Enfermedad de Castleman. Reporte de un caso

Fundamento: La enfermedad de Castleman es un proceso poco común y se caracteriza por la proliferación de linfocitos no clonales. Objetivo: Describir la presentación clínica, diagnóstico y tratamiento de un paciente con enfermedad de Castleman. Presentación del caso: Paciente masculino de 53 años de edad, color de la piel blanca, que acudió al servicio de cirugía por presentar una masa en región abdominal. Con la administración de anestesia general se realizó exéresis de la lesión y se diagnosticó por el departamento de Anatomía Patológica una enfermedad de Castleman unicéntrica variedad hialino vascular. Conclusiones: La enfermedad de Castleman es poco frecuente, su sintomatología y tratamiento varían según la presentación clínica; y el diagnóstico definitivo se obtiene del análisis de la biopsia de un ganglio afectado.

Background: Castleman disease is an uncommon process and is characterized by the non-clonal lymphocyte proliferation. Objective: To describe the clinical presentation, diagnosis and treatment in a patient with Castleman disease. Case presentation: 53 years old male patient, fair skin color, who attended to the surgery service for presenting a mass in the abdominal region. With the general anesthesia administration, the lesion was excised and an unicentric Castleman disease was diagnosed by the Pathological Anatomy department, hyaline vascular variety. Conclusions: Castleman disease is not frequent, its symptomatology and treatment vary according to the clinical presentation; and the definitive diagnosis is obtained by a biopsy analysis of an affected ganglion.

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

Niveles de β2microglobulina, variables clínicas y sociodemográficas en pacientes con síndrome linfoproliferativo crónico de reciente diagnóstico

Fundamento: la β2microglobulina está reconocida como marcador tumoral para diferentes propósitos en hematopatías malignas de estirpe linfoide; sin embargo, no hay antecedentes de su utilización en la provincia de Cienfuegos. Objetivo describir las características sociodemográficas, clínicas y la distribución de los niveles séricos de β2microglobulina en pacientes con síndrome linfoproliferativo crónico y su relación con los estadios clínicos y la respuesta al tratamiento de primera línea. Métodos: estudio observacional descriptivo transversal. La serie se conformó con todos los pacientes adultos con diagnóstico reciente (sin comenzar terapia antitumoral específica) de mieloma múltiple, leucemia linfoide crónica, linfoma no Hodgkin y linfoma Hodgkin, ingresados en el Servicio de Hematología del Hospital General Universitario Dr. Gustavo Aldereguìa Lima, durante el año 2020. La información se obtuvo mediante revisión documental de historias clínicas y ensayos de laboratorio. Se analizaron las variables: sexo, edad, color de la piel, niveles de β2microglobulina, tipo de enfermedad, estadios clínicos y respuesta al tratamiento. Resultados: el 84 % de la serie presentó niveles elevados del analito, más acentuado en el mieloma. Se constató relación entre los niveles estratificados de β2microglobulina con los estadios clínicos y la respuesta al tratamiento de primera línea. Conclusiones: las características sociodemográficas y las variables clínicas observadas no difieren de forma sustantiva con lo reportado. La distribución de los niveles de la β2microglobulina es sugerente de una relación directa entre los estadios clínicos e inversa con la respuesta al tratamiento.

Background: β2microglobulin is recognized as a tumor marker for different purposes in malignant hematopathies of lymphoid lineage; however, there is no history of its use in the Cienfuegos province. Objective: to describe the sociodemographic and clinical characteristics and the distribution of serum β2microglobulin levels in patients with chronic lymphoproliferative syndrome and their relationship with clinical stages and response to first-line treatment. Methods: cross-sectional descriptive observational study. The series was made up of all adult patients (universe 50) recently diagnosed (without starting specific antitumor therapy) of multiple myeloma, chronic lymphoid leukemia, non-Hodgkin lymphoma and Hodgkin lymphoma, admitted to the Hematology Service of the Dr. Gustavo Aldereguìa Lima General University Hospital, during the year 2020. The information was obtained through documentary review of medical records and laboratory tests. The analyzed variables were: sex, age, skin color, β2microglobulin levels, type of disease, clinical stages and response to treatment. Results: 84% of the series presented high levels of the analyte, more accentuated in myeloma. A relationship was found between the stratified levels of β2microglobulin with the clinical stages and the response to first-line treatment. Conclusions: the sociodemographic characteristics and the clinical variables observed do not differ substantially from what was reported. The distribution of β2microglobulin levels is suggestive of a direct relationship between clinical stages and an inverse relationship with response to treatment.

