Paraneoplastic pemphigus presenting as a prodrome to aggressive T cell lymphoma.

Paraneoplastic pemphigus (PNP) is a rare disease with an unclear mechanism of pathogenesis. We present a case of a male patient who presented with wound management after being diagnosed with Castleman disease-associated paraneoplastic pemphigus (PNP). The patient's condition was not improving; as a result, extensive workup was repeated, which confirmed the diagnosis of aggressive T cell lymphoblastic lymphoma. Our case signifies the importance of keeping a high index of suspicion for PNP-associated malignancies. This case report also adds emphasis to the diagnostic challenges faced by clinicians, making clinical correlation with multidisciplinary approach essential. Therefore, if clinically indicated, we need to revisit the diagnosis and seek alternative explanations to prevent delays in management.

Unicentric Castleman's disease in the parotid gland associated with psoriasis: a case report.

BACKGROUND: Castleman's disease is a rare lymphoproliferative disorder that is often misdiagnosed because of its untypical clinical or imaging features except for a painless mass. Besides, it is also difficult to cure Castleman's disease due to its unclear pathogenesis. CASE PRESENTATION: We present a Castleman's disease case with diagnostic significance regarding a 54-year-old Chinese male who has a painless mass in his left parotid gland for 18 months with a 30-years history of autoimmune disease psoriasis. Computed tomography scan showed a high-density nodule with clear boundaries in the left parotid and multiple enlarged lymph nodes in the left submandibular and neck region. General checkup, the extremely elevated serum interleukin-6 and lymph node biopsy in the left submandibular region gave us an initial suspicion of Castleman's disease. Then the patient underwent a left superficial parotidectomy. Based on histopathologic analysis, we made a certain diagnosis of Castleman's disease and gave corresponding treatments. In 18 months of follow-up, the patient showed no evidence of recurrence, with the level of serum interleukin-6 decreased. CONCLUSIONS: Clinicians should be aware of the possibility of Castleman's disease when faced with masses or enlarged lymph nodes in the parotid gland to avoid misdiagnosis, especially in patients with autoimmune diseases and elevated serum interleukin-6.

Can We Differentiate Between Primary Sjögren Syndrome and Idiopathic Multicentric Castleman Disease Based on the Characteristics of Pulmonary Cysts?

PURPOSE: To identify radiological characteristics that could help differentiate cystic lung diseases between primary Sjögren syndrome (pSS) and idiopathic multicentric Castleman disease (iMCD). PATIENTS AND METHODS: Patients with pSS or iMCD who had cysts were enrolled. Cyst characteristics (number, size, morphology, and distribution) and other accompanying manifestations (nodules, ground-glass opacities, calcification, and thickening of the bronchovascular bundles and interlobular septa) were compared between them. RESULTS: Eleven patients with pSS and 25 patients with iMCD were eligible for our study. Eleven patients with pSS (100.0%) and 23 patients with iMCD (92.0%) had round or oval cysts. None of the patients with pSS had irregular cysts, but 21 (84.0%) patients with iMCD had irregular cysts ( P = 0.005). Smooth-walled cysts were present in 11 patients with pSS (100.0%) and 18 patients with iMCD (72.0%). Only 1 patient with pSS (9.1%) exhibited non-smooth-walled cysts, whereas 23 patients with iMCD (92.0%) had non-smooth-walled cysts ( P = 0.003). The presence of nodules was common in both groups ( P = 1.000). However, the nodules were more likely to be larger and more numerous in patients with iMCD ( P < 0.001). Cysts with mural nodules (52.2%) and central nodules (47.8%) were only observed in iMCD ( P = 0.007). CONCLUSION: Although regular and smooth-walled cysts were common in the 2 diseases, irregular and non-smooth-walled cysts were more often associated with iMCD than pSS. Nodules in iMCD tended to be larger and more numerous, and a close positional relationship between nodules and cysts was only observed in iMCD.

[Paraneoplastic pemphigus with underlying Castleman's disease in a 16-year-old girl]. / Morbus Castleman-assoziierter paraneoplastischer Pemphigus bei einer 16-jährigen Patientin.

Paraneoplastic pemphigus is a rare, life-threatening autoimmune disease that is clinically characterized by mostly extensive and refractory mucosal erosions and polymorphous skin lesions. We report here on a 16-year-old girl with isolated oral erosions, in whom mucosal pemphigoid was initially suspected and after treatment with prednisolone and dapsone marked improvement was achieved. However, a few months later the patient developed massive respiratory insufficiency as a result of bronchiolitis obliterans, so that a lung transplant was planned. As part of the preparatory diagnostic workup, unicentric, abdominally localized Castleman's disease was diagnosed, which ultimately led to the diagnosis of paraneoplastic pemphigus as evidenced by envoplakin autoantibodies. Tumor resection and subsequent lung transplantation achieved good results with sustained mucocutaneous remission.

