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Malignant histiocytosis

Malignant histiocytosis
Classification & external resources
ICD-10 C96.1
ICD-9 202.3
ICD-O: 9750/3
MeSH D006647

Malignant histiocytosis is a hereditary disease found in the Bernese Mountain Dog characterized by histiocytic infiltration of the lungs and lymph nodes. 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.[1]


Histiocytic disorders

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 cells are dendritic cells found in the skin and function by internalizing antigens (foreign particles) and presenting them to T cells. They arise from monocytes.[2] Histiocytic disorders refer to diseases that are caused by abnormal behavior of these cells. They include the following:

  • Reactive diseases of Langerhans cells
  • Reactive diseases of macrophages
  • Malignant diseases of Langerhans dells
    • Malignant histiocytosis
    • Diffuse histiocytic sarcoma
    • Localized histiocytic sarcoma
  • Malignant diseases of macrophages
    • Histiocytic lymphoma

Breed disposition

Up to 25 percent of Bernese Mountain Dogs may develop malignant histiocytosis in their lifetime.[3] Other breeds with a possible genetic tendency toward malignant histiocytosis include Rottweilers, Flat-Coated Retrievers, and Golden Retrievers[4].

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.[5] Signs and symptoms include cough, loss of appetite, weight loss, anemia, and difficulty breathing.[6] Seizures and rear limb weakness can be seen. Invasion of the bone marrow can cause pancytopenia.[7] Diagnosis requires a biopsy.


Treatment with chemotherapy has been used with some success, particularly using lomustine, prednisone, doxorubicin, and cyclophosphamide.[8] Because of the rapid progression of this aggressive disease, the prognosis is very poor.[9]

Other histiocytic diseases in Bernese Mountain Dogs

A similar disease is diffuse histiocytic sarcoma, a term used to designate a localized histiocytic sarcoma that has spread throughout the body.[8]

Another disease of histiocytic origin that affects Bernese Mountain Dogs is systemic histiocytosis. This condition generally begins as lesions on the eyelids, nasal mucosa, and skin, especially the scrotum. It progresses to a more generalized disease affecting the lymph nodes, bone marrow and spleen.[10] Other signs and symptoms include weight loss and loss of appetite.[8] It also has a very poor prognosis.

See also


  1. ^
  2. ^ Ginhoux F, Tacke F, Angeli V, Bogunovic M, Loubeau M, Dai XM, Stanley ER, Randolph GJ, Merad M (2006). "Langerhans cells arise from monocytes in vivo". Nat. Immunol. 7 (3): 265-73. PMID 16444257.
  3. ^ Parker, A.G., Rutteman, G.R., Cadieu, E., et al. (2006). Malignant Histiocytosis in the Bernese Mountain Dog: Design and Execution of a Case-Control Study. Genes, Dogs and Cancer: Fourth Annual Canine Cancer Conference. Retrieved on 2007-01-28.
  4. ^ Ettinger, Stephen J.;Feldman, Edward C. (1995). Textbook of Veterinary Internal Medicine, 4th ed., W.B. Saunders Company. ISBN 0-7216-6795-3. 
  5. ^ Kessler, Martin (2006). Radiographic diagnosis of primary and metastatic lung tumors. Proceedings of the North American Veterinary Conference. Retrieved on 2007-01-28.
  6. ^ Affolter, Verena K. (2004). Histiocytic Proliferative Diseases in Dogs and Cats. Proceedings of the 29th World Congress of the World Small Animal Veterinary Association. Retrieved on 2007-01-28.
  7. ^ Kearns, Shawn Ann; Ewing, Patty (Feb. 2006). "Causes of canine and feline pancytopenia". Compendium on Continuing Education for the Practicing Veterinarian 28 (2): 122-133. Veterinary Learning Systems.
  8. ^ a b c Cronin, Kim (Dec. 2006). "Deciphering the histiocytic code". DVM: 1S-8S. Advanstar Communications.
  9. ^ Affolter V, Moore P (2002). "Localized and disseminated histiocytic sarcoma of dendritic cell origin in dogs". Vet Pathol 39 (1): 74-83. PMID 12102221.
  10. ^ Tumors with Histiocytic Differentiation. The Merck Veterinary Manual (2006). Retrieved on 2007-01-28.
This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Malignant_histiocytosis". A list of authors is available in Wikipedia.
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