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.

Enfermedad de Castleman unicéntrica, una causa infrecuente de masa cervical

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.

Enfermedad de castleman multicéntrica y tuberculosis diseminada en paciente HIV positivo / Multicentric Castleman disease and tuberculosis in HIV positive patient

Resumen Presentamos el caso de un hombre de 40 años, HIV positivo con regular adherencia al tratamiento, que consultó por episodios febriles intermitentes de dos años de evolución, agregando en los últimos dos meses dolor abdominal difuso progresivo y adenomegalias ge neralizadas indoloras. En el laboratorio presentó panci topenia, coagulopatía, hipoalbuminemia y reactantes de fase aguda aumentados. La tomografía computarizada (TC) de tórax, abdomen y pelvis únicamente evidenció hepato-esplenomegalia y adenomegalias generalizadas. Se realizaron múltiples exámenes microbiológicos, in cluyendo cultivos para Mycobacterium sp. de distintas muestras, todos con resultados negativos, a excepción de la RT-PCR para VHH-8. Se tomó biopsia escisional de ganglio iliaco izquierdo con hallazgos compatibles con enfermedad de Castleman. A pesar de reiniciar terapia antirretroviral, la sintomatología progresó, iniciando tratamiento con corticoides y ganciclovir. Luego de una semana, desarrolló falla mulitorgánica y anasarca, que contraindicaron los fármacos iniciados. Se realizó nueva TC de tórax que mostró infiltrados con patrón en árbol en brote en lóbulo superior derecho asociado a derra me pleural bilateral, y a nivel abdominal, progresión de hepato-esplenomegalia y ascitis. Pasó a unidad de cui dados intensivos 48 horas después por falla hepática ful minante. El paciente falleció en pocas horas. Se recibió postmortem el cultivo del aspirado traqueal positivo para Mycobacterium tuberculosis y la punción-biopsia hepática con granulomas no necrotizantes. Nuestro objetivo es remarcar la importancia de pesquisar tuberculosis activa en nuestro país frente a la sospecha de enfermedad de Castleman antes de iniciar tratamiento, y resaltar que la TBC también puede presentarse como complicación infecciosa elevando la morbimortalidad.

Abstract We present the case of a 40-year-old man, HIV posi tive with regular adherence to treatment, who consulted for intermittent febrile episodes during a two-years period, progressive diffuse abdominal pain and painless generalized lymphadenopathy in the last two months. Laboratory analysis showed pancytopenia, altered co agulation tests, hypoalbuminemia, and increased acute phase reactants. Computed tomography (CT) of the chest, abdomen and pelvis revealed hepato-splenomegaly and generalized lymphadenopathy. Multiple microbiological tests were performed, including cultures for Mycobacte rium sp. from different samples, with negative results, except for the RT-PCR for HHV-8. An excisional biopsy of the left iliac lymph node was taken with findings compatible with Castleman's disease. Despite restarting antiretroviral therapy, the symptoms progressed, starting treatment with steroids and ganciclovir. After a week, he developed multiple organ failure and anasarca, which contraindicated the drugs previously started. A new CT of the chest showed infiltrates with a tree-in-bud pattern in the right upper lobe with bilateral pleural effusion, and at the abdominal level, progression of hepatospleno megaly and ascites. He was transferred to the intensive care unit 48 hours later due to fulminant hepatic fail ure. The patient died within a few hours. Postmortem recieved culture results of the tracheal aspirate were positive for tuberculosis (TB) and the histopathology of the liver biopsy showed non-necrotizing granulomas. Our objective is to highlight the importance of screening for active TB, in our country, when Castleman's disease is suspected, before starting treatment, and to stand out that TB can also present as an infectious complication, increasing morbidity and mortality.

Clinical analysis of intraabdominal unicentric Castleman disease:report of 8 cases / 中华普通外科杂志

Objective:To summarize the clinical characteristics of patients with unicentric Castleman disease(UCD).Method:The clinical data of 8 abdominal UCD patients who received surgical resection at the Second Hospital of Hebei Medical University from Oct 2019 to Oct 2022 were analyzed, and the imaging characteristics, pathological types and prognosis were summarized.Result:There were 2 males and 6 females. The average age of patients was (33.0±13.7) years old, and their BMI was (23.2±4.5) kg/m 2. The median maximum diameter of the tumor was 4.5 (3.0-4.9) cm. The average postoperative hospital stay was 6.5 (3.3-12.0) days. One was lost during follow up, there was no recurrence or other postoperative complications in the remaining 7 patients. Conclusion:The incidence of unicentric Castleman disease is rare. Complete resection of the tumor is the main treatment for UCD patients, and the prognosis of UCD is good.