Clinical Features and Outcomes of the Patients with B-Cell Chronic Lymphoproliferative Disease in the New Drug Era / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical characteristics of the patients with B-cell chronic lymphoproliferative disease（B-CLPD） in the new drug era and the effect of new drug treatment on efficacy and survival.@*METHODS@#The clinical and laboratory data of 200 cases B-CLPD patients diagnosed between April 2015 and August 2021 were analyzed retrospectively. The clinical efficacy and survival of the patients under different treatments including Bruton tyrosine kinase（BTK） inhibitors， rituximab， and chemotherapy alone were analyzed. The prognostic factors affecting the survival of patients were analyzed by univarite analysis and multivariate analysis.@*RESULTS@#There were 119 male（59.5％） and 81 female（40.5%） in 200 cases B-CLPD patients， the sex ratio(male/female) was 1.5∶1 with median age of 61（30- 91） years old. The distribution of subtypes were as fallows: 51 cases (25.5%) of chronic lymphocytic leukemia/small lymphocytic lymphoma（CLL/SLL）， 64（32.0%） cases of follicular lymphoma（FL）， 40（20.0%） cases mantle cell lymphoma（MCL）， 30（15.0%） cases of marginal zone lymphoma（MZL）， 10（5%） cases of lymphoplasmacytic lymphoma/waldenstrom macroglobulinemia（LPL/WM）， 5（2.5%） cases of B cell chronic lymphoproliferative disorders unclassified（B-CLPD-U） . The main clinical manifestation of 102 patients was lymph node enlargement, 32 cases were complicated with B symptoms. Among CLL/SLL patients， there were 12（23.5%） cases in Binet A and 39（76.5%） cases in Binet B/C. There were 29 patients（20.9%） in Ann Arbor or Lugano stage I-II and 110 cases（79.1%） in stage III-IV of other subtypes. The complete remission（CR） rate was 43.1%（25/58）， 40.2%（39/97）， 7.1%（1/14）， and overaIl response rate（ORR） was 87.9%（51/58）， 62.9%（61/97）， 28.6%（4/14） in the groups of BTK inhibitors， rituximab-based therapy， and chemotherapy alone. The 3-year OS rate and PFS rate in all patients was 79.2% and 72.4% respectively. The 3-year OS rate of patient with MZL, CLL/SLL, FL,WM was 94.7%, 87.7%, 86.8% and 83.3% respectively, while the 3-year OS rate of MCL was only 40.6%, which was significantly lower than other subtypes. The median OS of patients treated with BTK inhibitors and rituximab-based therapy was 20.5 and 18.5 months respectively， and the 3-year OS rate was 97.4% and 90.7%. However， the median PFS of patients receiving chemotherapy alone was 4 months， and the 1-year OS rate was 52.7%， which was statistically significant compared with the other two groups（P<0.05）. Univarite analysis showed that anemia， elevated lactate dehydrogenase， elevated β2-microglobulin， and splenomegaly were the poor prognostic factors for OS（P<0.05）， elevated lactate dehydrogenase was also poor prognostic factors for PFS（P<0.05）. Multifactor analysis showed that anemia and elevated lactate dehydrogenase were the independent poor prognostic factors for survival（P<0.05）.@*CONCLUSION@#The clinical features of B-CLPD was various， anemia and elevated lactate dehydrogenase are the prognostic factors for poor survival. BTK inhibitors and new immunotherapy can improve the survival and prognosis of patients in the new drug era.

