Multicentric plasmocytic Castleman's disease with polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome and coexistent human herpes virus-6 infection – a possible relationship

Fazakas, Ádám; Csire, Márta; Berencsi, György; Szepesi, Ágota; Matolcsy, András; Jakab, L; Karádi, István; Várkonyi, Judit

doi:10.1080/10428190903162743

Cited by 10 publications

(7 citation statements)

References 17 publications

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“…In Sjögren's syndrome, a number of findings from literature have implicated the presence of a B-cell hyperactivity that may evolve to a lymphoproliferative disorder. 18,19 Our patient had a previous underlying autoimmune disorder in the form of Sjögren's and this autoimmune response may be the trigger of Castleman disease. 19 The first reported case of MCD diagnosed in Ghana and successfully treated also had plasma cell variant and presented with two major and four minor criteria (constitutional symptoms, anaemia, thrombocytopenia and elevated ESR).…”

Section: Discussionmentioning

confidence: 94%

“…The manifestations of CD are nonspecific and require histological diagnosis. Sjögren's syndrome is a systemic autoimmune disease that mainly affects the exocrine glands with lymphocytic infiltration of the glands particularly the salivary and lacrimal glands leading to the development of xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes) with eventual damage or destruction of the glands 18 Sjögren's syndrome primarily affects predominantly Caucasian perimenopausal women, with an incidence of 4-5 cases per 100 000. 17 The syndrome can occur by itself (primary) or as secondary Sjögren's syndrome when it is associated with another connective tissue disease.…”

Section: Discussionmentioning

confidence: 99%

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Sjögren's and plasma cell variant Castleman disease: a case report

Dei‐Adomakoh

Quarcoopome

Abrahams

et al. 2018

Ghana Medical Journal

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Castleman disease is a rare cause of lymphoid hyperplasia and may result in localized symptoms or an aggressive, multisystem disorder. It can mimic other diseases like lymphoma or tuberculosis. It classically presents as a mediastinal mass that involves the lymphatic tissue primarily but can also affect extra lymphatic sites including the lungs, larynx, parotid glands, pancreas, meninges, and muscles. In HIV and HHV8-negative patients with idiopathic multi-centric Castleman disease, pathogenesis may involve autoimmune mechanisms. We highlight and report a case of a 34-year-old Ghanaian female who was successfully diagnosed and managed for Sjögren's as well as plasma cell variant Castleman disease with combination chemotherapy and rituximab followed by eighteen months maintenance therapy with pulse chlorambucil and prednisolone and three monthly rituximab.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Sjögren's and plasma cell variant Castleman disease: a case report

Dei‐Adomakoh

Quarcoopome

Abrahams

et al. 2018

Ghana Medical Journal

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“…The pathophysiology involves antidesmoglein 3 antibodies with an intraepidermal intercellular pattern identical to pemphigus vulgaris. Although concomitant HHV-8 infection is classically reported, more recently an involvement of human herpesvirus 6 in certain cases has been suggested (23). Removal of the Castleman disease mass, if possible, is often more effective than systemic immunosuppressant therapy (7).…”

Section: Diseases Associated With Castleman Diseasementioning

confidence: 99%

Castleman Disease: The Great Mimic

Bonekamp¹,

Horton

Hruban³

et al. 2011

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Castleman disease is a nonclonal lymphoproliferative disorder and one of the more common causes of nonneoplastic lymphadenopathy. Because of its diverse manifestations and ability to affect any body region, Castleman disease is a great mimic of both benign and malignant abnormalities in the neck, chest, abdomen, and pelvis. Castleman disease most commonly manifests as unicentric disease (unicentric Castleman disease) with a hyperenhancing lymph nodal mass and should be considered in the differential diagnosis of lymphoma, metastatic adenopathy, and infectious and/or inflammatory diseases that result in adenopathy. Castleman disease includes a spectrum of pathologic variants, including the classic hyaline vascular type, the less common plasma cell variant of Castleman disease, and the more recently described multicentric Castleman disease and Castleman disease associated with human herpesvirus 8. Castleman disease has been associated with the human immunodeficiency virus, lymphoma, POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome, paraneoplastic pemphigus, and plasma cell dyscrasias. Aggressive forms of Castleman disease with systemic manifestations may occur. Unicentric hyaline vascular Castleman disease is often curable with surgery; however, multicentric Castleman disease may require steroid treatment, chemotherapy, antiviral medication, or the use of antiproliferative regimens because a surgical procedure cannot be curative in this setting. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.316115502/-/DC1.

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“…In a 2009 study, Fazakas et al . supposed a possible etiological relationship between a coexistent HHV-6 infection and multicentric plasmocytic Castleman’s disease with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) [18]. …”

Section: Discussionmentioning

confidence: 99%

Localized retroperitoneal Castleman’s disease: a case report and review of the literature

et al. 2014

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IntroductionCastleman’s disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which play a major role in determining therapy. We focus here on the unicentric type, which can be treated and cured by surgery. To date, approximately 1000 cases of Castleman’s disease have been reported in the literature.Case presentationA 50-year-old Caucasian woman presented to our Department of Hematology and Internal Oncology with increasing fatigue as her sole symptom. Diagnostic investigations including laboratory studies, ultrasound, computed tomography and magnetic resonance imaging were performed. These revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected. We conducted a laparotomy with radical extirpation of the tumor mass (10×9×5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures. Histological and immunohistochemical analysis confirmed the diagnosis of Castleman’s disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype. Staining for cyclin D1 and CD30 was negative. Expression of CD15 was positive in the enlarged sinusoidal vessels. A supplementary clonality analysis was without pathological findings. Tests for human immunodeficiency virus and human herpes virus 8 were negative and results from a bone marrow biopsy were normal. Our patient recovered well from surgery and was discharged from our hospital. To date, no recurrence of the disease has been detected.ConclusionCastleman’s disease is a rare disorder that remains a diagnostic challenge. Radical surgical resection is considered to be the gold standard for treating the unicentric variant of this disease.

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Multicentric plasmocytic Castleman's disease with polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome and coexistent human herpes virus-6 infection – a possible relationship

Cited by 10 publications

References 17 publications

Sjögren's and plasma cell variant Castleman disease: a case report

Sjögren's and plasma cell variant Castleman disease: a case report

Castleman Disease: The Great Mimic

Localized retroperitoneal Castleman’s disease: a case report and review of the literature

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