ADA2 Deficiency Mimicking Idiopathic Multicentric Castleman Disease
نویسندگان
چکیده
منابع مشابه
Multicentric Carpotarsal Osteolysis Mimicking Juvenile Idiopathic Arthritis
Background Multicentric carpotarsal osteolysis (MCTO), a skeletal dysplasia presents in early childhood mimicking juvenile idiopathic arthritis (JIA). Recognition of this syndrome is essential to avoid unnecessary treatment with immunosuppressive agents because of different course and treatment. Case Report A 3-year-old boy presented with swelling and restriction of right wrist joint and left ...
متن کاملChronic Myelomonocytic Leukemia following Multicentric Castleman Disease
Multicentric Castleman disease (MCD) is a rare nonmalignant lymphoproliferative disorder presenting systemic symptoms such as fever, night sweats, fatigue, anemia, effusions, and multifocal lymphadenopathy. The etiology of MCD has not been clarified to date. The coexistence of MCD with chronic myelomonocytic leukemia (CMML) has been rarely reported. Although the pathogenesis remains unclear, th...
متن کاملSiltuximab for Multicentric Castleman Disease-Letter.
We read with great interest the article by Deisseroth and colleagues (1) focusing on the recent global approval of the monoclonal IL6 antibody siltuximab for the treatment of patients with HHV-8-, HIV-negative multicentric Castleman disease (MCD). Up to now, treatment options for this rare lymphoproliferative disorder are very limited. Lately, the phase III, double-blind, randomized trial of va...
متن کاملMulticentric Castleman disease: consensus at last?
In collaboration with an international cohort of clinicians and pathologists who reviewed more than 240 cases, the authors concurred on a set of evidence-based criteria, thereby paving the way for a uniform approach to managing this enigmatic condition. In 1956, Castleman et al described clinicopathologic features of 13 asymptomatic patients who presented with a mediastinal mass. This form of u...
متن کاملInternational, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Human herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases of MCD and can occur in individuals...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Pediatrics
سال: 2018
ISSN: 0031-4005,1098-4275
DOI: 10.1542/peds.2017-2266