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Int. j. med. surg. sci. (Print) ; 6(1): 14-17, mar. 2019. ilus
Article in English | LILACS | ID: biblio-1254180


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

Humans , Male , Adult , Castleman Disease/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Biopsy , Radiography, Thoracic
Autops. Case Rep ; 8(3): e2018033, July-Sept. 2018. ilus
Article in English | LILACS | ID: biblio-911974


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.

Humans , Female , Adolescent , Castleman Disease/diagnosis , Clavicle/pathology , Lymph Nodes/pathology
Rev. bras. reumatol ; 54(4): 326-329, Jul-Aug/2014. tab, graf
Article in Portuguese | LILACS | ID: lil-722288


A doença de Castleman (DC) é uma desordem linfoproliferativa policlonal, também conhecida como hiperplasia nodular gigante ou hiperplasia angiofolicular linfoide. Esta é uma doença rara que está frequentemente associada ao vírus da imunodeficiência humana (HIV) e ao herpes vírus 8 (HHV-8). Os achados histopatológicos encontrados na DC sugerem uma intensa resposta aos estímulos antigênicos observada em várias doenças associadas com ativação imune, como a artrite reumatoide. Um fator importante implicado na patogênese da DC é a produção autônoma da interleucina-6 (IL-6). Nessa doença, as manifestações clínicas estão relacionadas aos níveis de IL-6, e a remoção cirúrgica dos linfonodos acometidos ou a utilização de anticorpos anti-IL-6 fazem regredir os sintomas. Descrevemos um caso da DC multicêntrica em uma mulher jovem, não associada à infecção pelo vírus HHV-8 ou à imunossupressão. Uma breve revisão da literatura se segue à descrição do caso clínico.

Castleman's disease (CD) is a polyclonal lymphoproliferative disorder also known as giant nodular hyperplasia or angiofollicular lymph node hyperplasia. It is a rare disease often associated to human immunodeficiency virus (HIV) and human herpes virus 8 (HHV-8). Histopathological findings in Castleman's disease suggest an exaggerated response to antigenic stimuli seen in other diseases associated with immune activation, such as rheumatoid arthritis. An important aspect of its pathogenesis is the autonomous production of interleukin-6 (IL-6). In this disease, the clinical manifestations are associated to IL-6 serum levels, and surgical removal of the compromised lymph nodes or use of anti-IL-6 antibodies can slow down the symptoms. We describe a multicentric Castleman's disease in a young woman not associated to HHV-8 virus infection or immunosuppression. A short review of the literature follows the description of this clinical case.

Humans , Female , Adult , Castleman Disease/diagnosis , HIV-1 , Herpesvirus 8, Human
Rev. cuba. hematol. inmunol. hemoter ; 30(2): 184-189, abr.-jun. 2014.
Article in Spanish | LILACS | ID: lil-714395


La enfermedad de Castleman es una entidad poco frecuente, caracterizada por hiperplasia linfoide angiofolicular. Tiene dos tipos histológicos principales: el hialino vascular y el de células plasmáticas; y dos formas clínicas: la localizada y la multicéntrica. Se presenta el caso de una paciente femenina de 19 años de edad con diagnóstico de enfermedad de Castleman tipo hialino vascular, con tres adenopatías localizadas en la región cervical y antecedentes de cuadros infecciosos a repetición previos al diagnóstico. En el estudio inmunohistoquímico se observó CD 20, CD 3 patrón normal y Ki 67 positivo en los folículos linfoides. El estudio inmunológico de sangre periférica arrojó una disminución marcada de los linfocitos CD3+, fundamentalmente a expensas de los CD4+, con conservación de la inmunidad humoral y presencia de anticuerpos antinucleares. Se detectaron anticuerpos IgG anticitomegalovirus y antivirus de Epstein Barr y no se detectaron anticuerpos anti-VIH1,2. Las manifestaciones clínicas sugestivas de inmunodeficiencia antes del diagnóstico de la enfermedad de Casttleman y los resultados de laboratorio obtenidos, permiten sugerir la influencia de la desregulación del sistema inmune en la patogenia de la enfermedad proliferativa. El estado de inmunodeficiencia, además, favorece las infecciones por gérmenes oportunistas, que a su vez potencian los desórdenes inmunes y linfoproliferativos. Se obtuvo una respuesta clínica favorable con tratamiento inmunoestimulante

