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| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{DiseaseDisorder infobox | |
| | Name = Castleman's Disease | |
| | ICD10 = {{ICD10|D|36|0|}} | |
| | UMLS = C0017531 - C2931179 | |
| | MeshID = D005871 | |
| | MedDRA = 10050251 | |
| | SNOMED CT = 207036003 - 238809002 |
| | }} |
| | {{Castleman's disease}} |
| {{CMG}}; {{AE}} {{RT}} | | {{CMG}}; {{AE}} {{RT}} |
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| {{SK}} Angiofollicular lymph node hyperplasia; lymphoid hamartoma | | {{SK}} Angiofollicular lymph node hyperplasia; lymphoid hamartoma; angiofollicular ganglionic hyperplasia |
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| ==Overview== | | ==[[Castleman's disease overview|Overview]]== |
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| '''Castleman's disease''' is a rare disorder characterized by non-cancerous growths (tumors) that may develop in the lymph node tissue throughout the body. It involves hyperproliferation of certain [[B cell]]s that often produce cytokines.
| | ==[[Castleman's disease historical perspective|Historical Perspective]]== |
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| ==Historical Perspective== | | ==[[Castleman's disease classification|Classification]]== |
| In 1954 Dr. Benjamin Castleman, a pathologist described an unusual histopathology of a lymph node in a patient with mediastinal mass.
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| ==Classification== | | ==[[Castleman's disease pathophysiology|Pathophysiology]]== |
| 2 types of classifications exist for Castleman's disease. They are:
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| * Clinical and Radiologic classification:
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| ** Unicentric - single lymph node is involved, most commonly in the mediastinum or the mesentery.
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| ** Multicentric - wide spread involvement of lymph nodes and also liver and spleen in some cases
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| * Histopathologic classification:
| | ==[[Castleman's disease causes|Causes]]== |
| ** Hyaline vascular
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| ** Plasmacytic
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| ** Mixed cellularity - has features of both hyaline vascular type and plasmacytic type.
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| | ==[[Castleman's disease differential diagnosis|Differentiating Castleman's disease from other Diseases]]== |
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| | ==[[Castleman's disease epidemiology and demographics|Epidemiology and Demographics]]== |
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| There are several variants of Castleman's disease. About 50% of '''Multicentric Castleman's disease''' (MCD) is caused by ''[[Kaposi's sarcoma-associated herpesvirus]]'' (KSHV), a gammaherpesvirus that is also the cause of [[Kaposi's sarcoma]] and [[primary effusion lymphoma]], while the remainder of MCD are of unknown cause. The form of MCD most closely associated with KSHV is the plasmacytic form of Castleman's disease while another pathologic form, the hyaline-vascular form, is generally negative for this virus. In all cases, Castleman's disease is likely due to hypersecretion of the cytokine [[Interleukin 6|IL-6]]. In KSHV positive tumors, this is most likely due to expression of the a virus-encoded cytokine, vIL-6, while KSHV negative tumors appear to be the result of over secretion of human IL-6.
| | ==[[Castleman's disease risk factors|Risk Factors]]== |
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| ==Symptoms== | | ==[[Castleman's disease natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| The most common 'B Symptoms' of MCD are high [[fevers]], [[anemia]], [[weight loss]], [[loss of appetite]], and [[low white blood cell counts]], which may to be due to the overproduction of [[interleukin 6]]. Symptomatically, therefore, MCD can be difficult to diagnose and even in the case of a lymph-node biopsy a conclusive diagnosis remains problematic.
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| ==Treatment== | | ==Diagnosis== |
| ===Unicentric===
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| In the Unicentric form of the disease, surgical [[Segmental resection|resection]] is often curative,<ref> name=pmid21934347 </ref> <ref name="pmid18419976">{{cite journal |author=Talarico F, Negri L, Iusco D, Corazza GG |title=Unicentric Castleman's disease in peripancreatic tissue: case report and review of the literature |journal=G Chir |volume=29 |issue=4 |pages=141–4 |year=2008 |month=April |pmid=18419976 |doi= |url=http://www.giornalechirurgia.it/index.php?PAGE=article&ID=2770}}</ref> and the prognosis is excellent.
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| ===Multicentric===
| | [[Castleman's disease history and symptoms|History and Symptoms ]] | [[ Castleman's disease physical examination|Physical Examination]] | [[Castleman's disease laboratory findings|Laboratory Findings]] | [[ Castleman's disease chest x ray|Chest X Ray]] | [[Castleman's disease CT|CT]] | [[Castleman's disease MRI|MRI]] | [[Castleman's disease ultrasound|Ultrasound]] | [[Castleman's disease other imaging findings|Other Imaging Findings]] | [[Castleman's disease other diagnostic studies|Other Diagnostic Studies]] |
| There is no standard therapy for MCD at the moment.
