Enfermedad de Castleman unicéntrica, una causa infrecuente de masa cervical / Unicentric Castleman disease, an infrequent cause of cervical mass

La enfermedad de Castleman (EC) describe una serie de desórdenes linfoproliferativos de patrones histológicos similares, pero de etiologías, presentaciones clínicas y enfrentamientos notoriamente variables. Se presenta el caso de un paciente que consultó por masa cervical, cuyo estudio final concluyó EC unicéntrica, la cual se resolvió de forma quirúrgica. Además, se presenta una revisión actualizada del tema, con foco en la enfermedad unicéntrica.

Castleman's disease (CD) describes several lymphoproliferative disorders with similar histological patterns, but with notoriously variable etiologies, clinical presentations, and management. We present the case of a patient who consulted with cervical mass, whose final study concluded with unicentric CD, which was treated surgically. In addition, an updated review of the subject is presented, focusing on unicentric disease.

Clinical Features and Prognosis of Patients with Castleman's Disease / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical features and prognosis of patients with Castleman's disease (CD) and improve the diagnosis and treatment of CD.@*METHODS@#Clinical data of patients diagnosed with CD by pathological biopsy in Gansu Provincial Hospital from January 2009 to November 2020 were retrospectively analyzed. According to clinical classification, the patients were divided into two groups: UCD (unicentric CD) group (n=20) and MCD (multicentric CD) group (n=9). The clinical manifestations, laboratory examination, treatment regimens, pathological examination and follow-up data were statistically analyzed.@*RESULTS@#There were no significant differences in average age and gender ratio between UCD group and MCD group. In UCD patients, 80.0% were hyaline vascular type, and 20.0% were plasma cell type. In MCD patients, 33.3% were hyaline vascular type, 55.6% were plasma cell type, and 11.1% were mixed type. There was significant difference in pathological classification between the two groups (P=0.039). The UCD patients usually presented asymptomatic single lymph node enlargement with mild clinical symptoms, while the MCD patients were characterized by multiple superficial and deep lymph node enlargement throughout the body. The incidences of asthenia, splenomegaly, serous effusion in MCD group were higher than those in UCD group (P<0.05). Meanwhile, the incidences of anemia, hypoproteinemia, increased ESR, elevated serum globulin and elevated β2-microglobulin were significantly higher than those in UCD group too (P<0.05). There was no significant difference in the incidences of abnormal WBC, PLT and elevated LDH between the two groups (P>0.05). Among 20 patients with UCD, 13 cases reached complete remission (CR), 1 case achieved partial remission (PR). Among 9 patients with MCD, 3 cases received CR and 4 cases received PR.@*CONCLUSION@#Patients with CD requires pathological examination for diagnosis. Patients with UCD show mild clinical symptoms, good surgical treatment effect and good prognosis. Patients with MCD have diversified clinical manifestations and relatively poor prognosis, and these patients require comprehensive treatment.

Pathological significance of plasma cell infiltration in diagnosing lymph node diseases / 中华病理学杂志

Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.

TAFRO syndrome mimicking systemic lupus erythematosus: Case report and literature review

TAFRO syndrome is a very rare disease, with less than 100 cases reported in the literature. It is classified as a type of idiopathic multicentric Castleman disease, but it has clinical, paraclinical, and histopathological characteristics that differentiate between TAFRO and idiopathic forms of Castleman disease not otherwise specified. However, it is a challenging exclusion diagnosis. TAFRO syndrome is characterized by systemic inflammatory involvement, often severe, which can present with kidney failure, and become a severe disease with a high mortality rate. The clinical manifestations of TAFRO can be confused with hematology malignancies or various autoimmune diseases. Although there are some reports of TAFRO syndrome associated with autoimmune compromise, there is no published consensus for the diagnosis or treatment. The case presented is a patient who meets the criteria to be classified as SLE, and with manifestations with significant clinical involvement, but with no improvement with standard treatment. It was found that the patient's systemic involvement was due to TAFRO, and that therefore the TAFRO syndrome could simulate SLE, something previously not described in the literature.

