ABSTRACT
A doença de Castleman é um distúrbio linfoproliferativo raro. Há três tipos histológicos: hialino-vascular (mais comum), variante de células plasmáticas e forma mista. A forma hialino-vascular é caracterizada tipicamente por apresentar uma evolução clínica benigna e localizada, sem sintomas constitucionais. É geralmente tratada com cirurgia e/ou radioterapia. A doença multicêntrica apresenta sintomas sistêmicos. Ainda não há um consenso sobre qual a melhor abordagem terapêutica. Reportamos o caso da doença em um homem de 47 anos com diagnóstico de doença de Castleman variante hialino-vascular e anemia hemolítica autoimune associada, com presença de CD-20, CD-10, CD3 e Ki67 positivos. Foi tratado com protocolo quimioterápico esquema CHOP e corticoterapia com prednisona, evoluindo com melhora do quadro.
Castleman's disease is a rare lymphoproliferative disorder. There are three histological types: hyaline-vascular (most common), plasma cell variant, and mixed form. The hyaline-vascular form is typically characterized by a benign and localized clinical course without constitutional symptoms. It is usually treated with surgery and/or radiotherapy. The multicentric disease has systemic symptoms. There is still no consensus on the best therapy approach. We report a case of the disease in a 47-year-old man diagnosed with hyaline-vascular variant of Castleman's disease, and associated Autoimmune Hemolytic Anemia, with the presence of CD20-positive, CD10-positive, CD3-positive and Ki67-positive cells. He was treated with chemotherapy protocol of CHOP regimen and corticotherapy with Prednisone, and evolved with improvement.
Subject(s)
Humans , Male , Middle Aged , Anemia, Hemolytic, Autoimmune/diagnosis , Castleman Disease/drug therapy , Diagnosis, Differential , Immunohistochemistry , Lymphoproliferative DisordersABSTRACT
Introducción: La enfermedad de Castleman es una entidad patológica poco comprendida, descrita originalmente en pacientes europeos. Informamos nuestra experiencia con esta entidad clinicopatológica en pacientes del Instituto Nacional de Cancerología de la Ciudad de México. Material y métodos: Analizamos retrospectivamente los expedientes de pacientes con enfermedad de Castleman de 1996 a 2003. La enfermedad fue monocéntrica si había solo un ganglio o multicéntrica si se encontraba linfoadenopatía generalizada. Además, se dividió en las variantes histológicas hialinovascular y de células plasmáticas. Resultados: Once pacientes con enfermedad de Castleman fueron diagnosticados en el periodo referido, seis tenían enfermedad monocéntrica y cinco multicéntrica. La mediana de seguimiento fue de 40 meses. Todos los pacientes con enfermedad monocéntrica tenían la variante hialinovascular. De los cinco con multicéntrica, cuatro tenían la variante de células plasmáticas y uno la hialinovascular. Cinco pacientes con enfermedad monocéntrica se trataron con cirugía y uno con quimioterapia; al momento de este informe todos permanecían vivos y sin enfermedad. Tres pacientes con enfermedad multicéntrica recibieron quimioterapia y dos, quimioterapia más radioterapia por enfermedad residual; a dos pacientes se les prescribió quimioterapia de segunda línea, con buena respuesta. Dos pacientes con una condición asociada evolucionaron desfavorablemente. Conclusiones: Las características clínicas, patológicas y los resultados del tratamiento son similares a los señalados en otras poblaciones.
BACKGROUND: Castleman's disease (CD) is a rare, poorly understood pathological entity. We report our experience with this clinicopathological entity. METHODS: We retrospectively analyzed records of all patients with CD from 1996 to 2003. The disease was classified as unicentric if a solitary mass was present or multicentric if generalized lymphadenopathy was present. We further subdivided the disease into hyaline vascular (HV) and plasma cell (PC) histological variants. RESULTS: We found 11 patients with CD. Six patients had unicentric disease and five had multicentric disease. Median follow-up was 40 months. All patients with unicentric disease had the HV variant. Of the five patients with multicentric disease, four had the PC variant and one had the HV. Five patients with unicentric disease were treated surgically with complete resection, and only one patient was treated with chemotherapy. All remain alive without disease. Three patients with multicentric disease were treated with chemotherapy, and two patients received chemotherapy plus radiotherapy for residual disease. Two patients received second-line chemotherapy with a favorable outcome. Two patients with a comorbid condition had a poor outcome. CONCLUSIONS: Clinical characteristics, pathological features and treatment results are similar to that reported in other populations.