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.

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.

A rare case report of Castleman's disease and review of literature

Castleman’s disease (CD) is a rare lymphoproliferative disorder, described for the first time by Castleman, et al. in 1954 in a single case then in 1956 in a group of 13 patients with localized benign lymphadenopathy. The incidence of CD is unknown and can occur at any age, however it is mainly reported in adults in the literature with a slight feminine predominance (60%). The majority of the previously reported cases of CD in the neck were of the hyaline vascular type and the most common sign was an asymptomatic neck mass. In the present case the patient is having unicentric CD and histologically it is Mixed Type. We are reporting this case because of its rarity.

Castleman Disease: A Rare and Intriguing Malady: A Case Series

Castleman disease (CD), first described by Benjamin Castleman as angiofollicular mediastinal lymph杗ode hyperplasia, is a rare benign lymphoproliferative disorder with varied modes of presentation. Its common presentation within the mediastinum misleads the clinician and merits special attention since it is essentially a diagnosis of exclusion. We are sharing our experience with three patients, within a relatively short period of 2 years. All three presented with a mediastinal mass, however, each of them came with an entirely different clinical scenario and diagnosis. All three were successfully operated and Castleman disease [hyaline杤ascular (HV) type] was diagnosed only after the final histopathology.

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.

Enfermedad de Castleman. Reporte de caso / Castleman Disease. Case report and literature review

Resumen INTRODUCCIÓN: La enfermedad de Castleman, o hiperplasia angiofolicular de los ganglios linfáticos, es todo un reto diagnóstico y terapéutico para la mayoría de los médicos. Puede estar asociada con infecciones virales, como el herpes virus tipo 8 (HHV-8), o ser idiopática. A su vez, puede localizarse en una sola región (unicéntrica) o afectar varias (multicéntrica). Suele diagnosticarse en la cuarta década de la vida y ser un hallazgo cuando se trata de la variante unicéntrica. CASO CLÍNICO: Paciente de 19 años que acudió a consulta debido a la aparición de un nódulo en la mama derecha. En el ultrasonido mamario y axilar se encontraron fibroadenomas bilaterales y adenomegalias en el lado izquierdo, con alta vascularidad. Se catalogó como BIRADS 3. El reporte histopatológico de la biopsia, con aguja de corte, del ganglio axilar izquierdo fue de: proliferación linfoide atípica. La inmunohistoquímica reportó positividad para: CD20, CD3, CD21 en células dendríticas interfoliculares, Ki-67 y negatividad para HHV-8 en centros germinales residuales. CONCLUSIÓN: La extirpación quirúrgica de una masa unicéntrica de tipo hialino-vascular-plasmático es curativa. La evaluación de pacientes con sospecha de esta enfermedad debe incluir, además de la evaluación patológica con inmunotinción, estudios de laboratorio y de imágenes sistémicas con PET-TAC para determinar la extensión de la enfermedad (unicéntrica o multicéntrica) y para los marcadores de seguimiento.

Abstract BACKGROUND: Castleman's disease, or angiofollicular lymph node hyperplasia, is a diagnostic and therapeutic challenge for most physicians. It may be associated with viral infections, such as herpes virus type 8, or be idiopathic. In turn, it can be localized in a single region (unicentric) or affect several (multicentric). It is usually diagnosed in the fourth decade of life and is a finding when it is the unicentric variant. CLINICAL CASE: 19 year old patient who came to consult due to the appearance of a nodule in the right breast. Breast and axillary ultrasound showed bilateral fibroadenomas and adenomegaly on the left side, with high vascularity. It was classified as BIRADS 3. The histopathological report of the biopsy, with cutting needle, of the left axillary node was: atypical lymphoid proliferation. Immunohistochemistry reported positivity for: CD20, CD3, CD21 on interfollicular dendritic cells, Ki-67 and negativity for HHV-8 in residual germinal centers. CONCLUSION: Surgical removal of a unicentric hyaline-vascular-plasmic type mass is curative. Evaluation of patients with suspected disease should include, in addition to pathologic evaluation with immunostaining, laboratory and systemic imaging studies with PET-CT to determine the extent of disease (unicentric or multicentric) and for follow-up markers.