El síndrome TAFRO es una enfermedad muy poco común, con menos de 100 casos reportados en la literatura. Se clasifica como un tipo de enfermedad de Castleman multicéntrica idiopática, pero tiene características clínicas, paraclínicas e histopatológicas que permiten diferenciarla de las formas de la enfermedad Castleman idiopática no clasificadas de otra manera; sin embargo, es un diagnóstico de exclusión difícil de hacer. El síndrome TAFRO se caracteriza por compromiso inflamatorio sistémico, en muchas ocasiones severo, que puede presentarse con falla renal y convertirse en una enfermedad grave, con una alta tasa de mortalidad. Las manifestaciones clínicas de TAFRO pueden confundirse con neoplasias hematológicas o varias enfermedades autoinmunes. En la literatura existen algunos reportes de síndrome TAFRO asociados con compromiso autoinmune, pero no se ha publicado un consenso para su diagnóstico ni para su tratamiento. El caso que se presenta es un paciente que cumple con los criterios para ser clasificado como LES, que tenía manifestaciones con gran compromiso clínico, pero sin mejoría con el tratamiento estándar. Se encontró que el compromiso sistémico del paciente era por TAFRO y que, por lo tanto, el síndrome TAFRO podría simular LES, algo no descrito previamente en la literatura.

Enfermedad de Castleman multicéntrica en pacientes HIV positivos. Reporte de dos casos y revisión de la literatura / Multicentric Astleman disease in HIV positive patients. Two cases. Report and literature review

La enfermedad de Castleman (EC) es un proceso linfoproliferativo poco frecuente que se caracteriza por hiperplasia de los ganglios linfáticos. Existen dos variedades histológicas bien diferenciadas la hialino-vascular y la plasmocelular, que a su vez pueden ser localizadas o multicéntricas. La forma hialino-vascular suele ser asintomática y localizada en mediastino mientras que la plasmocelular se presenta frecuentemente con signo-sintomatología sistémica y suele ser difusa o multicéntrica. En el contexto de la enfermedad debida al virus de la inmunodeficiencia humana (VIH), la EC se asocia en su patogenia a la infección por el herpes virus humano tipo-8 (HHV-8). La mayoría de los casos corresponden a la variante hialino-vascular (80/90%) en tanto un pequeño porcentaje (10/20%) son de la variante plasmocelular. En algunos pacientes, el patrón histopatológico puede ser mixto. Se describen dos casos de enfermedad de Castleman multicéntrica HHV8- positiva en pacientes con enfermedad HIV/SIDA.

Castleman's disease (CD), is a rare hematological condition of uncertain etiology, involves a massive proliferation of lymphoid tissues and typically presents as mediastinal masses. This is considered as a distinct type of lymphoproliferative disorder associated with inflammatory symptoms. In the context of human immunodeficiency virus (HIV) infection, CD is associated with human herpesvirus-8 (HHV8) infection. Most cases of CD represent either the hyaline vascular variant (80­90% of cases) or the plasma cell variant (10­20%); a small percentage present with a mixed histologic appearance. Two cases of Castleman's disease associated with HHV-8 and HIV/AIDS infection are described

Anemia hemolítica autoimune na doença de Castleman multicêntrica: relato de caso / Autoimmune hemolytic anemia in multicentric Castleman's disease: case report

A doença de Castleman é um distúrbio linfoproliferativo raro, podendo se manifestar sob a forma de massas localizadas ou como doença multicêntrica. A doença de Castleman multicêntrica é caracterizada por adenopatias generalizadas, visceromegalias, manifestações autoimunes e infecções recorrentes. Este artigo apresenta o relato de caso de anemia hemolítica autoimune por anticorpos quentes em paciente com doença de Castleman multicêntrica. Resposta eficaz foi obtida com uso de corticoterapia sistêmica e tocilizumabe.

Castleman disease is a rare lymphoproliferative disorder that can manifest as localized masses or as multicentric disease. Multicentric Castleman disease is characterized by generalized adenopathies, visceromegaly, autoimmune manifestations, and recurrent infections. This article presents the case report of a patient with multicentric Castleman's disease and autoimmune hemolytic anemia by warm antibodies. Effective response was obtained with systemic corticotherapy and tocilizumab.