Castleman'sdisease is a rare disorder characterized by angiofollicularlymphoid hyperplasia. There are two main histological patterns of the disease: the hyaline vascular type and the plasma cell type; andtwo clinical forms: localized and multicentric. We report a case of a 19-year-old female patient with hyaline vascular type Castleman's disease with cervical lymphadenopathies and medical history of repetitive infectious diseases before diagnosis. The inmunohistochemical study reported CD 20, normal pattern of CD3 and Ki 67 positive only in lymphoidfollicles. The immunological study showed low levels of CD3+ CD4+ lymphocytes, slightly low levels of CD3+ CD8+ lymphocytes, and the presence of antinuclear antibodies (ANA). The humoral immunity was not affected.Other findings were IgG antibodies against CMV and EBV positive, and VIH 1,2 antibodies negative. Clinical history suggesting Immunodeficiency and laboratory findings support the hypothesis that deregulation of the immune system could lead to the pathogenesis of the proliferative disease. Also, the immunodeficiency state promotes the onset of opportunistic infections which may increase immune and proliferative disorders. A favorable response to immunostimulant treatment was observed

Humans , Female , Adolescent , Adjuvants, Immunologic/therapeutic use , Castleman Disease/diagnosis , Castleman Disease/immunology , Case Reports
An. bras. dermatol ; 88(6,supl.1): 11-14, Nov-Dec/2013. graf
Article in English | LILACS | ID: lil-696785


We present a case of PNP associated with Castleman's Disease. We have also reviewed the literature and described the characteristics of the two associated diseases. Gene clonal rearrangement was done to help diagnosis. We used, in addition, stereotactic radiosurgery which, as far as we know, has never before been employed to treat PNP associated with Castleman's Disease. This produced a good response, suggesting that it might be a good alternative treatment for PNP associated with Castleman's Disease when it is too difficult to operate.

Apresentamos um caso de PNP associada à doença de Castleman.Também revisamos a literatura, e referenciamos as características das duas doenças associadas. Um rearranjo genético clonal foi feito para ajudar o diagnóstico. Além disso, usamos a radiocirurgia que até então nunca havia sido utilizada para tratar PNP associada à doença de Castleman. Esta produziu uma boa resposta, sugerindo que pode ser uma boa alternativa para o tratamento de PNP associada com a doença de Castleman quando é muito difícil fazer uma cirugia convencional.

Humans , Male , Middle Aged , Castleman Disease/complications , Paraneoplastic Syndromes/complications , Pemphigus/complications , Biopsy , Castleman Disease/diagnosis , Polymerase Chain Reaction , Paraneoplastic Syndromes/diagnosis , Pemphigus/diagnosis , Treatment Outcome
Arch. argent. pediatr ; 111(4): e89-e93, ago. 2013. ilus, tab
Article in Spanish | LILACS | ID: lil-694654


La enfermedad de Castleman es una rara entidad, caracterizada por la hiperplasia de los folículos linfoides. Excepcionalmente afecta a los niños. Presentamos el caso de una niña de 9 años con enfermedad de Castleman en la región parotídea. A pesar de su baja incidencia en la población pediátrica, esta afección puede simular una neoplasia y debe ser tenida en cuenta entre los diagnósticos diferenciales de una masa cervical.

Castleman's disease (CD) is a rare entity, characterized by lymph node follicles hyperplasia. It rarely occurs in children. We present a case of a 9 year old girl with CD in the parotid region. This disease, although it's low incidence in pediatric population, may mimic a malignant neoplasm and should be a differential diagnosis in cervical masses.

Child , Female , Humans , Castleman Disease , Castleman Disease/diagnosis , Castleman Disease/surgery , Neck
Rev. cuba. hematol. inmunol. hemoter ; 28(1): 77-83, ene.-mar. 2012.
Article in Spanish | LILACS | ID: lil-628581


La enfermedad de Castleman es un proceso linfoproliferativo poco frecuente que se caracteriza por hiperplasia benigna de los ganglios linfáticos. Existen 2 variedades histológicas bien diferenciadas: la hialino-vascular y la plasmo-celular, que a su vez, pueden ser localizadas o multicéntricas. La forma hialino-vascular suele ser asintomática y localizada con mayor frecuencia en mediastino, mientras que la plasmo-celular se presenta frecuentemente con sintomatología sistémica y suele ser difusa o multicéntrica. Por lo poco frecuente de la asociación, presentamos un caso con antecedentes de anemia drepanocítica que después de 12 años de diagnóstico de una enfermedad de Castleman localizada, variante de células plasmáticas y exéresis total, hizo una recaída ganglionar con la misma variante, con buena respuesta clínica al tratamiento