Adrenal Castleman disease: a case report / 中华泌尿外科杂志

Adrenal Castleman's disease is rare. One case of left adrenal Castleman's disease, who underwent retroperitoneal laparoscopic left adrenal gland and tumor resection. The postoperative pathological diagnosis was adrenal Castleman's disease (transparent vascular type), and no tumor recurrence was found after 2 years of follow-up.

Multicentric Castleman disease with high IgG4 level: report of one case and review of literature / 白血病·淋巴瘤

Objective:To investigate the diagnosis and treatment of multicentric Castleman disease (MCD).Methods:The diagnosis and treatment of one MCD patient admitted in Hangzhou First People's Hospital in July 2020 was analyzed and related literatures were reviewed.Results:The patient was a 55-year-old male with anemia, elevated globulin levels and IgG4 > 10 g/L, and enlarged lymph nodes. He was undiagnosed for 7 years. Lymph node biopsy revealed a large number of polyclonal plasma cell hyperplasia, and the ratio of IgG4/IgG was less than 0.40; the serum interleukin (IL)-6 was more than 6 000 pg/ml and then he was eventually diagnosed as MCD (plasma cell type). Rituximab + cyclophosphamide + dexamethasone (RCD) regimen was not effective, and it was changed to anti-IL-6 receptor antibody tocilizumab for 2 courses and then the patient obtained good results.Conclusions:Castleman disease is a rare disease with a poor prognosis. It has high heterogeneity and is easy to be misdiagnosed clinically. The diagnosis requires pathological examination. IL-6 is considered to be closely related to the onset of Castleman disease and has become an effective target for treatment.

Clinicopathological features and possible pathogenesis of Castleman disease / 中国肿瘤临床

Castleman disease (CD) refers to a group of uncommon lymph node proliferative disorders that have highly clinically heterogeneous and obvious pathological features. The etiology and pathogenesis of CD remain unclear. The pathogenesis of unicentric CD (UCD) is most likely driven by clonal proliferation and acquired mutations of tumor stromal cells. Idiopathic multicentric CD (iMCD) intersects with a variety of diseases in terms of its clinical manifestation and pathological features including autoimmune factors, paraneoplastic syndrome, viral infectious factors and inflammatory factor disorders. The pathogenesis of iMCD is probably the common pathway of immune dysregulation and cytokine increase caused by the combined action of multiple etiologies. Here, we aim to summarize the latest reported etiology and pathogenesis of CD, aiming to deepen the understanding of this disease.

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.

Clinical analysis of 30 cases of Castleman disease with different types of thoracic involvement / 中华血液学杂志