Immunogenicity profile after COVID-19 vaccination in patients with onco-hematological diseases

ABSTRACT Objective To evaluate the influence of onco-hematological pathologies on seroconversion to COVID-19 vaccines, in addition to the effects of chemotherapy treatment on this response. Methods The present study evaluated the immunogenic response of 76 patients with onco-hematological diseases to multiple vaccine platforms compared to 25 control individuals. Results Our results showed positive response rates of 74.02% in patients with onco-hematological diseases and 100% in controls. When analyzed according to etiological group, patients with lymphoproliferative disorders achieved a positive vaccine response rate of 58.7%, whereas those with myeloproliferative diseases achieved a 100% response rate. We also observed that patients previously exposed to COVID-19 presented a 75% increase in their antibody values after vaccination, and these values were 37% higher than those of patients who did not have such exposure. We found that patients who underwent B-lymphocyte-depleting therapy in the last 2 years before vaccination had a worse response rate of 18.75%. Conclusion Despite the immunosuppression of patients with onco-hematological diseases, caused by the biology of their diseases and treatment, benefit and safety in vaccinating these patients are observed, in view of the important recall immune response and incidence of adverse effects similar to those of the healthy population.

Clinical analysis of posttransplant lymphoproliferative disorder in pediatric liver transplant recipients: a report of 11 cases / 中华器官移植杂志

Objective:To explore the clinicopathological characteristics, treatments and outcomes of posttransplant lymphoproliferative disorder(PTLD)in pediatric liver transplant recipients.Methods:From October 2016 to October 2021, retrospective data analysis was performed for 11 pediatric liver transplant recipients with PTLD. There were 5 males and 6 females with a diagnostic age of 1-8 years. Living donor liver transplantation(LDLT, n=9)and deceased donor liver transplantation(DDLT, n=2)were performed. All recipients received tacrolimus plus methylprednisolone. The major clinical manifestations included lymphadenopathy, splenomegaly, anemia, fever and digestive system symptoms(diarrhea, abdominal pain, ascites, hematochezia & intussusception, etc.). Laboratory tests hinted at hypoproteinemia, elevated transaminases and serum positivity of EBV-DNA. Positron emission tomography and computed tomography(PET-CT)revealed PTLD( n=9). Ten children were diagnosed by pathology, including lymphoid hyperplasia( n=3), plasmacytic hyperplasia PTLD( n=1), polymorphic PTLD( n=2), diffuse large B-cell lymphoma( n=2), infectious mononucleosis PTLD( n=1)and Burkitt lymphoma( n=1). Results:After a definite diagnosis of PTLD, tacrolimus was tapered or discontinued. And rituximab was prescribed. Two patients received chemotherapy(R-COP & R-CHOP)while 2 cases of local masses were operated. Up until February 2022, 10 cases survived and their conditions improved. One patient died of infection.Conclusions:PTLD is one of the most serious and fatal complications after liver transplantation in children. Clinical manifestations are diverse and an early diagnosis is difficult. The changes of EBV-DNA load should be closely monitored after liver transplantation. Imaging and pathological examinations may aid in an early diagnosis of PTLD. A treatment regimen based on immunosuppression reduction and rituximab improves the prognosis of PTLD in pediatric liver transplant recipients.

Linfoma primario no Hodgkin de mama / Primary Non-Hodgkin's Breast Lymphoma

Introducción: El cáncer de mama se ha convertido en un verdadero desafío a la calidad y expectativa de vida de la población mundial, con un aumento considerable en su incidencia y prevalencia. Cuba cuenta, desde 1987, con un Programa de Control de Cáncer Mamario. Objetivo: Ejemplificar el estudio de un caso con linfoma no Hodgkin primario de la mama, su cuadro clínico y los medios diagnósticos utilizados. Caso clínico: Paciente femenina de 70 años de edad, color de la piel negra, que acudió al Consultorio Médico de la Familia por presentar malestar general, molestias y aumento de volumen en la mama derecha. Se le remitió a consulta de cirugía y se le realizaron estudios complementarios. Se detectó nódulo de mediana densidad, contornos parcialmente definidos, distorsión del tejido vecino, otros nódulos de menos tamaño y densidad en el cuadrante inferior interno, calcificaciones vasculares en ambas mamas. Una biopsia corrobora linfoma no Hodgkin difuso de células grandes de alto grado. Conclusiones: El linfoma primario no Hodgkin de mama es muy raro y de difícil diagnóstico clínico porque no se dispone de signos, síntomas o criterios de imagen específicos para ello. El autoexamen de mama continúa siendo el principal método de diagnóstico del cáncer de mama y aunque el examen clínico, el ultrasonido de mama y la mamografía apoyan la presunción, es la biopsia quien solo brinda la confirmación diagnóstica precisa(AU)