Castleman's disease is a rare lymphoproliferative process characterized by benign hyperplasia of the lymph nodes. There are 2 distinct histologic variants: hyaline-vascular and cellular plasmodia, which in turn, may be localized or multicentric. The hyaline vascular form is usually asymptomatic and most often located in the mediastinum, whereas cellular plasmodia frequently presents with systemic symptoms and it is usually diffuse or multicentric. Due to the rarity of this association, we present a case with a history of sickle cell disease 12 years after diagnosis of localized Castleman's disease, plasma cell variant and total excision, lymph node had a relapse with the same variant, having good clinical treatment response

Humans , Male , Adult , Castleman Disease/diagnosis , Castleman Disease/pathology , Castleman Disease , Sickle Cell Trait/complications , Case Reports , Prednisone/therapeutic use
Int. j. morphol ; 30(1): 170-175, mar. 2012. ilus
Article in Spanish | LILACS | ID: lil-638780


Pseudolinfoma cutáneo es el término utilizado para indicar un grupo de trastornos linfoproliferativos benignos de la piel, siendo el linfocitoma cutis una forma de seudolinfoma de células B. Clínicamente se manifiesta como nódulos o placas solitarias, generalmente localizados en la cara de mujeres jóvenes. Su etiopatogenia estaría relacionada con una respuesta inmunológica local exagerada ante diversos desencadenantes tales como, picadura de insectos, acupuntura, vacunas, tatuajes, medicamentos e infecciones. Se presentan dos casos de pacientes con diagnóstico de linfocitoma cutis realizado en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez de Temuco.

Cutaneous pseudolymphoma is the term used to indicate a group of benign lymphoproliferative disorders of the skin, being the cutaneous lymphomas a form of B-cell pseudolymphoma. Clinically it manifests as solitary nodules or plaques, usually located on the face of young women. Its pathogenesis could be related to an exaggerated local immune response to various causes such as insect bites, acupuncture, vaccinations, tattoos, drugs and infections. We present two cases of patients diagnosed with skin linfocitoma made in the Pathology Unit in Hernán Henríquez Hospital of Temuco.

Aged , Pseudolymphoma/diagnosis , Pseudolymphoma/therapy , Skin Diseases/diagnosis , Skin Diseases/therapy , Castleman Disease/diagnosis , Castleman Disease/therapy
Article in English | WPRIM | ID: wpr-169427


Castleman's disease is an uncommon disorder characterized by benign proliferation of the lymphoid tissue that occurs most commonly in the mediastinum. Although unusual locations and manifestations have been reported, involvement of the renal parenchyma and sinus, and moreover, manifestations as cardiac tamponade are extremely rare. Here, we present a rare case of Castleman's disease in the renal parenchyma and sinus that also accompanied cardiac tamponade.

Cardiac Tamponade/diagnosis , Diagnosis, Differential , Diagnostic Imaging , Castleman Disease/diagnosis , Humans , Kidney Diseases/diagnosis , Male , Middle Aged , Nephrectomy
Article in English | WPRIM | ID: wpr-187904


This report presents the case of a patient demonstrating multicentric Castleman's disease (MCD) with a lung lesion that was successfully treated with an anti-interleukin-6 receptor antibody, tocilizumab in combination with corticosteroid and tacrolimus. A 43-yr-old female with abnormal shadows on a chest X-ray was referred to the hospital for further examination. She was diagnosed as having MCD based on the characteristic pathology of inguinal lymph node, lung lesions, laboratory data, and undifferentiated arthritis. Corticosteroid and rituximab therapy did not fully ameliorate the symptoms; thus, the therapeutic regimen was changed to include tocilizumab, oral corticosteroid and tacrolimus. This regimen resulted in clinical remission and the dose of tocilizumab and corticosteroid could be tapered. Tocilizumab in combination with corticosteroid and tacrolimus may therefore be a beneficial treatment regimen for lung lesions associated with MCD.

Adrenal Cortex Hormones/therapeutic use , Adult , Antibodies, Monoclonal/therapeutic use , Drug Therapy, Combination , Female , Castleman Disease/diagnosis , Humans , Immunosuppressive Agents/therapeutic use , Lung Diseases, Interstitial/drug therapy , Lymph Nodes/pathology , Receptors, Interleukin-6/antagonists & inhibitors , Tacrolimus/therapeutic use , Tomography, X-Ray Computed
Cir. & cir ; 77(3): 187-192, mayo-jun. 2009. tab, ilus
Article in Spanish | LILACS | ID: lil-566501


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.