Objective@#To improve the clinical understanding of Castleman disease (CD) with different types of thoracic involvement, including their clinical features, radiological and pathological findings, diagnosis and current treatment strategies.@*Methods@#Retrospective analysis of 30 patients diagnosed with CD with thoracic involvement and hospitalized between June 2009 and May 2019 in The First Affiliated Hospital of Guangzhou Medical University was performed. Patients were divided into three groups for subsequent analysis based on the clinical data: CD with bronchiolitis obliterans (BO) , unicentric Castleman disease (UCD) without BO, and multicentric Castleman disease (MCD) without BO.@*Results@#Among the 30 patients, there were 5 (16.7%) patients diagnosed with BO, 18 (60.0%) patients had UCD without BO and 7 (23.3%) patients had MCD without BO. The average age of MCD without BO patients was significantly older than that of BO and UCD without BO patients[ (49.29±5.39) ys vs (27.20±3.76) ys and (37.17±2.87) ys; P=0.005 and 0.034, respectively) ]. Pulmonary symptoms were commonly seen in BO group (100%) and MCD without BO group (71.4%) . while no pulmonary symptoms were seen in UCD without BO group. Key abnormal laboratory findings were erythrocyte sedimentation rate (ESR) increase (40%in BO group and 57.1% in MCD without BO group) and hypoxia (60% in BO group and 28.6% in MCD without BO group) . Other abnormal laboratory findings seen in MCD without BO group included anemia and IgG increase (both 57.1%) . Notably, all patients in BO group had extremely severe mixed ventilation dysfunction in the lung function test. CT scan showed lung parenchyma involvement in BO group (100%) , in UCD without BO group (11.1%) featured by solitary pulmonary nodule and in MCD without BO group (57.1%) featured by diffuse lesions in bilateral lungs. The size of lymph nodes was significantly smaller in MCD without BO group comparing to that in BO group and UCD without BO group[short diameter (1.83±0.51) cm vs (4.73±1.63) cm and (3.62±0.26) cm; P=0.006 and 0.011, respectively]. All patients (100%) in the BO group had a pathological type of transparent vascular variant while the same pathological type accounts for 88.9% in UCD without BO patients. The predominantly pathological type (57.1%) was plasma cell variant in the MCD without BO group. Oral ulcers presented in all patients in BO group but were relieved after the mass resection and immunomodulatory therapy, but the pulmonary symptoms were still progressively aggravated. Thoracoscopic mass excision was the main treatment for UCD without BO patients while chemotherapy, immunomodulatory and targeted therapy were commonly used for MCD without BO treatment.@*Conclusion@#The age, clinical symptom, laboratory finding, lung function, imaging manifestation, pathology, treatment and prognosis were different among the three groups. This classification could improve clinical understanding of the disease.

Síndrome Poems asociado a enfermedad de Castleman. Un reporte de caso / Castleman disease variant of Poems Syndrome. A case report

POEMS (Polineuropatía, Organomegalia, Endocrinopatía, Proteína Monoclonal y cambios en la piel) es un trastorno multisistémico raro. La enfermedad de Castleman es un trastorno linfoproliferativo atípico de causa desconocida, que puede estar asociado a POEMS. Paciente mujer de 37 años de edad con cuadro clínico de 2 años de evolución caracterizado por múltiples adenopatías, adormecimiento, y debilidad de extremidades inferiores asociado a hiperpigmentación cutánea e hipertricosis. La electromiografía muestra una polineuropatía crónica activa, sensitiva-motora de tipo axonal; y la electroforesis sérica mostró banda monoclonal de Inmunoglobulina A tipo lambda. La biopsia de ganglio submandibular fue compatible con Enfermedad de Castleman. Este reporte resalta el hecho que la enfermedad de Castleman se presenta en forma frecuente como parte de una variante del síndrome POEMS, y debe ser descartado en estos pacientes. Asimismo, la plasmaféresis puede ser útil en pacientes con síntomas neurológicos severos.

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein and skin changes) is a rare multisystemic disorder. Castleman's disease is an atypical lymphoproliferative disorder of unknown cause, which may be associated with POEMS.A 37-year-old female patient with a clinical picture of 2 years of clinical evolution due to multiple adenopathies, numbness, and weakness of the lower extremities associated with cutaneous hyperpigmentation and hypertrichosis. Electromyography shows an active, sensory-motor chronic polyneuropathy of the axonal type; and serum electrophoresis showed monoclonal band of lambda type Immunoglobulin A. Submandibular ganglion biopsy was compatible with Castleman's disease.This report highlights the fact that Castleman's disease occurs frequently as part of a variant of the POEMS syndrome, and should be ruled out in these patients. Also, plasmapheresis can be useful in patients with severe neurological symptoms.

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.

Enfermedad de Castleman en pediatría. Presentación de un caso y revisión de la literatura / Castleman Disease in Pediatrics. Case Presentation and Bibliography Review

Resumen La enfermedad de Castleman es un raro padecimiento con igual frecuencia en ambos sexos, que puede aparecer a cualquier edad. Se caracteriza por crecimiento de tumores benignos del tejido linfático, localizados con mayor frecuencia en mediastino, estómago y cuello y son menos frecuentes en axila, pelvis y páncreas. Histológicamente se describen 3 variedades: la hialinovascular, la plasmocelular y la mixta. Se presenta el caso de un paciente de 13 años de edad, con antecedentes de microesferocitosis congénita e infección por citomegalovirus, con ingresos frecuentes por síndrome febril prolongado, el último ocurrió a los 11 años donde apareció aumento de tamaño de las cadenas ganglionares a predominio de ganglios intraabdominales. Se realizó estudio histológico que informó enfermedad de Castleman con afectación ganglionar difusa, además de hepatitis crónica reactiva. Actualmente el paciente se mantiene con seguimiento ambulatorio con control hematológico y ultrasonido abdominal, así como tratamiento con vitaminoterapia. Se presenta este reporte por la baja prevalencia de este tipo de tumor en el Hospital Pediátrico Universitario Paquito González Cueto de Cienfuegos.

