scholarly journals Unicentric Castleman disease

2021 ◽  
Author(s):  
Khawaja Waheed
Keyword(s):  
2019 ◽  
Vol 3 (1) ◽  
pp. 97-105
Author(s):  
Mary Zuccato ◽  
Dustin Shilling ◽  
David C. Fajgenbaum

Abstract There are ∼7000 rare diseases affecting 30 000 000 individuals in the U.S.A. 95% of these rare diseases do not have a single Food and Drug Administration-approved therapy. Relatively, limited progress has been made to develop new or repurpose existing therapies for these disorders, in part because traditional funding models are not as effective when applied to rare diseases. Due to the suboptimal research infrastructure and treatment options for Castleman disease, the Castleman Disease Collaborative Network (CDCN), founded in 2012, spearheaded a novel strategy for advancing biomedical research, the ‘Collaborative Network Approach’. At its heart, the Collaborative Network Approach leverages and integrates the entire community of stakeholders — patients, physicians and researchers — to identify and prioritize high-impact research questions. It then recruits the most qualified researchers to conduct these studies. In parallel, patients are empowered to fight back by supporting research through fundraising and providing their biospecimens and clinical data. This approach democratizes research, allowing the entire community to identify the most clinically relevant and pressing questions; any idea can be translated into a study rather than limiting research to the ideas proposed by researchers in grant applications. Preliminary results from the CDCN and other organizations that have followed its Collaborative Network Approach suggest that this model is generalizable across rare diseases.


2020 ◽  
Vol 99 (2) ◽  
pp. 32-42
Author(s):  
V.I. Burlakov ◽  
◽  
A.L. Kozlova ◽  
I.N. Abramova ◽  
Yu.A. Rodina ◽  
...  

2021 ◽  
pp. 632-638
Author(s):  
Yu Ming Jin ◽  
Gui Ying Jing

Castleman disease (CD) is a rare chronic lymphoproliferative disease with unknown etiology and pathogenesis disease. When the lesion is located in the mediastinum, the diagnosis of CD is easy. However, if the lesion presents as a perigastric mass mimicking other subserosal gastric mesenchymal tumors, the diagnosis can be challenging. As few sonographic manifestations of hyaline-vascular variant CD, especially contrast-enhanced ultrasound (CEUS) imaging, as well as computed tomography (CT) and histopathological imaging, have been reported in literature, this case may provide a vivid example of a comprehensive CEUS and CT usage in the diagnosis and surgery with regard to CD. This report presents a case of a 50-year-old female diagnosed with hyaline-vascular variant CD in a random physical examination, the ultrasound examination first revealed a 24.3 mm × 15.4 mm hypoechogenic lesion abutting the stomach, esophagus, and liver, which was under the suspicion of gastrointestinal stromal tumor. Following a series of medical examinations, including CEUS, CT, postoperative histopathological examination, and immunohistochemical analysis, the patient was diagnosed with hyaline-vascular variant unicentric CD. After the mass was completely excised through laparoscopic surgery, the woman recovered very well without recurrence during a follow-up period of 15 months. Thus, mastering ultrasound and CT-imaging characteristics of CD and applying ultrasound and CT examination together would do help to preoperative diagnosis.


2020 ◽  
Vol 7 (11) ◽  
Author(s):  
Annabelle Pourbaix ◽  
Romain Guery ◽  
Julie Bruneau ◽  
Estelle Blanc ◽  
Gregory Jouvion ◽  
...  

Abstract We report a case of chronic hepatosplenic aspergillosis following immune reconstitution complicating colic aspergillosis in an AIDS patient with multicentric Castleman disease. Symptoms mimicked the clinical presentation of chronic disseminated candidiasis and responded to corticosteroid. This emerging entity enlarges the spectrum of fungal immune reconstitution inflammatory syndrome in the HIV setting.


Author(s):  
Yoshito Nishimura ◽  
David C. Fajgenbaum ◽  
Sheila K. Pierson ◽  
Noriko Iwaki ◽  
Asami Nishikori ◽  
...  

2021 ◽  
Vol 32 (3) ◽  
pp. 286-289
Author(s):  
I-Fan Lin ◽  
Jiun-Nong Lin ◽  
Tsung-Heng Tsai ◽  
Chao-Tien Hsu ◽  
Yu-Ying Wu ◽  
...  

Coexistence of multicentric Castleman disease and Kaposi sarcoma is rare and might be missed without an experienced pathologists’ interpretation. A 46-year-old man had been diagnosed with HIV infection and treated with combination antiretroviral therapy of dolutegravir/abacavir/lamivudine (Triumeq) for one year. The latest viral load was 49 copies/mL and CD4 T-cell count was 192 cells/uL. He was admitted due to fever off and on, splenomegaly, general lymphadenopathy, and severe thrombocytopenia for two months. Biopsy of a purplish skin lesion and gastric tissue showed Kaposi sarcoma. The pathology of inguinal lymph nodes revealed coexistence of Kaposi sarcoma and multicentric Castleman disease. The plasma Kaposi sarcoma herpesvirus viral load was 365,000 copies/mL. During hospitalization, progressive pancytopenia and spiking fever persisted, and he died of multi-organ failure before completion of chemotherapeutic treatments with rituximab plus liposomal doxorubicin.


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