Malignant histiocytosis

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Malignant histiocytosis
Other namesHistiocytic medullary reticulosis [1] :744
Specialty Oncology, angiology   OOjs UI icon edit-ltr-progressive.svg

Malignant histiocytosis is a rare hereditary disease found in the Bernese Mountain Dog and humans, 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. [2]

Contents

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. [3] Histiocytic disorders refer to diseases that are caused by abnormal behavior of these cells. They include the following:

Breed disposition

Up to 25 percent of Bernese Mountain Dogs may develop malignant histiocytosis in their lifetime. [4] [ better source needed ] Other breeds with a possible genetic tendency toward malignant histiocytosis include Rottweilers, Flat-Coated Retrievers, and Golden Retrievers. [5] [ page needed ]

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. [6] Signs and symptoms include cough, loss of appetite, weight loss, anemia, and difficulty breathing. [7] Seizures and rear limb weakness can be seen. Invasion of the bone marrow can cause pancytopenia. [8] Diagnosis requires a biopsy.

Treatment

Treatment with chemotherapy has been used with some success, particularly using lomustine, prednisone, doxorubicin, and cyclophosphamide. [9] Because of the rapid progression of this aggressive disease, the prognosis is very poor. [10]

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. Other signs and symptoms include weight loss and loss of appetite. [9] [ better source needed ]

See also

Related Research Articles

Dendritic cell Accessory cell of the mammalian immune system

Dendritic cells (DCs) are antigen-presenting cells of the mammalian immune system. Their main function is to process antigen material and present it on the cell surface to the T cells of the immune system. They act as messengers between the innate and the adaptive immune systems.

Tumors of the hematopoietic and lymphoid tissues Medical condition

Tumors of the hematopoietic and lymphoid tissues or haematopoietic and lymphoid malignancies are tumors that affect the blood, bone marrow, lymph, and lymphatic system. Because these tissues are all intimately connected through both the circulatory system and the immune system, a disease affecting one will often affect the others as well, making myeloproliferation and lymphoproliferation closely related and often overlapping problems.

Panniculitis Medical condition

Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue. Symptoms include tender skin nodules, and systemic signs such as weight loss and fatigue.

Langerhans cell

Langerhans cells (LC) are tissue-resident macrophages of the skin, and contain organelles called Birbeck granules. They are present in all layers of the epidermis and are most prominent in the stratum spinosum. They also occur in the papillary dermis, particularly around blood vessels, as well as in the mucosa of the mouth, foreskin, and vaginal epithelium. They can be found in other tissues, such as lymph nodes, particularly in association with the condition Langerhans cell histiocytosis (LCH).

Langerhans cell histiocytosis Medical condition

Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes.

A histiocyte is a vertebrate cell that is part of the mononuclear phagocyte system. The mononuclear phagocytic system is part of the organism's immune system. The histiocyte is a tissue macrophage or a dendritic cell.

Lymphoma in animals

Lymphoma (lymphosarcoma) in animals is a type of cancer defined by a proliferation of malignant lymphocytes within solid organs such as the lymph nodes, bone marrow, liver and spleen. The disease also may occur in the eye, skin, and gastrointestinal tract.

Histiocytoma (dog)

A histiocytoma in the dog is a benign tumor. It is an abnormal growth in the skin of histiocytes (histiocytosis), a cell that is part of the immune system. A similar disease in humans, Hashimoto-Pritzker disease, is also a Langerhans cell histiocytosis. Dog breeds that may be more at risk for this tumor include Bulldogs, American Pit Bull Terriers, American Staffordshire Terriers, Scottish Terriers, Greyhounds, Boxers, and Boston Terriers. They also rarely occur in goats and cattle.

Langhans giant cell

Langhans giant cells are large cells found in granulomatous conditions.

CD68

CD68 is a protein highly expressed by cells in the monocyte lineage, by circulating macrophages, and by tissue macrophages.

In medicine, histiocytosis is an excessive number of histiocytes, and the term is also often used to refer to a group of rare diseases which share this sign as a characteristic. Occasionally and confusingly, the term "histiocytosis" is sometimes used to refer to individual diseases.

