Localised Hyaline Vascular Type of Castleman's Disease Mimicking Adult-Onset Still's Disease

Department of Pediatrics, Chang Gung Children's Hospital, Kweishan, Taoyuan, Taiwan.
Clinical Rheumatology (Impact Factor: 1.7). 02/1999; 18(6):485-7. DOI: 10.1007/s100670050143
Source: PubMed


A previously healthy 18-year-old boy presented with daily spiking fever, polyarthritis, and evanescent skin rashes, as well as hepatomegaly and Raynaud's phenomena for 2 months. He was initially diagnosed with adult-onset Still's disease (AOSD). During the period of follow-up, intermittent fever and migratory polyarthritis persisted and an insidiously growing mass over the right axillary region was noted 1 year after the diagnosis of AOSD. Excisional biopsy of the mass revealed a group of lymph nodes with histological features of the hyaline vascular type of Castleman's disease. The patient's symptoms disappeared soon after excision of the lymph nodes. evanescent rash, lymphadenopathy, hepatosplenomegaly and serositis [5]. A clinical picture compatible with the diagnosis of AOSD has not been described in the localised hyaline vascular type of Castleman's disease. We report such a case in an 18-year-old male patient who presented prolonged fever and polyarthritis with an initial diagnosis of AOSD. The diagnosis of hyaline vascular type of Castleman's disease was made 1 year later, when the patient developed an insidiously growing mass over the right axilla.

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    • "Ces différents symptômes étant aspécifiques et la MCM rare et parfois peu connue, le délai diagnostique peut être long. Ces différents symptômes peuvent mimer une maladie auto-inflammatoire telle que la maladie de Still et la MCM en est un diagnostic différentiel connu [4] [5]. De plus, il a été récemment décrit au Japon une nouvelle entité proche de la MCM appelée le syndrome TAFRO (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly) [6] avec, à notre connaissance, un seul cas caucasien rapporté fin 2014 [7]. "
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    ABSTRACT: Introduction: Multicentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia. Case report: We report a 32-year-old woman with an association of oligoanuria, anasarca, thrombotic microangiopathy with features compatible with adult-onset Still disease. The outcome was initially favorable with corticosteroids, immunoglobulins and plasmapheresis but with the persistence of relapses marked by severe autonomic syndrome and necessity of high dose corticosteroids. The diagnosis of mixed type Castleman's disease, HHV8 and HIV negative, was obtained four years after the onset of symptoms by a lymph node biopsy. The outcome was favorable after tocilizumab and corticosteroids but tocilizumab had to be switched to anakinra to ensure a proper and long-lasting control of the disease. Conclusion: Our patient partially fits the description of TAFRO syndrome (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly), a MCM rare variant, recently described in Japanese patients.
    No preview · Article · Sep 2015 · La Revue de Médecine Interne
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    • "The diagnosis of CD in these cases is surprising clinically. On the other hand, due to the histopathological similarities CD may mimic some collagen vascular diseases or in some cases, autoimmune disorders and pathological findings of some reactive lymphoid hyperplasias may mimic CD [15] [16] [17] [18]. So CD may cause some difficulties in some situations in rheumatology clinics. "
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    ABSTRACT: Castleman disease (CD) is an uncommon lymphoproliferative disorder with a unique histopathology. Generally diagnosis is not difficult for an experienced pathologist. However, not usually, some histopathological entities or tissue reactions may mimic CD. Here CD-like reaction due to toxic substance ingestion has been presented.
    Full-text · Article · Jun 2009 · European Journal of Internal Medicine
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    ABSTRACT: Castleman's disease (angiofollicular lymphoid hyperplasia) is a rare lymphoproliferative disorder that can be found in both nodal and extranodal sites. It is classified histologically as the more common hyaline vascular variant, a plasma cell variant, or a mixed form. The hyaline vascular variant is typically characterized by a benign clinical course with no constitutional symptoms other than localized pressure from the mass. We report an atypical case of the hyaline vascular variant with constitutional symptoms that have been clinically associated with the aggressive plasma cell variant. Diagnosis was confirmed histopathologically after surgical resection, which was curative and resulted in resolution of all symptoms. This case broadens our understanding of Castleman's disease as a part of the spectrum of lymphoproliferative and inflammatory diseases. Furthermore, it supports recent studies suggesting that systemic pathogenicity is related to associated cytokine dysregulation and highlights the need to include Castleman's disease in the differential diagnosis of pediatric lymphoproliferative disorders.
    No preview · Article · Mar 2001 · Southern Medical Journal
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