Humans , Male , Female , Adolescent , Adult , Middle Aged , Castleman Disease/diagnosis , Castleman Disease/drug therapy , Castleman Disease/pathology , Mexico , Retrospective Studies , Young Adult
West Indian med. j ; 58(1): 61-64, Jan. 2009. ilus
Article in English | LILACS | ID: lil-672437


A 51-year old male patient with a three-month history of constant and dull left flank pain was investigated by ultrasonography, computed tomography (CT) scan and magnetic resonance imaging (MRI) of the abdomen, which disclosed a 8 x 7 x 6 cm retroperitoneal pararenal mass with heterogeneous imaging characteristics and bright enhancement following intravenous contrast injection. Based on the hypervascularity of the mass and the lack of specific signs in the imaging investigation, lymphoma, sarcoma or vascular tumour were considered as probable diagnoses and the patient underwent an exploratory laparotomy. The histologic examination of the surgically resected specimen disclosed "a hyaline type of Castleman's disease". Further evaluation of the patient with antibody testing for HIV 1 and 2, as well as viral load by PCR for Herpes Virus-8 (HHV-8) were negative. Bone marrow aspiration, biopsy and immunophenotypic study did not disclose any evidence of lymphoma. Molecular study of the bone marrow for immunoglobulin heavy chain rearrangement showed a polyclonal pattern; serum protein electrophoresis did not show any evidence of hypergamma-globulinaemia and serum immunofixation electrophoresis did not show any monoclonal protein. A diagnosis of localized - unicentric type of Castleman's disease was made. Castleman's Disease should be included in the differential diagnosis of any solitary, heterogeneous and hypervascular retroperitoneal mass. Discovery of Castleman's disease at any area of the body should be followed by a thorough imaging and laboratory work-up in order to exclude the multicentric type of the disease and the co-existence of lymphoma.

Un paciente varón de 51 años con una historia de tres meses de dolor constante y sordo en el costado izquierdo, fue sometido a investigación mediante ultrasonografía, tomografía axial computarizada (IAC) e imagen por resonancia magnética (IRM) del abdomen. La investigación reveló una masa retro-peritoneal pararenal de 8 x 7 x 6 cm, con imagen de características heterogéneas y aumento de la luminosidad tras la inyección intravenosa de contraste. Sobre la base de hipervascularidad de la masa y la falta de signos específicos en la investigación por imágenes, el linfoma, el sarcoma o el tumor vascular fueron considerados como diagnósticos probables y el paciente fue sometido a una laparotomía exploratoria. El examen histológico del espécimen resecado quirúrgicamente reveló "un tipo hialino de la enfermedad de Castleman." La evaluación ulterior del paciente con prueba de anticuerpos de VIH 1 y 2, así como la carga viral por PCR para la detección del virus herpes humano tipo 8, dio resultados negativos. La aspiración de médula ósea, la biopsia y el estudio inmunofenotípico no mostraron ninguna evidencia de linfoma. El estudio molecular de la médula ósea para el reordenamiento de la cadena pesada de inmunoglobulina mostró un patrón policlonal. La electro-foresis de la proteína en suero no mostró evidencia alguna de hipergammaglobulinemia y la electroforesis de inmunofijación sérica no mostró proteína monoclonal alguna. Se hizo un diagnóstico de tipo unicéntrico y localizado de la enfermedad de Castleman. La Enfermedad de Castleman debe incluirse en el diagnóstico diferencial de cualquier masa retroperitoneal solitaria, heterogénea e hipervascular. El descubrimiento de la enfermedad de Castleman en cualquier área del cuerpo debe ser seguido de un examen por imágenes completo y pruebas exhaustivas de laboratorio, a fin de eliminar la posibilidad de un tipo multicéntrico de la enfermedad y la co-existencia de un linfoma.

Humans , Male , Middle Aged , Castleman Disease/diagnosis , Retroperitoneal Neoplasms/diagnosis , Contrast Media , Diagnosis, Differential , Diagnostic Imaging , Castleman Disease/pathology , Castleman Disease/surgery , Retroperitoneal Neoplasms/pathology , Retroperitoneal Neoplasms/surgery
Article in Portuguese | LILACS | ID: lil-566993


A Doença de Castleman é uma desordem linfoproliferativa que apresenta relação etiológica com o herpes vírus tipo 8 e o HIV, manifestando-se de duas formas distintas. A forma unicêntrica é benigna, não cursa com sintomas sistêmicos e geralmente é curada com a ressecção cirúrgica. A forma multicêntrica geralmente apresenta-se com linfadenopatia generalizada, febre, emagrecimento, entre outras manifestações sistêmicas e pode estar associada a malignidades, entre as quais a síndrome de POEMS (polineuropatia, organomegalia, endocrinopatia, gamopatia monoclonal e alterações de pele). Relatamos o caso de um homem de 50 anos que se apresentava com emagrecimento, febre e linfadenopatia, associado a gamopatia monoclonal, organomegalia, endocrinopatia e neuropatia, cujo diagnóstico foi de Doença de Castleman cursando com síndrome de POEMS.