Introduction: Breast cancer has become a real challenge to the quality of life and to life expectancy of the world population, with a considerable increase in its incidence and prevalence. Cuba has, since 1987, a breast cancer control program. Objective: To present a case with primary non-Hodgkin's lymphoma of the breast in a 70-year-old patient, its clinical picture and the diagnostic means used. Clinical case: 70-year-old female patient, with black skin, who came to the family medical office due to malaise, discomfort and increased volume in her right breast. She was referred to surgery and complementary studies were performed. Nodule of medium density was identified, with partially defined contours, distortion of the neighboring tissue, together with other nodules of less size and density in the lower internal quadrant, and vascular calcifications in both breasts. A biopsy confirms diffuse high-grade large-cell non-Hodgkin's lymphoma. Conclusions: Primary non-Hodgkin's lymphoma of the breast is very rare and difficult to diagnose clinically because there are no specific signs, symptoms or imaging criteria for it. Breast self-examination continues to be the main method of diagnosis for breast cancer and, although clinical examination, breast ultrasound and mammography support such diagnostic presumption, it is the biopsy that only provides the precise diagnostic confirmation(AU)

Interstitial mycosis fungoides / Micose fungoide intersticial

ABSTRACT Interstitial mycosis fungoides (IMF) is a rare variant of mycosis fungoides, a cutaneous T-cell non-Hodgkin's lymphoma. It is characterized by an interstitial dermal infiltrate of lymphocytes and histiocytes between collagen bundles. We report the case of a 54-year-old patient with pruritic hypochromic macules on the arms and forearms diagnosed with IMF. Special attention was given to the anatomopathological features that differentiate this entity from its differential diagnoses, such as inflammatory morphea, interstitial annular granuloma, and other variants of the mycosis fungoides itself. We also present a review of the literature on the classification of the IMF.

RESUMEN La micosis fungoide intersticial (MFI) es una variante poco común de la micosis fungoide, un linfoma cutáneo de células T no Hodgkin. Se caracteriza por un infiltrado dérmico intersticial de linfocitos e histiocitos entre haces de colágeno. Presentamos el caso de un paciente de 54 años con máculas hipocrómicas pruriginosas en brazos y antebrazos diagnosticado de MFI. Se prestó especial atención a las características anatomopatológicas que diferencian a esta entidad de sus diagnósticos diferenciales, como morfea inflamatoria, granuloma anular intersticial y otras variantes de la propia micosis fungoide. También presentamos una revisión de la literatura sobre la clasificación de la MFI.

RESUMO A micose fungoide intersticial (MFI) é uma variante rara da micose fungoide, um linfoma cutâneo de células T não Hodgkin. É caracterizada por um infiltrado dérmico intersticial de linfócitos e histiócitos entre feixes de colágeno. Relatamos o caso de um paciente de 54 anos com máculas hipocrômicas pruriginosas nos braços e antebraços com diagnóstico de MFI. Atenção especial foi dada às características anatomopatológicas que diferenciam essa entidade de seus diagnósticos diferenciais, como morfeia inflamatória, granuloma anular intersticial e outras variantes da própria micose fungoide. Apresentamos também uma revisão da literatura sobre a classificação da MFI.

Imaging features and clinical analysis of posttransplantation lymphoproliferative disorders after liver transplantation in children / 中华放射学杂志