Clinicopathological and therapeutic analysis of Castleman's disease in children / 中华儿科杂志

Objective: To investigate the clinical features, pathological phenotype, treatment and prognosis of Castleman's disease in children. Methods: Clinical data of 15 children diagnosed with Castleman's disease in Henan Provincial People's Hospital and the First Affiliated Hospital of Zhengzhou University from May 2010 to October 2019 were analyzed retrospectively. The clinical characteristics, laboratory examination and histopathological data were analyzed. Results: Among the 15 Castleman's disease patients, 12 were males and 3 females. The age of first visit was 12 (10, 15) years. The time from mass discovery to pathologic diagnosis was 9.0 (2.0, 13.0) months. The majority of patients were unicentric (13 cases), and the histopathological type was hyaline vascular (11 cases). Unicentric lesions were most common in the neck (11 cases), all 13 patients received complete surgical resection of the lesions, the follow-up time was 20.0 (13.5, 50.5) months, and the prognosis was good. Two cases were multicentric type, the pathological types were mixed variant, meeting the criteria of idiopathic Castleman's disease, the two children underwent partial surgical resection, one was treated with rituximab and prednisone and the other was treated with thalidomide and prednisone. The follow-up time was 32 months and 10 month, both of them had good prognosis. Conclusions: Most cases of Castleman's disease in children are diagnosed late, and the unicentric type is dominant. The most common pathological type is hyaline vascular, which is characterized by painless lymphadenopathy, while multicentric type has systemic symptoms and both of them have a good overall prognosis.

Enfermedad de Castleman variante multicéntrica: reporte de caso / Castleman's disease multicentric variant: case report

La enfermedad de Castleman es un reto diagnóstico que se debe tener presente en pacientes con linfadenopatías, debido a que pertenece a un grupo heterogéneo de trastornos linfoproliferativos, fue descrita por Benjamín Castleman en 1956, es una patología poco frecuente, de etiopatogenia aún no establecida, en el que intervienen varios factores como ser: la estimulación antigénica viral, VHH-8, VIH, inmunodeficiencias, elevados niveles de IL-6 que llevan a un proceso inflamatorio lo que desencadena una sintomatología diversa, el diagnóstico definitivo se realiza mediante biopsia excisional. Se presenta el caso clínico de una paciente femenina de 41 años de edad, que acude al servicio de emergencias con cuadro clínico de aproximadamente 1 año de evolución caracterizado por presentar pérdida de peso progresivo, adenopatías palpables, evidencia de proceso linfoproliferativo y esplenomegalia observados en la tomografía, VIH negativo, anemia y compromiso del estado general.

Castleman's disease is a diagnostic challenge that must be taken into account in patients with lymphadenopathy, because it belongs to a heterogeneous group of lymphoproliferative disorders, it was described by Benjamin Castleman in 1956, it is a rare pathology, of still unknown etiopathogenesis, In which several factors intervene such as viral antigenic stimulation, HHV-8, HIV, immunodeficiencies, high levels of IL-6 that lead to an inflammatory process that produces diverse symptoms, the definitive diagnosis is made with excisional biopsy. We present the clinical case of a 41-year-old female patient who came to the emergency department with a clinical picture of approximately 1 year of evolution characterized by progressive weight loss, palpable lymphadenopathy, evidence of lymphoproliferative process and splenomegaly in the tomography, HIV negative, anemia and compromise of the general state.

A case report of TAFRO syndrome / 北京大学学报(医学版)

The study is to improve clinicians' understanding of TAFRO syndrome, to explore the diagnosis and treatment of TAFRO syndrome and to identify TAFRO syndrome in the early stage. The clinical manifestations, laboratory examination results, imaging manifestations, diagnosis and treatment of TAFRO syndrome were reported, and the literature of TAFRO syndrome was reviewed. The main clinical manifestations of the female were intermittent vaginal bleeding, fever, depressive edema of both legs, red blood cell and thrombocytopenia, and renal function deterioration. The results showed that leukocytes increased, anemia, thrombocytopenia and severe renal dysfunction were found; With fever, C-reactive protein and procalcitonin increased significantly, bone penetration suggested that granulocyte proliferation was active, and megakaryocytes were seen. But anti-infection treatment was ineffective; CT suggested that there was a high uptake of multiple fluorodeoxyglucose (FDG) in many parts of the body; The lymph node biopsy was considered to be in accordance with the transparent vascular type of Castleman disease; Renal biopsy was used to return thrombotic microvascular disease with subacute renal tubulointerstitial nephropathy. In terms of treatment, the clinical condition of the patients was improved after methylprednisolone (60 mg, once a day), the temperature was normal, and the effusion in the serous cavity was better than before. The blood transfusion and platelet support therapy were intermittently given. Hemoglobin and platelets were increased in sex, and the urine volume increased to 1 000 mL/day. However, the platelet dropped at a later time, after 1 month of treatment with topizumab, the clinical condition of the patients was further improved. At present, the blood pigment and platelets returned to normal and had been separated from dialysis. TAFRO syndrome is a special subtype of idiopathic multicentric Castleman disease, and it is a group of systemic inflammatory diseases with its own characteristics. Its clinical manifestations and diagnosis and treatment are unique compared with other idiopathic multicentric Castleman diseases. For the enlargement of lymph nodes of unknown reasons, it is suggested to improve the lymph node biopsy actively. Renal insufficiency is an important part of TAFRO syndrome. Renal biopsy is of great significance to study the pathogenesis of TAFRO syndrome and to judge the prognosis of patients. The clinical diagnosis of the disease requires comprehensive clinical manifestations and the results of various examinations. Early diagnosis and early treatment of the disease can often achieve good clinical effect.

Follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease in association with paraneoplastic pemphigus: study of the tumor and successful treatment

Abstract The authors have successfully treated and monitored a case of paraneoplastic pemphigus in association with follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease. The patient was a 56-year-old female who presented with recalcitrant erosive lichen planus of the oral cavity, tongue, and genital mucosa, along with polymorphous eruptions throughout her body. Histological examination of the cutaneous lesions, indirect immunofluorescence on rat bladder epithelium, and western blot of human keratinocyte proteins identified anti-epidermal antibodies in the patient's serum. Positron emission tomography and computed tomography scans found a mass in her retroperitoneal region. Pathology and immunohistochemistry investigation further corroborated the diagnosis of follicular dendritic cell sarcoma originated from hyaline-vascular Castleman's disease. Complete remission was achieved and the patient has been monitored for four years.

Idiopathic multicentric Castleman's disease, infrequent cause of Lupus-like: case report

Castleman's disease (CD) or angiofollicular lymph node hyperplasia includes a heterogeneous mix of reactive lymphoproliferative processes with well-defined histological features. However, they differ in their localization patterns, clinical expression and etiopathogenesis. There are 4 types, one of them is the multicentric CD that is not associated with any viruses and has re-cently been called idiopathic MCD (iMCD). iMCD is a lymphoproliferative disorder with specific histopathological characteristics, more than one region of affected lymph nodes and absence of infection associated to human herpesvirus 8 and human immunodeficiency virus (HIV). iMCD covers multiple differential diagnoses and might simulate autoimmune diseases such as syste-mic lupus erythematosus. The aim of this article is to report the case of a patient with Castle-man's disease and lupus-like presentation. We present the case of a 38-year-old man without morbid history, who presented lumbago, fever, diaphoresis and asthenia with two months of evolution, associated to bilateral cervical adenopathies. General examinations result negative, antinuclear antibodies at a dilution of 1/640 were positive, and extractable nuclear antigens were positive suggesting moderate Systemic Lupus Erythematosus (SLE) plus secondary Sjö-gren's. Methylprednisolone and Hydroxycloroquine boli were thus initiated. The patient evolved with anasarca, severe anemia, acidosis, polyserositis and multiple mediastinal adenopathies. Immunoglobulin and cyclophosphamide were thus initiated. He later presented fever, throm-bocytopenia and nephrotic syndrome. Biopsy of cervical lymph node reported lymphadenitis with polyclonal plasmacytosis and concentric lymphoid hyperplasia, in agreement with iMCD. Treatment with Rituximab was initiated, which led to the favorable evolution of the patient. iMCD is a systemic inflammatory disease, its presentation corresponds to a constitutional syndrome resulting in a wide differential diagnosis. Every time suspicious adenopathies appear, they must be biopsied since this might lead to a definitive diagnosis

Preliminary Study on the Characteristic of Plasma Cytokine Profiles in Patients with Idiopathic Multicentric Castleman Diseases / 中国实验血液学杂志