Acquired Hemophilia: A Rare Complication of Pediatric Idiopathic Multicentric Castleman Disease.

Acquired hemophilia is caused by acquired autoantibodies to 1 of the factors of the coagulation cascade, usually factor VIII or IX, and is an exceedingly rare phenomenon in children. The finding of an acquired factor VIII inhibitor in a pediatric patient with idiopathic multicentric Castleman disease has never been reported. Patients with acquired hemophilia can have life-threatening bleeds that are refractory to blood product support, requiring bypassing agents to manage bleeding symptoms. We present the novel finding of acquired hemophilia resulting from an autoantibody to factor VIII in a pediatric patient with idiopathic multicentric Castleman disease and discuss the optimal management of bleeding in a patient with acquired hemophilia.

Kaposi sarcoma herpesvirus/human herpesvirus 8-positive diffuse large B-cell lymphoma characterized by malignant ascites: A case report.

Herein, we report a rare case of Kaposi sarcoma herpesvirus/human herpesvirus 8 (KSHV/HHV8)-positive diffuse large B-cell lymphoma (DLBCL), which is characterized by malignant ascites and complex karyotypes. A 72-year-old male patient who tested negative for human immunodeficiency virus presented with thrombocytopenia and lymphadenopathies. He was diagnosed with KSHV/HHV8-associated multicentric Castleman disease (MCD). After three years, he developed progressive lymphadenopathies and massive ascites. The lymphoma cells in the ascitic fluid presented with characteristic immunophenotype and monoclonality, which support the diagnosis of KSHV/HHV8-positive DLBCL. Lymphadenopathies and massive splenomegaly are common manifestations of KSHV/HHV8-positive DLBCL. Nevertheless, peritoneal involvement, as observed in this case, is a rare presentation. This emphasizes the diagnostic complexities of KSHV/HHV8-associated lymphoproliferative disorders. Within the context of preexisting KSHV/HHV8-associated multicentric Castleman disease, the differential diagnosis of this disorder can be challenging.

Urologic Presentation of Unicentric Pediatric Castleman Disease in the Setting of Acute Renal Colic.

An 11-year-old otherwise healthy female presented with renal colic and during computed tomography imaging evaluation, she was found to have a right distal ureteral stone with associated hydroureteronephrosis, medially deviated ureter, and 4-cm solid retroperitoneal mass. The mass was palpable on physical exam and was further categorized with magnetic resonance imaging, ultrasound, and laboratory testing. A multidisciplinary team approach, including pediatric surgery, radiology, oncology, and urology, led to the patient undergoing a right retrograde pyelogram, ureteroscopy with stent placement, and laparoscopic excision of retroperitoneal mass. Her pathology revealed lymphoid hyperplasia with histologic features of Castleman disease.

Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease.

Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. There are vastly different clinical subtypes, with some patients demonstrating thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly (TAFRO) and others having milder/more moderate symptoms with potential for severe disease (not otherwise specified, NOS). Due to its rarity and heterogeneity, the natural history and long-term burden of iMCD are poorly understood. We investigated real-world medical data from ACCELERATE, a large natural history registry of patients with Castleman disease, to better characterize the long-term disease burden experienced by these patients. We found that iMCD-TAFRO patients face a significant hospitalization burden, requiring more time in the hospital than iMCDNOS patients during the year surrounding diagnosis (median [interquartile range]: 36 [18-61] days vs. 0 [0-4] days; P<0.001). In addition, we found life-sustaining interventions, such as mechanical ventilation (17%) and dialysis (27%), were required among iMCD patients, predominantly those with iMCD-TAFRO. iMCD-NOS patients, however, spent a significantly greater proportion of time following disease onset in a state of disease flare (median 52.3% vs. 18.9%; P=0.004). Lastly, we observed severe iMCD-related morbidities, such as acute renal failure, sepsis and pneumonia, among others, arising after iMCD diagnosis, impairing the patients' quality of life. These data demonstrate a substantial disease burden experienced by iMCD patients and emphasize the importance of ongoing research into iMCD to aid disease control.

Outcomes in Kaposi's sarcoma-associated herpesvirus -associated primary effusion lymphoma and multicentric Castleman's disease in patients with human immunodeficiency virus (HIV) in a safety-net hospital system.