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| It is important to distinguish AIDS-related Multicentric Castleman’s disease from other forms of Multicentric Castleman’s disease. Treatment for the former can be focused upon the same protocols used for treating the underlying AIDS.<ref name="pmid14760135">{{cite journal |author=Sprinz E, Jeffman M, Liedke P, Putten A, Schwartsmann G |title=Successful treatment of AIDS-related Castleman's disease following the administration of highly active antiretroviral therapy (HAART) |journal=Ann. Oncol. |volume=15 |issue=2 |pages=356–8 |year=2004 |month=February |pmid=14760135 |doi= 10.1093/annonc/mdh066|url=http://annonc.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=14760135}}</ref>
| | ==Treatment== |
| | | [[Castleman's disease medical therapy|Medical Therapy]] | [[Castleman's disease surgery |Surgery]] | [[Castleman's disease primary prevention|Primary Prevention]] | [[Castleman's disease secondary prevention|Secondary Prevention]] | [[Castleman's disease cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Castleman's disease future or investigational therapies|Future or Investigational Therapies]] |
| Prior to 1996 MCD carried a poor prognosis of about 2 years, due to [[autoimmune hemolytic anemia]] and [[non-Hodgkin's lymphoma]] which may arise as a result of proliferation of infected cells. The timing of diagnosis, with particular attention to the difficulty of determining the cause of B symptoms without a CT scan and lymph node biopsy, may impact significantly on the prognosis and risk of death. Left untreated, MCD usually gets worse and becomes increasingly difficult and unresponsive to current treatment regimens.
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| Recent work with HIV-positive patients with KSHV-related MCD suggests that treatment with the antiherpesvirus drug [[ganciclovir]] or the antiCD20 B cell [[monoclonal antibody]], [[rituximab]], may markedly improve outcome. These drugs target and kill B cells via the B cell specific CD20 marker. Since B cells are required for the production of antibodies, the body's immune response is weakened whilst on treatment and the risk of further viral or bacterial infection is increased. Due to the uncommon nature of the condition there are not many large scale research studies from which standardized approaches to therapy may be drawn, and the extant case studies of individuals or small cohorts should be read with caution. As with many diseases, the patient's age, physical state and previous medical history with respect to infections may impact on the disease progression and outcome.
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| Use of [[tocilizumab]] has been proposed.<ref name="pmid17541233">{{cite journal |author=Matsuyama M, Suzuki T, Tsuboi H, ''et al.'' |title=Anti-interleukin-6 receptor antibody (tocilizumab) treatment of multicentric Castleman's disease |journal=Intern. Med. |volume=46 |issue=11 |pages=771–4 |year=2007 |pmid=17541233 |doi= 10.2169/internalmedicine.46.6262|url=http://joi.jlc.jst.go.jp/JST.JSTAGE/internalmedicine/46.6262?from=PubMed |format= – <sup>[http://scholar.google.co.uk/scholar?hl=en&lr=&q=intitle%3AAnti-interleukin-6+receptor+antibody+%28tocilizumab%29+treatment+of+multicentric+Castleman%27s+disease&as_publication=Intern.+Med.&as_ylo=2007&as_yhi=2007&btnG=Search Scholar search]</sup>}} {{dead link|date=April 2009}}</ref>
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| [[Siltuximab]], a monoclonal antibody that binds interleukin-6, is currently being investigated in a Phase II clinical trial.<ref>[http://clinicaltrials.gov/ct2/show/NCT01400503 A Study to Evaluate the Safety of Long-term Treatment With Siltuximab in Patients With Multicentric Castleman's Disease] ''ClinalTrails.gov''.</ref>
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| Other treatments for multicentric castleman disease include the following:
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| *[[Corticosteroids]]
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| *[[Chemotherapy]]
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| *[[Thalidomide]]<ref>http://www.mayoclinic.com/health/castleman-disease/DS01000/DSECTION=treatments-and-drugs</ref>
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| ==References==
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| {{reflist|2}}
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| * Aoki Y, Yarchoan R, Wyvill K, Okamoto S, Little RF, Tosato G. Detection of viral interleukin-6 in Kaposi sarcoma-associated herpesvirus-linked disorders. Blood 2001;97(7):2173-6.
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| * Yarchoan R, Little RF. Immunosuppression-related malignancies. In: DeVita Jr. VT, Hellman S, Rosenberg SA, eds. Cancer, Principles and Practice of Oncology 6th Edition. Philadelphia: Lippincott Williams and Wilkins; 2001:2575-97.
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| | ==Case Studies== |
| | [[Castleman's disease case study one|Case #1]] |
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| [[Category:Disease]] | | [[Category:Disease]] |