Objective:To investigate the imaging and clinical characteristics of posttransplantation lymphoproliferative disorders (PTLD) after liver transplantation in children.Methods:From February 2017 to November 2020, the imaging and clinical data of 17 children with PTLD after liver transplantation confirmed by pathology or clinical diagnosis were retrospectively analyzed in Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiao Tong University. The site, range, density/signal/echo of the lesions were observed.Results:The mean age at transplantation was 8 (7, 11) months, and 14 patients were younger than 1 year old. The interval between liver transplantation and PTLD diagnosis was 22 (10, 34) months, ranging from 3 to 54 months. The interval was less than 1 year in 6 patients (early onset) and equal or greater than 1 year in 11 patients (late onset). Fifteen patients had Epstein-Barr virus infection. Among the 12 pathologically confirmed PTLD cases, 8 cases were diffuse large B-cell lymphoma, 3 cases were Burkitt lymphoma, and 1 case was reactive plasma cell hyperplasia. Among the 17 children with PTLD, 8 cases demonstrated involvement of lymph nodes and 16 cases had extranodal involvement. The latter included 15 cases of abdominal involvement. Abdominal sites involved included small intestine in 14 cases, colon in 7 cases, mesentery in 4 cases, kidney in 3 cases, liver in 2 cases, abdominal lymph nodes in 2 cases, peritoneum in 1 case, and stomach in 1 case. The sites of extra-abdominal involvement included lymph nodes in 7 cases, lung in 3 cases, skull in 1 case, brain in 1 case, pleura in 1 case, chest wall in 1 case, and nasopharynx in 1 case. The most common abdominal imaging abnormalities were thickening of the intestinal wall, eccentric mass and dilation of the lumen. Both small intestines and colons could be involved, and the former more commonly. Multiple masses were found in patients with liver and kidney involvement. The most common imaging manifestation of PTLD outside the abdomen was lymph node enlargement, which was found in 7 cases, and the most common was in the neck. The manifestation was shorter diameter of lymph nodes>10 mm, uniform density and signal, with mild enhancement.Conclusions:PTLD can occur months to years after liver transplantation in children, which can affect many parts of the whole body. Extranodal lesions are more than intranodal lesions. Abdominal involvement is most common in PTLD, and the infection rate of EB virus is high. Combined with medical history, EB virus infection status and imaging examination are helpful for early diagnosis.

Oral Mucosal Diffuse Large B-cell Lymphoma Caused by Long-term Oral Methotrexate:Report of One Case / 中国医学科学院学报

A case of primary oral mucosal diffuse large B-cell lymphoma(DLBCL)due to long-term use of methotrexate(MTX)for the treatment of rheumatoid arthritis(RA)was admitted to the Department of Hematology,Fujian Medical University Union Hospital.We analyzed and discussed the clinical features,diagnosis and treatment,and prognosis of specific malignant lymphoma induced by MTX in this RA patient.Our purpose is to improve the awareness and knowledge of other iatrogenic immunodeficiency-associated lymphoproliferative disorders of clinicians and pathologists.This study provides a new reference for the clinical diagnosis and treatment of MTX-associated DLBCL.

Trastornos linfoproliferativos en una cohorte de pacientes adultos con trasplante hepático atendidos en un hospital de referencia en Bogotá, Colombia / Post-transplant lymphoproliferative disorders in a cohort of adult patients with a liver transplant from a reference hospital in Bogotá, Colombia

Introducción. Los trastornos linfoproliferativos después de un trasplante se caracterizan por la proliferación descontrolada de linfocitos como consecuencia del tratamiento inmunosupresor posterior a este. Objetivo. Caracterizar clínica y patológicamente los casos de trastornos linfoproliferativos después de trasplante (Post-Transplant Lymphoproliferative Disorders, PTLD) en una cohorte de pacientes adultos con trasplante de hígado atendidos a lo largo de 15 años en el Hospital Universitario Fundación Santa Fe de Bogotá. Materiales y métodos. Se hizo un estudio observacional retrospectivo a partir de la revisión de las bases de datos de la Unidad de Trasplante Hepático y del Departamento de Patología del Hospital en busca de los casos de PTLD diagnosticados durante el periodo de estudio. Se recolectó la información epidemiológica, clínica y patológica, y se adelantaron los análisis estadísticos. Resultados. Durante el periodo de estudio, hubo 572 pacientes con trasplante de hígado, la incidencia de trastornos linfoproliferativos fue de 2,44 %, el 79 % en hombres, y la edad promedio en el momento del diagnóstico fue de 62,5 años. El 71 % de los casos se presentó durante los primeros 12 meses después del trasplante y el mismo porcentaje fue seropositivo para el virus de Epstein-Barr (EBV). El fenotipo patológico más frecuente fue el monomorfo y la mayoría de los tumores se detectaron en el hilio hepático. La supervivencia al año fue del 50 %. Conclusiones. Llamó la atención el alto porcentaje de casos de presentación temprana, así como la gran frecuencia de seropositividad para el EBV tanto en los donantes como en los receptores. Deben adelantarse estudios más detallados para una mejor comprensión de esta enfermedad en el país. Este es el primer análisis clínico y patológico de PTLD en pacientes con trasplante de hígado adelantado en Colombia hasta la fecha.

