autoimmunity. It is unknown the cause of CLL although initial reports suggest the involvement of Bcl-1 and Bcl-2 gene is why the current therapy is directed to inhibition of Bcl-2 as this is responsible in regulating apoptosis(AU)

Multifocal nodular lymphoid hyperplasia of the lung / 영남의대학술지

Nodular lymphoid hyperplasia (NLH) is a benign lymphoproliferative disease that can affect the lung. Because of its rarity, little is known about the etiology and natural history of NLH. Most cases are usually asymptomatic and found incidentally on imaging studies. Imaging finding of NLH has shown most commonly as a solitary lesion, although multifocal pulmonary nodules may be seen. Surgical resection has proved curative in the cases previously described. We report a rare case of NLH in a 55 year-old man who presented with bilateral multiple pulmonary nodules on chest radiography. Open biopsy was performed from the upper and lower lobe of the left lung. The lesions were pathologically diagnosed as pulmonary NLH. Multifocal residual nodules in both lungs remain stable without spontaneous regression during the 3 years of follow-up.

Composite Cutaneous Lymphoma (Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorder) in a Patient with Rheumatoid Arthritis Treated with Methotrexate: Staging and Evaluation of Response to Therapy with ¹⁸F-FDG PET/CT / 대한핵의학회잡지

A 67 year old woman with a 10 year history of rheumatoid arthritis (RA) treated with methotrexate and prednisone, presented with a 2 year history of worsening multiple cutaneous plaques of variable appearance. Two distinct skin lesions were biopsied to reveal a composite cutaneous lymphoma, possibly caused by long term methotrexate therapy. An [18F] fluoro-2-deoxy-D-glucose (¹⁸F-FDG) positron emission tomography/computed tomography (PET/CT) was performed to stage the malignancy, and was later repeated to evaluate response to chemotherapy, which guided subsequent management. We present the PET/CT imaging findings of this very rare iatrogenic (methotrexate induced) immunodeficiency-associated lymphoproliferative disorder.

Nursing care of patients with Epstein-Barr virus related post-transplantation lymphoproliferative disorder after allogeneic hematopoietic stem cell transplantation / 中国实用护理杂志

Objective To summarize the nursing care measures about patients with Epstein-Barr virus related post-transplantation lymphoproliferative disorder (EBV-PTLD) after allogeneic hematopoietic stem cell transplantation. Methods The nursing care measures about five patients with EBV-PTLD after allogeneic hematopoietic stem cell transplantation from October 2014 to May 2015 were summarized and analyzed. Results After carefully treating and nursing care, three patients were discharged. One patient with exacerbations, respiratory, epilepsy, liver failure, and disseminated intravascular coagulation was automatically discharged and then died. And the other patient died of other diseases. Conclusions Epstein-Barr virus infection is a complication after allogeneic hematopoietic stem cell transplantation. The key points to prevention and treatment of EBV infection included monitoring regularly, diagnosing and early intervention, which was proved to be effective, timely and active.

Progress of EB virus related post-transplantation lymphoproliferative diseases / 白血病·淋巴瘤

Epstein-Barr virus (EBV) related post-transplant lymphoproliferative diseases (EBV-PTLD) is a rare and deadly complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). The clinical manifestations often show the unexplained fever, swollen lymph nodes, hepatosplenomegaly, pharyngitis, and central nervous system symptoms, with rapid disease progression and high fatality rate. As allo-HSCT for treatment of complex and refractory hemopathy has made a great progress, the prevention of graft versus host disease (GVHD) requires more application of immune inhibitors, resulting in an increasing incidence rate of PTLD. In addition to conventional treatments, such as antiviral therapy, reduction of immune suppression , local surgery and traditional chemotherapy, monoclonal antibodies and adoptive immunotherapy with T-cells for treatment of PTLD have brought a profound influence. This paper reviews the latest progress of treatment of EBV-PTLD.

Multifocal nodular lymphoid hyperplasia of the lung / 영남의대학술지

Nodular lymphoid hyperplasia (NLH) is a benign lymphoproliferative disease that can affect the lung. Because of its rarity, little is known about the etiology and natural history of NLH. Most cases are usually asymptomatic and found incidentally on imaging studies. Imaging finding of NLH has shown most commonly as a solitary lesion, although multifocal pulmonary nodules may be seen. Surgical resection has proved curative in the cases previously described. We report a rare case of NLH in a 55 year-old man who presented with bilateral multiple pulmonary nodules on chest radiography. Open biopsy was performed from the upper and lower lobe of the left lung. The lesions were pathologically diagnosed as pulmonary NLH. Multifocal residual nodules in both lungs remain stable without spontaneous regression during the 3 years of follow-up.