OBJECTIVE: To describe cases of Kaposi's sarcoma-associated herpesvirus (KSHV)-associated multicentric Castleman's disease (MCD) and primary effusion lymphoma (PEL) in patients with HIV from a large, safety-net hospital system in Dallas, Texas, USA. METHODS: We conducted a retrospective review of patients with HIV-associated PEL and/or MCD. RESULTS: Twelve patients with PEL and 10 patients with MCD were identified. All patients were male and 17 of 20 were men who have sex with men; 66.7% of PEL patients and 50% of MCD patients had concurrent KS at the time of diagnosis; 42% of patients with PEL and 20% of patients with MCD died during the follow-up period. We noted improved survival in our cohort compared to previous studies, particularly in our PEL patients with a median survival of 11.4 months compared to 3-6-month median survival historically. Median follow-up time for MCD patients was 17.5 months. This improved survival is despite suboptimal antiretroviral therapy (ART) adherence at diagnosis, with only 50% of patients on ART at the time of MCD/PEL diagnosis. CONCLUSION: These data highlight the importance of early recognition of PEL and MCD, and the larger-scale efforts needed to better understand the pathogenetic drivers of clinical outcomes in patients affected by KSHV-related diseases.

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.

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)

Linfoma de células grandes B originado en enfermedad de Castleman: reporte de un caso y revisión de la literatura / Large B-cell lymphoma coexisting with Castleman's disease: report of one case

We report a 73-year-old female patient with Castleman's disease coexistent with large B cell type non-Hodgkin's lymphoma in a right axillary lymphadenopathy. An excisional biopsy was performed: microscopically, the lymph node revealed the presence of numerous plasma cells and small lymphoid cells characteristic of Castleman's disease. An analysis of another portion of the specimen revealed lymphoid cells with large abnormal nuclei gathered locally that were CDD 79+, CD 38+ and MUM-1+ as well as positive for Kaposi sarcoma-associated herpesvirus and negative for Epstein Barr virus encoded RNA-1 (EBER).

A case of Paraneoplastic Pemphigus associated with Castleman's disease / Um caso de Penfigo Paraneoplastico (PNP) associado a doenca de Castleman

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.

Síndrome de POEMS associada à doença de Castleman: relato de caso e revisão da literatura / POEMS syndrome associated with Castleman disease: case report and literature review

JUSTIFICATIVA E OBJETIVOS: A síndrome de POEMS é uma entidade rara e pouco diagnosticada devido a sua variedade de manifestações clínicas, a maior parte delas inespecíficas. Pode estar associada à doença de Castleman em até 25% dos casos, uma entidade histopatológica também pouco comum. O diagnóstico da síndrome de POEMS é clínico, baseado em critérios definidos pela Mayo Clinic e seu tratamento ainda é um desafio. O objetivo deste estudo foi relatar um caso de síndrome de POEMS associada à doença de Castleman. RELATO DO CASO: Paciente do sexo masculino, 52 anos, foi encaminhado ao serviço com quadro de seis meses de evolução de emagrecimento, ginecomastia, hiperpigmentação de face e extremidades e parestesia distal em membros inferiores. Ao exame físico, notava-se também linfonodomegalia difusa. Exames laboratoriais mostravam eritrocitose, trombocitose, hipotireoidismo subclínico, testosterona no limite inferior da normalidade e hiperproteinorraquia. Exames de imagem para detecção de neoplasia foram todos normais. A biópsia de linfonodo supraclavicular esquerdo demonstrou doença de Castleman e a imunofixação sérica evidenciou componente monoclonal de cadeia leve, o que levou ao diagnóstico de síndrome de POEMS associada à doençade Castleman. CONCLUSÃO: A síndrome de POEMS deve ser lembrada em pacientes com quadro neurológico associado à doença monoclonal e manifestações clínicas diversas, enquanto a doença de Castleman deve fazer parte do diagnóstico diferencial de linfonodomegalia. A associação das duas entidades, apesar de rara, pode estar presente.

BACKGROUND AND OBJECTIVES: POEMS syndrome is a rare, often poorly diagnosed entity because of its variety of clinical manifestations, the majority of them being unspecific. It can be associated with Castleman disease, also a rare histopathological entity, in up to 25% of cases. The diagnosis of POEMS syndrome is a clinical one, based on Mayo Clinic criteria, and its management is still a challenge. The objective of this article was to report a case of POEMS syndrome associated with Castleman disease. CASE REPORT: Male patient, 52-year-old was referred to our service due to weight loss, gynecomastia, face and extremities hyperpigmentation and distal paresthesia of lower limbs that have been progressing for 6 months. At clinical examination, diffuse lymphadenopathy was also noted. Laboratory tests demonstrated polycythemia, thrombocytosis, subclinical hypothyroidism, testosterone at the lower limit of normal, and elevated cerebrospinal fluid protein. Images to detect tumors were all normal. Aleft supraclavicular lymph node biopsy revealed Castleman disease and serum immunoelectrophoresis demonstrated monoclonal light chain. With these findings, the diagnosis of POEMS syndrome associated with Castleman disease was established. CONCLUSION: POEMS syndrome should be considered in patients with a neurological condition associated with a monoclonal disease and many diverse clinical manifestations, while Castleman disease might be part of differential diagnosis of lymphadenomegaly. The association of the two entities, though rare,can be present.