OBJECTIVE@#To investigate the characteristic changes of the plasma cytokine profile in Chinese patients with idiopathic multicentric Castleman diseases (iMCD).@*METHODS@#The plasma samples from 22 patients with confirmed diagnosis of iMCD were collected before treatments; Specimens from 17 patients with newly diagnosed multiple myeloma, 10 non Hodgkin's lymphoma, and 15 healthy donors were used as control. Seventeen kinds of cytokines were measured by cytokine beads array (CBA) and ELISA respectively.@*RESULTS@#Six cytokines were measured by ELISA. The concentrations of IL-2, IL-6, IL-21 and VEGF were significantly higher in the plasma of iMCD patients than those of the healthy donors (P＜0.01) and the level of IL-21 was highest in the iMCD group. There was no significant difference in the levels of IL-1β and IL-4 between the iMCD and healthy donor groups. Thirteen cytokines were measured by CBA assay, besides IL-6 level was confirmed to be higher in iMCD group than that in healthy controls (P＜0.01）, IL-12-p70 and IL-33 levels were also higher in iMCD group than those in control group (P＜0.05）, no significant difference of the rest cytokines was found between iMCD and the control group.@*CONCLUSION@#IL-6 and VEGF has shown to involved in the pathogenesis of iMCD, the results of preliminary study imply the role of IL-2 、IL-21、IL-12-p70 and IL-33 in this rare lymphoproliferative disease. Further studies are needed to elucidate the mechanism of these cytokines, which may shed some light on the identification of novel therapeutic targets against iMCD.

A case of Castleman disease that improved after kidney transplantation / 대한이식학회지

This is a case of a 56-year-old man with Castleman disease (CD) who improved after kidney transplantation (KTP). CD is an uncommon lymphoproliferative disorder that was found incidentally on biopsy during dialysis in the current patient and was followed up without further treatment. However, the lesion showed improvement after KTP. Therefore, active KTP can be considered even if CD is one of the lymphoproliferative disorders that can occur as a complication after KTP.

Thoracic Splenosis after Splenic and Diaphragmatic Injury / 대한흉부외과학회지

Thoracic splenosis is a rare disease that develops as a result of autotransplantation of splenic tissue into the thoracic cavity following splenic and diaphragmatic injury. We report the case of a 53-year-old man with a chief complaint of heartburn and cough. He had a history of traumatic diaphragmatic rupture treated with surgical repair and splenectomy 15 years ago. Imaging studies revealed a paraesophageal mass, and surgical resection was performed considering the possibility of Castleman disease or an esophageal submucosal tumor. Pathologic results showed findings of normal splenic tissue. The patient was discharged on postoperative day 5 without any complications.

Development of Castleman Disease in the Paravertebral Space Mimicking a Neurogenic Tumor / 대한흉부외과학회지

Castleman disease is a relatively rare disease, characterized by well-circumscribed benign lymph-node hyperplasia. The disease may develop anywhere in the lymphatic system, but is most commonly reported as unicentric Castleman disease in the mediastinum along the tracheobronchial tree. It is usually asymptomatic and detected on plain chest radiography as an incidental finding. We report an incidentally detected case of Castleman disease in the paravertebral space that was preoperatively diagnosed as a neurogenic tumor and treated by complete surgical resection.

Síndrome POEMS: um diagnóstico desafiador / POEMS Syndrome: a challenging diagnosis

A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)

POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)

Calcified fibrous pseudotumor with Castleman disease

Simultaneous calcified fibrous pseudotumor (CFT) and Castleman disease (CD) is an extremely rare association. CD is an uncommon lymphoproliferative disease that can arise in various sites of the body, while CFT is a rare type of benign fibrous lesion that frequently affects children and young adults, occurring as solitary or multiple lesions throughout the human body. Both entities are rare and exhibit typical and diverse histomorphological features. We report the case of a 15-year-old female patient, who, at the age of 13 had a biopsy performed at an external medical center; however, after 4 months the lesion had regrown. This lesion was removed with a surgical operation; however, it regrew 2 years later and was removed a third time. The results of the latter two biopsies were the same: CFT accompanying CD. The histologic examination of the excised lymph node and the surrounding tissue showed hyalinized fibrous tissue containing dystrophic and psammomatous calcification. In this case, the hyaline vascular type of CD was found to be intertwined with a CFT, which hampered the differentiation of whether both entities emerged within the lymph node or if the CFT developed from the soft tissue and then involved the lymph node. Future studies involving larger case series will provide a more precise insight, which should serve to resolve the current uncertainty.