ABSTRACT Castleman's disease is an uncommon condition with similar frequency in both sexes, which can appear at any age. It is characterized by the growth of benign tumors of the lymphatic tissue, located more frequently in the mediastinum, stomach and neck and are less frequent in the axilla, pelvis and pancreas. Histologically, three varieties are described: the hyaline-vascular, the plasma-cellular and the mixed. A case of a 13-year-old patient with a history of congenital micro-spherocytosis and cytomegalovirus infection is presented. He had frequent admissions due to prolonged febrile syndrome; the last one occurred at age 11, when an increase in the size of ganglion chains appeared, predominantly intra-abdominal ganglia. A histological study was performed that reported Castleman's disease with diffuse lymph node involvement, in addition to chronic reactive hepatitis. Currently the patient is maintained with outpatient follow-up with hematological control and abdominal ultrasound, as well as treatment with vitamin therapy. This report is presented due to the low prevalence of this type of tumor in University Pediatric Hospital Paquito González Cueto of Cienfuegos.

Progress of Castleman disease / 白血病·淋巴瘤

Castleman disease (CD) is a rare group of lymphoproliferative disorders with similar clinical manifestations and pathological features to malignant lymphomas. However, due to the extremely low incidence, lack of comparison among large sample clinical cases, laboratory tests, treatment options and prognostic factors, the clinical diagnosis and treatment of CD has encountered great difficulties. The 60th American Society of Hematology (ASH) Annual Meeting has made progresses of CD. In basic research aspects, it mainly explores the relationship of serum proteomics with distinct subtypes and treatment response, gene expression and cell-driven processes of diseases, sources of high cytokine production, and so on. In terms of clinical treatments, personalized treatment based on disease activity status and specific classification provides a new hope for CD patients.

CT,MRI features and misdiagnosis of hyaline vascular type localized Castleman disease / 实用放射学杂志

Objective To summarize CT and MRI features of hyaline vascular type localized Castleman disease(LCD)and analyze the causes of misdiagnosis,to improve the preoperative diagnosis rate.Methods The clinical and imaging data of 7 patients with hyaline vascular type LCD confirmed by operation and pathology were analyzed retrospectively.Results (1)6 cases were misdiagnosed before operation,1 case was misdiagnosed as pancreatic neuroendocrine tumor,1 case as thymoma,1 case as neurogenic tumor,1 case as pheochromocytoma, 1 case as clear cell renal cell carcinoma and 1 case as small mesenteric stromal tumor.(2)1 case was located in the right neck,1 case in the anterior superior mediastinum,1 case in the neck of the pancreas,1 case in the upper part of the left kidney,2 cases in the retroperitoneum and 1 case in the lower abdomen.(3)3 cases were scaned by dynamic enhanced MRI,3 cases were scaned by dynamic enhanced CT, and 1 case was checked by plain CT and enhanced MRI.CT and MRI showed that 7 cases had a round or elliptical soft tissue mass, and 4 cases with well defined margin,3 cases were not clear in edge,2 cases with spot or strip calcification on CT images,4 cases had slightly longer T1 and longer T2 signal,4 cases were restricted of diffusion and had higher signal on DWI.All the lesions were enhanced in arterial phase,and went on in the delayed phase.There were 5 cases with distorted vascular shadow in the middle and/or around of the mass, 3 cases with strips,spoke-like low-density areas or low-signal areas,and some lesions were filled in delayed phase.Conclusion CT and MRI features of hyaline vascular type LCD have certain characteristics such as rich blood supply,enhancement in persistent,tortuosity of peripheral vascular,with some short strip calcification and high signal on DWI,which may be helpful for preoperative diagnosis.