Introduction: The post-transplant lymphoproliferative disorders (PTLD) are characterized by an uncontrolled pathological lymphoid proliferation as a consequence of transplant immunosuppression therapy. Objective: To characterize the clinical and pathological characteristics of PTLD in a cohort of adult patients with liver transplant during a 15 year period at the Hospital Universitario Fundación Santa Fe de Bogota. Materials and methods: We conducted an observational retrospective study by searching for the PTLD cases diagnosed during the study period in the databases of the Liver Transplantation Unit and the Pathology Department. We collected the epidemiological, clinical, and pathological information and performed the corresponding statistics analyses. Results: During the research period, 572 patients were transplanted; the incidence of PTDL was 2.44%; 79% of them were man and the average age at the time of diagnosis was 62.5 years; 71% of the cases were diagnosed during the first year after the transplant and the same percentage EBV-seropositive patients. The most frequent pathological phenotype was monomorphic and the majority of tumors was detected in the hepatic hilum. The one-year survival was 50%. Conclusion: The high proportion of early cases and the high frequency of Epstein-Barr virus seropositivity both in donors and receptors drewour attention. More studies are necessary to have a better understanding of this condition in Colombia. This is the first PTLD clinical and pathological analysis in liver-transplant patients from Colombia to date.

Hiperplasia linfoide de intestino delgado en escolar inmunodeprimido / Lymphoid hyperplasia of small intestine in immune-depressed student

RESUMEN Fundamento: la afección intestinal es signo clave del cuadro de inmunodeficiencia. Suele presentarse un desorden de malabsorción y diarrea, con hiperplasia nodular linfoide intestinal. La importancia del estudio de esta enfermedad radica en que debe ser detectada en etapas precoces pues estos pacientes son más susceptibles a presentar neoplasias de intestino delgado. Objetivo: presentar el caso de un escolar con diarreas crónicas, desnutrición proteico energética e inmunodeficiencia como manifestaciones de hiperplasia nodular linfoide de intestino delgado. Presentación del caso: paciente de nueve años, masculino, con historia de diarreas crónicas y desnutrición. Por esta sintomatología es remitido a consulta de Gastroenterología. Conclusiones: se debe sospechar la hiperplasia nodular linfoide de intestino delgado en pacientes pediátricos con síndrome de malabsorción intestinal. Se observó mejoría clínica de las manifestaciones digestivas, del estado de inmunidad y recuperación nutricional.

ABSTRACT Background: the intestinal affection is key sign of the immunodeficiency. It usually presents a mal-absorption disorder and diarrhea, with intestinal lymphoid nodular hyperplasia. The importance of the study of this entity resides in that it should be detected in precocious stages because these patients are more susceptible to present neoplasias of small bowel. Objective: to present the case of a student with chronic diarrheas, energy protein malnutrition and immunodeficiency like manifestations of nodular lymphoid hyperplasia of small intestine. Case report: patient of nine years, masculine, with history of chronic diarrheas and malnutrition. For these clinical symptoms he is remitted to Gastroenterology consultation. Conclusions: the nodular lymphoid hyperplasia of small bowel should be suspected in pediatric patients with syndrome of intestinal mal-absorption. Clinical improvement of the digestive manifestations, of the state of immunity, and nutritional recovery was observed.