Hiperplasia linfoide ileo-colónica en pacientes pediátricos / Ileo-colonic lymphoid hyperplasia in pediatric patients

Introducción: La Hiperplasia nodular linfoide gastrointestinal constituye una entidad infrecuente con manifestaciones clínicas diversas y con mayor frecuencia en la edad pediátrica. Objetivo: Caracterizar clínica, endoscópica e histológicamente a los pacientes pediátricos con hiperplasia linfoide de colon e íleon terminal diagnosticados en el Instituto de Gastroenterología. Material y Métodos: Se realizó un estudio descriptivo, transversal, en el periodo comprendido entre 2014 y 2016 en el Instituto de Gastroenterología. La muestra estuvo constituida por 50 pacientes, quienes cumplieron los criterios de inclusión y exclusión. Se evaluaron variables demográficas, clínicas, endoscópicas e histológicas, así como el comportamiento de la comorbilidad con enfermedades malignas, enfermedades inflamatorias intestinales, giardiosis, trastornos de la respuesta inmunohumoral y alergias alimentarias. Resultados: El sexo masculino, entre 7-10 años y el color de la piel blanca fueron los más frecuentes. El sangrado rectal fue el síntoma principal (62 por ciento) y la localización a nivel del íleon terminal en 69 por ciento, no se relacionó con enfermedad maligna, hubo tres pacientes con diagnóstico de hiperplasia linfoide de íleon terminal y colitis ulcerosa. El 74 por ciento presentó aspecto nodular por histología y 60 por ciento se le diagnosticó Giardia lamblia, en la evaluación inmunohumoral predominó los pacientes sin inmunocompromiso (78 por ciento), el Prick Test fue positivo en 60 por ciento, sobre todo, a la leche de vaca. Conclusiones: La manifestación clínica que predominó fue el sangrado rectal, endoscópicamente la localización en íleon y la forma nodular por histología. No encontramos comorbilidades con enfermedades malignas y fueron más frecuentes los trastornos alérgicos y parasitarios que las alteraciones inmunológicas(AU)

Introduction: Lymphoid nodular hyperplasia of the gastrointestinal tract is an uncommon entity with diverse clinical manifestations, which is more frequent in the pediatric age. Objective: To characterize clinically, endoscopically, and histologically, those pediatric patients with lymphoid hyperplasia of the colon and terminal ileum diagnosed in the Institute of Gastroenterology. Material and Methods: A descriptive, cross-sectional study was carried out during the period between 2014 and 2016 in the Institute of Gastroenterology. The sample consisted of 50 patients who met the inclusion and exclusion criteria. Demographic, clinical, endoscopic and histological variables were evaluated, as well as the behavior of comorbidity with malignant diseases, inflammatory bowel diseases, giardiasis, disorders of the humoral immune response and food allergies. Results: The male sex, the age group between 7-10 years, and the white skin color were the most frequent. Rectal bleeding was the main symptom (62 percent), and the location of the lesions in the terminal ileum was observed in 69 percent of the patients. There was no relationship between lymphoid hyperplasia and malignant disease, but three patients were diagnosed with lymphoid hyperplasia of the terminal ileum, and ulcerative colitis. 74 percent of the biopsies presented a nodular variety, and 60 percent of the patients were diagnosed with Giardia lamblia; the cases that were not immunocompromised prevailed in the evaluation of humoral immune response (78 percent); the Prick Test was positive in 60 percent of children, especially to cow's milk. Conclusions: The predominant clinical manifestation was rectal bleeding; the localization was in the ileum, which was seen by endoscopic procedure; and the nodular form was demonstrated by histology. We did not find comorbidities with malignant diseases, and allergic and parasitic disorders were more frequently diagnosed than immunological alterations(AU)

Scleritis in a Patient with Castleman Disease

PURPOSE: To report a case of multicentric Castleman disease that presented with scleritis. CASE SUMMARY: A 42-year-old male presented with decreased visual acuity in the left eye. Castleman disease had been diagnosed 21 months before and treated with systemic steroids and combined chemotherapy. Best-corrected visual acuity (BCVA) of the left eye was 0.02 and the intraocular pressure was 42 mmHg. Scleral edema and corneal edema were noted using a slit lamp examination. The anterior chamber cell was 2+ according to Standardization of Uveitis Nomenclature criteria. The fundus was invisible due to the anterior segment lesion. After one month, scleritis developed in the right eye and the patient complained of ocular pain. Topical steroids and non-steroidal anti-inflammatory drugs were prescribed. Due to recurrent scleritis and anterior uveitis, cataract extraction and laser iridectomy were performed on the left eye, and systemic steroids and the antimetabolite methotrexate were started. After 9 years of follow-up, all medications were stopped and there was no recurrence of inflammation, with a BCVA of 1.0 in both eyes. CONCLUSIONS: Treatment of a patient with scleritis accompanied with Castleman disease using systemic steroids and methotrexate resulted in a good prognosis.