Castleman’s disease is a lymphoproliferative disorder related with human herpesvirus 8 and HIV, presenting in two different ways. The unicentric form is benign, doesn’t have systemic symptoms and is usually cured with surgery. The multicentric form usually presents with generalized lymphadenopathy, fever, weight loss, and other systemic manifestations and it can be associated with malignancy, especially POEMS syndrome. We report a case of a 50 year old man presenting with weight loss, fever and lymphadenopathy, associated with monoclonal gammopathy, organomegaly, endocrinopathy and neuropathy, which diagnosis was Castleman’s disease with POEMS syndrome.

Humans , Male , Middle Aged , Castleman Disease/diagnosis , POEMS Syndrome/diagnosis , POEMS Syndrome/mortality , Diagnosis, Differential , Castleman Disease/etiology
LJM-Libyan Journal of Medicine. 2008; 3 (2): 101-103
in English | IMEMR | ID: emr-146607


Castleman's Disease [CD] is a rare lymphoproliferative disorder accompanied by marked systemic inflammatory response. Morphological diagnosis of CD requires biopsy of the whole of the involved lymph node tissue. Three histologic variants have already been described in CD morphology [hyaline vascular, plasma-cell, and mixed]. In this study, we report a case of a multicentric Castleman's disease of the plasma cell variant type with negative Herpes Virus 8. The clinical presentation of this patient was of systemic amyloidosis as a result of both a delayed diagnosis and medical management. Previously described cases of CD with secondary amyloidosis have been of the localized type. Regardless, long-standing clinical remission of CD by cytotoxic drugs and anti-CD20 antibody therapy was achieved, but the nephrotic syndrome remained irreversible

Lymphoproliferative Disorders/etiology , Lymphatic Diseases , Diagnosis, Differential , Castleman Disease/diagnosis , Amyloidosis/diagnosis , Nephrotic Syndrome , Delayed Diagnosis
Rev. chil. cir ; 59(6): 463-466, dic. 2007. ilus
Article in Spanish | LILACS | ID: lil-482842


El 70 por ciento de los casos de la Enfermedad de Castleman (EC) se presentan como masas ganglionares que afectan el mediastino. El compromiso del tubo digestivo de la EC es muy raro. Se presenta un caso excepcional de EC ubicado en el espesor de la pared rectal que fue sometida a una resección anterior baja con la sospecha clínica de una endometriosis. El estudio histopatológico demostró una EC del tipo hialino-vascular y la paciente está asintomática luego de 2 años de seguimiento. Se reconoce una variedad hialino-vascular de la EC, que es generalmente asintomática y que se presenta como lesiones solitarias. La variedad de células plasmáticas de la EC, en cambio, tiende a ser multicéntrica, con compromiso sistémico y en el 18 por ciento de los casos puede ocurrir la transformación a un linfoma maligno o un sarcoma de Kaposi. El diagnóstico preoperatorio de la EC localizada en el abdomen o en la pelvis es muy difícil. Como el estudio por imágenes no permite descartar un tumor maligno, habitualmente se requiere una exploración quirúrgica con el fin de realizar una resección completa o, al menos, una biopsia incisional diagnóstica. Las lesiones solitarias que son resecadas en forma completa tienen un pronóstico excelente y no hay casos de recidiva publicados. La variedad multicéntrica de la EC, en cambio, tiene un pronóstico reservado y requiere de un manejo multidisciplinario.

Catleman disease usually presents as mediastinal lymph nodes and gastrointestinal involvement is exceptional. We report a 49 years old female with a vascularized mass located in the sigmoid colon. The mass was excised and the pathological study showed a Castleman disease, hyaline vascular variety. The patient is free of symptoms after two years of follow up. This variety of Castleman disease usually presents as solitary asymptomatic masses and if it is completely excised, the prognosis is good.

Humans , Female , Middle Aged , Castleman Disease/surgery , Castleman Disease/diagnosis , Rectal Neoplasms/surgery , Rectal Neoplasms/diagnosis , Treatment Outcome , Lymphoproliferative Disorders/surgery , Lymphoproliferative Disorders/diagnosis