Criterios diagnósticos y nuevas opciones terapéuticas para los pacientes con diagnóstico de linfoma no Hodgkin / Diagnostic criteria and new therapeutic options for patients diagnosed with non-Hodgkin lymphoma

RESUMEN Fundamento: las hemopatías malignas día a día aumentan su incidencia y proliferación dentro de la población, entre ellas el síndrome linfoproliferativo ocupa un lugar importante, es un grupo de trastornos de origen clonal, que afecta a las células linfoides, con características heterogéneas desde el punto de vista clínico, histológico y molecular. Objetivo: agrupar diferentes criterios diagnósticos y divulgar nuevas opciones terapéuticas para los pacientes con diagnóstico de linfoma no Hodgkin. Métodos: se realizó una búsqueda bibliográfica en las plataformas de acceso a bases de datos de la red de información de salud cubana Infomed, dentro de ellas en EBSCOhost, PubMed/Medline y SciELO. Se utilizaron los siguientes descriptores: síndromes linfoproliferativos, linfomas, linfoma no Hodgkin. Resultados: se aborda la evolución histórica de los síndromes linfoproliferativos desde su descripción inicial hasta el momento actual, así como las diferentes clasificaciones, los tratamientos convencionales utilizados y una panorámica de las investigaciones que se realizan en busca de nuevos tratamientos. Conclusiones: la clasificación de los linfomas no Hodgkin ha cambiado a lo largo del tiempo, la extensión de la enfermedad tiene un papel fundamental en la selección del tratamiento y posible sobrevida del paciente. Existe una amplia variedad de prometedores agentes en desarrollo disponibles para el tratamiento del linfoma folicular.

ABSTRACT Background: malignant haemopathies increase their incidence and proliferation within the population, among them the lymphoproliferative syndrome occupies an important place, it is a group of disorders of clonal origin, which affects the lymphoid cells, with heterogeneous characteristics from the point of clinical, histological and molecular view. Objective: to group different diagnostic criteria and to disclose new therapeutic options for patients diagnosed with non-Hodgkin's lymphoma. Methods: a bibliographic search was carried out in the access platforms to databases of the Infomed Cuban health information network, within them in EBSCOhost, PubMed / Medline and SciELO. The following descriptors were used: lymphoproliferative syndromes, lymphomas, non-Hodgkin's lymphoma. Results: the historical evolution of the lymphoproliferative syndromes is addressed from its initial description to the present time, as well as the different classifications, the conventional treatments used and an overview of the research carried out in search of new treatments. Conclusions: the classification of non-Hodgkin lymphomas has changed over time; the extent of the disease has a fundamental role in the selection of treatment and possible survival of the patient. There is a wide variety of promising developing agents available for the treatment of follicular lymphoma.

Evolución de un linfoma no Hodgkin "triple expresor" en un paciente trasplantado renal con quimioterapia DA-R-EPOCH. Caso clínico / Triple expressor lymphoma in a kidney transplant patient

Patients transplanted from solid organs have an increased risk of cancer, especially lymphomas. Lymphomas correspond to 4 to 5% of malignant neoplasms in the general population and in solid organ transplant patients it reaches an incidence of 21%. The incidence of non-Hodgkin lymphomas is 10 times higher than in the non-transplanted population. We report the case of a 68-year-old man with a kidney transplant who 6 years after transplantation, developed a non-Hodgkin diffuse large cells B lymphoma with lymph node and pulmonary involvement, with markers of very poor prognosis (triple MYC expressor, BCL2 and BCL6). and its evolution with chemotherapy with DA R EPOCH.

Case for diagnosis. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Abstract: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease, with an indolent evolution and benign course. The classic presentation is a solitary nodule on the face or trunk. The disorder's rarity and clinical and histopathological characteristics, can make the diagnosis difficult. We present the case of a 36-year-old Caucasian woman with a purplish erythematous nodule, hardened, shiny, asymptomatic, on the left nasal ala, which had grown progressively for 45 days. Histopathological examination and immunohistochemistry panel demonstrated alterations consistent with primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was complete remission of the condition within 60 days of treatment with potent occlusive corticosteroids.