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Castleman Disease/diagnosis , Castleman Disease/drug therapy , Castleman Disease/pathology , Mexico , Retrospective Studies , Young AdultSubject(s)
Humans , Male , Female , Castleman Disease/drug therapy , Castleman Disease/mortality , Magnetic Resonance ImagingABSTRACT
Castleman's disease is an atypical lymphoproliferative disorder having two types of presentation--the localized and the multicentric form. The localized form presents as a slowly growing mass with a relatively benign course. Multicentric Castleman's disease has a more aggressive clinical course with diffuse lymph node enlargement and systemic illness. It is rarely seen in childhood and only thirteen cases have been reported in literature. This is the first report of 2 cases from the Indian subcontinent with a maximum follow-up of 44 months one of whom had asplenia.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy, Needle , Child , Follow-Up Studies , Castleman Disease/drug therapy , Humans , Immunohistochemistry , Male , Prednisolone/therapeutic use , Risk AssessmentABSTRACT
La enfermedad de Castleman (hamartoma linfoideo, linfoma, gigante benigno, hiperplasia angiofolicular de los nódulos linfáticos) es un transtorno linfoproliferativo raro, de curso usualmente benigno de etiología desconocida y pobremente entendido. Resulta de un crecimiento no regulado del tejido linfático y puede manifestarse clínicamente en 2 formas, localizada y diseminada o multicéntrica, con 3 variantes histopatológicas, vascular hialino, plasmocelular y mixto o transicional. Usualmente la forma vascular hialina tiene una evolución clínica benigna manifestada solamente por adenopatías en el cambio, la forma plasmocelular o mixta puede manifestar alteraciones físicas y de laboratorio, tales como fiebre, pérdida de peso, anemia e hiperglobulinemia. Las 2 últimas condiciones clínicas pueden resultar en transformación maligna similar al linfoma de Hodgkin. Revisamos la base de datos del Servicio de Patología de nuestro hospital y encontramos 14 casos reportados desde enero de 1990 hasta enero del 2002, 2 mostraron presentación inusual. Descriptores: Enfermedad de Castleman, trastorno linfoproliferativo, comportamiento clínico.
Subject(s)
Male , Adult , Humans , Female , Adolescent , Middle Aged , Castleman Disease/surgery , Castleman Disease/diagnosis , Castleman Disease/physiopathology , Castleman Disease/drug therapy , Castleman Disease/therapy , Costa RicaABSTRACT
A 66 years female, who was since last year under astenia, arthralgias, pimply lesions in spread plates and tests showing eritrosedimentation over 100 mm, anemi, leucocitosis with neutrofilia, policlonal hypergammaglobulinemia, slight proteinuria and IgE on 900. This patient was sporadically treated with corticoids. When made the medical consult had lost 34lb., was under anorexy, as well as dyspepsia. Hemoglobyn 6.9 gr/dl, leucocytes 20000/mm3, neutrofils at 90%, proteinogram the same as former, with hypoalbuminemia. She was taking prednisona, 16 mg/day. When examined showed depress of conscience, astenia, and dermic lesions already quoted. 4 cm nonpainful right axillary adenopaty adhered to deep planes. Medulogram with increased iron, hyperegenerative. Ganglionar biopsia: linfoid hyperplasic process linked to inmune response. Toracoabdominal tomography with adenomegalia in torax and retroperitoneo. Skin biopsia: neutrofilic vasculitis. The patient suspends the 16 mg of prednisona and fever as well as generalized adenopatias come up. After laying aside other ethiologies, and understanding as Castleman Multicentric disease, it is started to supply prednisona 1 mg/kg of weight with a clinical and biochemical fast and outstanding response. After 7 months it was progressively suspended the esteroids and 60 days later, the process fall back; for that, corticoids are restarted, with a good evolution. The illness of Castleman although it is not very frequent, it should be considered as differential diagnosis in those clinical cases that are accompanied with important general commitment, linphadenopaties and respons to steroid therapy.
Mujer de 66 años que un año previo a la consulta presentaba astenia, artralgias, lesiones pruriginosas y eritematosas en placa diseminadas. Eritrosedimentación mayor de 100mm, anemia, leucocitosis con neutrofilia, hipergammaglobulinemia policlonal, proteinuria leve e IgE de 900. Fue tratada esporádicamente con corticoides. Llega a la consulta con pérdida de 15kg de peso, anorexia y dispepsia. Hemoglobina 6.9gr/dl, leucocitos 20000/mm3, neutrófilos 90%, proteinograma similar al previo mas hipoalbuminemia. Recibía prednisona 16mg;día. Al examen bradipsíquica, asténica, lesiones dérmicas ya descritas, adenopatía axilar derecha de 4cm no dolorosa adherida a planos profundos. Medulograma con hierro aumentado, hiperregenerativa. Biopsia ganglionar: proceso hiperplásico linfoide relacionado a respuesta inmune. Tomografía tóracoabdominal con adenomegalias en medias tino y retroperitoneo. Biopsia de piel: vasculitis neutrofílica. Suspende el corticoide y aparece fiebre y adenopatías generalizadas. Tras descartar otras etiologías, se interpreta como Enfermedad de Castleman. Inicia prednisona a lmg/kg/ día con favorable, rápida y llamativa respuesta clínica y bioquímica. Luego de 7 meses se suspende de manera progresiva los esteroides, y a los 60 días presenta recaída por lo que se reinicia la terapéutica con una nueva favorable evolución. La enfermedad de Castleman si bien es poco frecuente, debe ser considerada como diagnóstico diferencial en aquellos cuadros clínicos que se acompañan de importante compromiso general. adenomegalias y respuesta a terapia con corticoides.
Subject(s)
Aged , Female , Humans , Castleman Disease/pathology , Skin/pathology , Antineoplastic Agents, Hormonal/therapeutic use , Biopsy , Diagnosis, Differential , Castleman Disease/drug therapy , Prednisolone/therapeutic useABSTRACT
La enfermedad de Castleman debe ser considerada en el diagnóstico diferencial de masas en el cuello. Describimos las características clínicas en la variante hialino-vascular y en la variante de células plasmáticas. El caso reportado corresponde a una mujer adolescente con una masa en el cuello. (