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| ==Overview== | | ==[[Malignant histiocytosis overview|Overview]]== |
| '''Malignant histiocytosis''' is a hereditary disease found in the Bernese Mountain Dog characterized by [[histiocyte|histiocytic]] infiltration of the lungs and [[lymph node]]s. The [[liver]], [[spleen]], and [[central nervous system]] can also be affected. Histiocytes are a component of the [[immune system]] that proliferate abnormally in this disease. In addition to its importance in veterinary medicine, the condition is also important in human pathology.<ref>http://dermnetnz.org/dermal-infiltrative/malignant-histiocytoses.html</ref>
| | ==[[Malignant histiocytosis pathophysiology |Pathophysiology]]== |
| | | ==[[Malignant histiocytosis causes|Causes]]== |
| ==Histiocytic disorders== | | ==[[Malignant histiocytosis differential diagnosis|Differentiating Malignant histiocytosis from other Diseases]]== |
| A histiocyte is a differentiated tissue cell that has its origin in the [[bone marrow]]. The source for histiocytes is the [[monocyte]]/[[macrophage]] line. Monocytes (found in the blood) and macrophages (found in tissue) are responsible for [[phagocytosis]] (ingestion) of foreign material in the body. [[Langerhans cell]]s are [[dendritic cell]]s found in the skin and function by internalizing [[antigen]]s (foreign particles) and presenting them to [[T cell]]s. They arise from monocytes.<ref>{{cite journal |author=Ginhoux F, Tacke F, Angeli V, Bogunovic M, Loubeau M, Dai XM, Stanley ER, Randolph GJ, Merad M |title=Langerhans cells arise from monocytes in vivo |journal=Nat. Immunol. |volume=7 |issue=3 |pages=265-73 |year=2006 |pmid=16444257}}</ref> Histiocytic disorders refer to diseases that are caused by abnormal behavior of these cells. They include the following:
| | ==[[Malignant histiocytosis epidemiology and demographics|Epidemiology and Demographics]]== |
| *Reactive diseases of Langerhans cells
| | ==[[Malignant histiocytosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| **[[Histiocytoma (dog)|Histiocytoma]]s
| | ==Diagnosis== |
| **Cutaneous [[histiocytosis]]
| | [[Malignant histiocytosis history and symptoms|History and Symptoms]] | [[Malignant histiocytosis physical examination|Physical Examination]] | [[Malignant histiocytosis laboratory findings|Laboratory Findings]] | [[Malignant histiocytosis CT|CT]] | [[Malignant histiocytosis MRI|MRI]] | [[Malignant histiocytosis other imaging findings|Other Imaging Findings]] | [[Malignant histiocytosis other diagnostic studies|Other Diagnostic Studies]] |
| **Systemic histiocytosis
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| *Reactive diseases of macrophages
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| **Hemophagocytic syndrome - a condition where macrophages phagocytose [[myeloid]] and [[erythroid]] precursors (similar to [[HLH (Hemophagocytic lymphohistiocytosis)|hemophagocytic lymphohistiocytosis]] in humans)
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| *Malignant diseases of Langerhans dells
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| **Malignant histiocytosis
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| **Diffuse histiocytic sarcoma
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| **Localized histiocytic sarcoma
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| *Malignant diseases of macrophages
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| **Histiocytic lymphoma
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| ==Breed disposition==
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| Up to 25 percent of Bernese Mountain Dogs may develop malignant histiocytosis in their lifetime.<ref>{{cite web|author=Parker, A.G., Rutteman, G.R., Cadieu, E., et al.|year=2006|title=Malignant Histiocytosis in the Bernese Mountain Dog: Design and Execution of a Case-Control Study|work=Genes, Dogs and Cancer: Fourth Annual Canine Cancer Conference| url=http://www.ivis.org/proceedings/keystone/2006/sessionB/parker/chapter.asp?la=1|accessdate=2007-01-28}}</ref> Other breeds with a possible genetic tendency toward malignant histiocytosis include Rottweilers, Flat-Coated Retrievers, and Golden Retrievers<ref name=Ettinger_1995>{{cite book|author=Ettinger, Stephen J.;Feldman, Edward C.|title=Textbook of Veterinary Internal Medicine|edition=4th ed.|publisher=W.B. Saunders Company|year=1995|id=ISBN 0-7216-6795-3}}</ref>.
