|
|
| (18 intermediate revisions by one other user not shown) |
| Line 1: |
Line 1: |
| __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}} |
|
| |
|
| {{SK}} Angiofollicular lymph node hyperplasia; lymphoid hamartoma | | {{SK}} Angiofollicular lymph node hyperplasia; lymphoid hamartoma; angiofollicular ganglionic hyperplasia |
|
| |
|
| ==Overview== | | ==[[Castleman's disease overview|Overview]]== |
|
| |
|
| '''Castleman's disease''' is a rare disorder characterized by non-cancerous growths ([[tumor]]s) 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]]== |
|
| |
|
| ==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.
| |
|
| |
|
| ==Classification== | | ==[[Castleman's disease pathophysiology|Pathophysiology]]== |
| 2 types of classifications exist for Castleman's disease. They are:
| |
| * Clinical and Radiologic classification:
| |
| ** Unicentric - single lymph node is involved, most commonly in the mediastinum or the mesentery.
| |
| ** Multicentric - wide spread involvement of lymph nodes and also liver and spleen in some cases
| |
|
| |
|
| * Histopathologic classification:
| | ==[[Castleman's disease causes|Causes]]== |
| ** Hyaline vascular
| |
| ** Plasmacytic
| |
| ** Mixed cellularity - has features of both hyaline vascular type and plasmacytic type.
| |
|
| |
|
| ==Pathophysiology== | | ==[[Castleman's disease differential diagnosis|Differentiating Castleman's disease from other Diseases]]== |
| 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.
| |
|
| |
|
| ===Associated Conditions=== | | ==[[Castleman's disease epidemiology and demographics|Epidemiology and Demographics]]== |
| Castleman's disease is sometimes associated with: | |
|
| |
|
| * [[POEMS syndrome]]
| | ==[[Castleman's disease risk factors|Risk Factors]]== |
| * [[Paraneoplastic pemphigus]]
| |
| * [[Thrombotic thrombocytopenic purpura]] ([[TTP]])
| |
|
| |
|
| ==Causes== | | ==[[Castleman's disease natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| * 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.
| |
| | |
| ==Epidemiology and Demographics==
| |
| ===Age===
| |
| * Average age of the patients with unicentric disease is 30 - 40 yrs
| |
| * For multicentric disease it is 50 - 60 yrs
| |
| | |
| ===Gender===
| |
| * No gender differentiation is seen in the occurrence of the disease.
| |
| | |
| ==Risk Factors==
| |
| * [[HIV]]/[[AIDS]] can act as a risk factor for this disease. The course of the disease is worse in these patients.
| |
| | |
| ==Natural History, Complications and Prognosis==
| |
| ===Complications===
| |
| * Life threatening infections
| |
| * Multi organ failure
| |
| * [[Kaposi sarcoma]]
| |
| * [[Lymphoma]]
| |
| * [[Death]]
| |
| | |
| ===Prognosis=== | |
| * 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.
| |
| * [[HIV]] patients with multicentric disease have a grave prognosis. They have a less favorable clinical course and tend to develop [[Kaposi sarcoma]] and even plasmablastic [[non-Hodgkin lymphoma]].
| |
|
| |
|
| ==Diagnosis== | | ==Diagnosis== |
| ===Symptoms===
| |
| The most common 'B Symptoms' of MCD are
| |
| * High [[fevers]]
| |
| * [[Anemia]]
| |
| * [[Weight loss]]
| |
| * [[Loss of appetite]]
| |
|
| |
| ===Laboratory Findings===
| |
| * Complete blood count and differential count - [[low white blood cell counts]], which may to be due to the overproduction of [[interleukin 6]].
| |
| * [[Erythrocyte sedimentation rate]] - elevated in 80% cases.
| |
|
| |
| ===Imaging===
| |
| ====CT====
| |
| * To detect the enlarged lymph nodes in the chest, neck, abdomen, groin.
| |
| * Enlargement of liver and spleen can also be detected.
| |
|
| |
|
| [[Image:Castleman_disease_CT.jpg|thumb|200 px|center|(A)Castleman's disease Preoperative CT scan of the abdomen showing a highly vascularized retroperitoneal tumor measuring 10 × 9.2 cm with intratumor calcifications. Right ureter dilatation (grade II), (coronal multiplanar reformation, MPR) (B) The same tumor in axial orientation. No other tumor localization in the abdomen nor enlarged lymph nodes were detected.]]{{clr}} | | [[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]] |
|
| |
|
| ==Treatment== | | ==Treatment== |
| ===Unicentric===
| | [[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]] |
| 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.
| |
| | |
| ===Multicentric===
| |
| There is no standard therapy for MCD at the moment.
| |
| | |
| 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>
| |
| | |
| 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.
| |
| | |
| 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>
| |
| | |
| [[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>
| |
| | |
| Other treatments for multicentric castleman disease include the following:
| |
| *[[Corticosteroids]]
| |
| *[[Chemotherapy]]
| |
| *[[Thalidomide]]<ref>http://www.mayoclinic.com/health/castleman-disease/DS01000/DSECTION=treatments-and-drugs</ref>
| |
| | |
| ==References==
| |
| {{reflist|2}}
| |
| * 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.
| |
| * 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.
| |
|
| |
|
| | ==Case Studies== |
| | [[Castleman's disease case study one|Case #1]] |
|
| |
|
| [[Category:Disease]] | | [[Category:Disease]] |