Posttransplant lymphoproliferative disorders: a clinicopathological analysis of 11 cases / 肿瘤研究与临床

Objective@#To analyze the clinicopathological features of the posttransplant lymphoproliferative disorders (PTLD) and to improve the diagnostic levels.@*Methods@#The clinical data of 11 patients diagnosed with PTLD between January 2008 and January 2018 from Henan Provincial People's Hospital, Peking University Science Center and the Affiliated Third Hospital of Peking University were collected. The clinicopathological features and the potential prognostic predictors were retrospectively analyzed by using immunohistochemical staining, EB virus in situ hybridization, fluorescence in situ hybridization and gene sequencing.@*Results@#There were 9 males and 2 females in 11 PTLD patients, and the median age of the total patients was 18 years old (3-34 years old). The median time of 9 cases who underwent hematopoietic stem cell transplantation developing PTLD was 4 months (2-24 months) after the transplantation. The other 2 cases undergoing solid organ transplantation (SOT) occurred PTLD after 6 months and 13 months, respectively. The lymph node was the most common site to be involved (9 cases), 1 case occurred in liver and 1 case occurred in nasopharynx site. Among 11 patients, 3 cases were classified as polymorphic PTLD (P-PTLD) and the other 8 cases were monomorphic PTLD (M-PTLD). EB virus of all cases was positive, and 8 cases of M-PTLD were classified as diffuse large B-cell lymphoma (DLBCL). Fluorescence in situ hybridization was used to detect bcl-2, myc, IGH and A20 gene, and only one case had the gene break of IGH, while other cases didn't find any other abnormalities. Ig gene clone analysis was made in 5 patients with PTLD, including 4 cases of M-PTLD with gene rearrangement and 1 case of P-PTLD without gene rearrangement. Univariate analysis showed that age (≤18 years old) was associated with poor prognosis (P = 0.040).@*Conclusions@#The clinicopathologic features of PTLD are various and infected by EB virus. Gene rearrangement can help the diagnosis.

Classification for primary cutaneous lymphoproliferative disorders: an update / 中华皮肤科杂志

Primary cutaneous lymphoproliferative disorders are a group of heterogeneous diseases that mainly involve the skin and are completely different from lymph node lymphomas. Although rare, they are still an important part of lymphoid and hematopoietic tumors. In the 2017 World Health Organization (WHO) classification for tumors of hematopoietic and lymphoid tissues and the 2018 updated version of WHO-European Organization for the Research and Treatment of Cancer (EORTC) classification for cutaneous lymphomas, classifications of most diseases do not change, but the understanding of some diseases such as Epstein-Barr virus-associated lymphoproliferative diseases and primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder has been changed, and new diseases have been added. This article introduces updates on the classification of primary cutaneous lymphoproliferative disorders based on the 2017 WHO and 2018 WHO-EORTC classification systems.

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.

Clinicopathological features of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract: a report of five cases / 中华病理学杂志

Objective@#To investigate the clinicopathological features of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract.@*Methods@#Five cases of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract from the Affiliated Hospital of Qingdao University from 2016 to 2019 were retrospectively reviewed. The clinical and pathological parameters were analyzed by combining clinical data and reviewing the available literature of 35 cases (34 cases abroad and 1 case in China).@*Results@#There were 4 males and 1 female with a median age of 47 years (18-66 years). All patients had abdominal pain and constitutional symptoms including diarrhea, emaciation, intermittent mucous stool or oral and epiglottic ulcers. Endoscopic manifestations included multiple punctate congestion, erosion and ulcer at the terminal ileum and colorectum. Two cases had congestion and erosion of antrum and angle of stomach, and the lesions did not fuse and form tumors. Histologically, the lamina propria was expanded by a dense, medium to small lymphocyte infiltration, which was monomorphic, with slightly irregular nuclei without prominent nucleolus or lymphoepithelial lesions. There were admixed small amount of plasma cells and eosinophils. In 4 cases, immunohistochemistry showed the lesional cells were positive for CD3, CD8, TIA1, and negative for CD4, CD56, granzyme B and Ki-67 index was ≤10%. In situ hybridization showed that EBER was negative and clonal TCR gene rearrangement was detected. One consultation case was CD3+, CD5- and Ki-67 index of 10%, although other indicators were not done. All five patients were treated with symptomatic support. In follow-up observation for 2 to 25 months, all patients were alive with the disease.@*Conclusions@#Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract is a newly classified monoclonal T-cell proliferative disease, with low incidence, clinical inertia and long-term survival. It has unique clinicopathological features but pathologically it is easily misdiagnosed as inflammatory bowel disease or T-cell lymphoma. Correct diagnosis is of great important clinical significance.

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.