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| ==Signs and symptoms== | |
| The disease in the lungs is characterized by enlargement of the tracheobronchial lymph nodes and infiltration of the lungs, sometimes leading to lung lobe consolidation and [[pleural effusion]].<ref>{{cite web|author=Kessler, Martin|year=2006|title=Radiographic diagnosis of primary and metastatic lung tumors|work=Proceedings of the North American Veterinary Conference| url=http://www.ivis.org/proceedings/navc/2006/SAE/275.pdf?LA=1|accessdate=2007-01-28}}</ref> Signs and symptoms include cough, loss of appetite, weight loss, anemia, and difficulty breathing.<ref>{{cite web | last = Affolter | first = Verena K. | title = Histiocytic Proliferative Diseases in Dogs and Cats | work = Proceedings of the 29th World Congress of the World Small Animal Veterinary Association | date = 2004 | url = http://www.vin.com/proceedings/Proceedings.plx?CID=WSAVA2004&PID=8600&O=Generic | accessdate = 2007-01-28 }}</ref> [[Seizure]]s and rear limb weakness can be seen. Invasion of the bone marrow can cause [[pancytopenia]].<ref>{{cite journal | last = Kearns | first = Shawn Ann | coauthors = Ewing, Patty | title = Causes of canine and feline pancytopenia | journal = Compendium on Continuing Education for the Practicing Veterinarian | volume = 28 | issue = 2 | pages = 122-133 | publisher = Veterinary Learning Systems | date = Feb. 2006 }}</ref> Diagnosis requires a [[biopsy]].
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| ==Treatment== | | ==Treatment== |
| Treatment with [[chemotherapy]] has been used with some success, particularly using [[lomustine]], [[prednisone]], [[doxorubicin]], and [[cyclophosphamide]].<ref name=DVM>{{cite journal | last = Cronin | first = Kim | title = Deciphering the histiocytic code | journal = DVM | pages = 1S-8S | publisher = Advanstar Communications | date = Dec. 2006 }}</ref> Because of the rapid progression of this aggressive disease, the [[prognosis]] is very poor.<ref>{{cite journal |author=Affolter V, Moore P |title=Localized and disseminated histiocytic sarcoma of dendritic cell origin in dogs |journal=Vet Pathol |volume=39 |issue=1 |pages=74-83 |year=2002 |id=PMID 12102221}}</ref>
| | [[Malignant histiocytosis medical therapy|Medical Therapy]] | [[Malignant histiocytosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Malignant histiocytosis future or investigational therapies|Future or Investigational Therapies]] |
| | | ==Case Studies== |
| ==Other histiocytic diseases in Bernese Mountain Dogs==
| | :[[Malignant histiocytosis case study one|Case #1]] |
| A similar disease is '''diffuse histiocytic sarcoma''', a term used to designate a localized histiocytic sarcoma that has spread throughout the body.<ref name=DVM/>
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| Another disease of histiocytic origin that affects Bernese Mountain Dogs is '''systemic histiocytosis'''. This condition generally begins as lesions on the [[eyelid]]s, nasal [[mucous membrane|mucosa]], and skin, especially the [[scrotum]]. It progresses to a more generalized disease affecting the lymph nodes, bone marrow and spleen.<ref>{{cite web | title = Tumors with Histiocytic Differentiation | work = The Merck Veterinary Manual | date = 2006 | url = http://www.merckvetmanual.com/mvm/index.jsp?cfile=htm/bc/72232.htm | accessdate = 2007-01-28 }}</ref> Other signs and symptoms include weight loss and loss of appetite.<ref name=DVM/> It also has a very poor prognosis.
| | ==Related chapters== |
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| ==See also==
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| *[[Histiocytosis]] | | *[[Histiocytosis]] |
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| ==References==
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| {{Reflist|2}}
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| {{Hematology}} | | {{Hematology}} |