Chronic multifocal Langerhans cell histiocytosis Medical condition

Chronic multifocal Langerhans cell histiocytosis, previously known as Hand–Schüller–Christian disease, is a type of Langerhans cell histiocytosis (LCH), which can affect multiple organs. The condition is traditionally associated with a combination of three features; bulging eyes, breakdown of bone, and diabetes insipidus, although around 75% of cases do not have all three features. Other features may include a fever and weight loss, and depending on the organs involved there maybe rashes, asymmetry of the face, ear infections, signs in the mouth and the appearance of advanced gum disease. Features relating to lung and liver disease may occur.

Lymphoid hyperplasia is the rapid proliferation of normal lymphocytic cells that resemble lymph tissue which may occur with bacterial or viral infections. The growth is termed hyperplasia which may result in enlargement of various tissue including an organ, or cause a cutaneous lesion.

Rosai–Dorfman disease Medical condition

Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as Destombes–Rosai–Dorfman disease, is a rare disorder of unknown cause that is characterized by abundant histiocytes in the lymph nodes or other locations throughout the body.

Non-X histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of monocytes/macrophages, as opposed to X-type histiocytoses in which the infiltrates contain Langerhans cells. Conditions included in this group are:

X-type histiocytoses are a clinically well-defined group of cutaneous syndromes characterized by infiltrates of Langerhans cells, as opposed to Non-X histiocytosis in which the infiltrates contain monocytes/macrophages. Conditions included in this group are:

Histiocytic diseases in dogs are a group of diseases in dogs which may involve the skin, and which can be difficult to differentiate from granulomatous, reactive inflammatory or lymphoproliferative diseases. The clinical presentation and behaviour as well as response to therapy vary greatly among the syndromes.

The Xanthogranulomatous Process (XP), is a form of acute and chronic inflammation characterized by an exuberant clustering of foamy macrophages among other inflammatory cells. Localization in the kidney and renal pelvis has been the most frequent and better known occurrence followed by that in the gallbladder but many others have been subsequently recorded. The pathological findings of the process and etiopathogenetic and clinical observations have been reviewed by Cozzutto and Carbone.

Epstein–Barr virus-associated lymphoproliferative diseases are a group of disorders in which one or more types of lymphoid cells, i.e. B cells, T cells, NK cells, and histiocytic-dendritic cells, are infected with the Epstein–Barr virus (EBV). This causes the infected cells to divide excessively, and is associated with the development of various non-cancerous, pre-cancerous, and cancerous lymphoproliferative disorders (LPDs). These LPDs include the well-known disorder occurring during the initial infection with the EBV, infectious mononucleosis, and the large number of subsequent disorders that may occur thereafter. The virus is usually involved in the development and/or progression of these LPDs although in some cases it may be an "innocent" bystander, i.e. present in, but not contributing to, the disease.

References

  1. James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN   0-7216-2921-0.
  2. "Malignant histiocytoses | DermNet NZ". dermnetnz.org.
  3. 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. doi:10.1038/ni1307. PMC   4727824 . PMID   16444257.
  4. E Cadieu, B Hedan, G Parker H, Gr Rutteman, A Baffoe-Bonnie, et al.. Genetics of Malignant Histiocytosis in the Bernese Mountain Dog. 3rd International Conference on Advances in Feline and Canine Genomics, 2006, UC Davis, United States. pp.Inconnu. ⟨hal-02815226⟩
  5. Ettinger, Stephen J.; Feldman, Edward C. (1995). Textbook of Veterinary Internal Medicine (4th ed.). W.B. Saunders Company. ISBN   0-7216-6795-3.
  6. Kessler, Martin (2006). "Radiographic diagnosis of primary and metastatic lung tumors" (PDF). Proceedings of the North American Veterinary Conference. Archived from the original (PDF) on 2007-09-29. Retrieved 2007-01-28.
  7. 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 2007-01-28.
  8. Kearns, Shawn Ann; Ewing, Patty (Feb 2006). "Causes of canine and feline pancytopenia". Compendium on Continuing Education for the Practicing Veterinarian. Veterinary Learning Systems. 28 (2): 122–133.
  9. 1 2 Cronin, Kim (Dec 2006). "Deciphering the histiocytic code". DVM. Advanstar Communications: 1S–8S.
  10. Affolter V, Moore P (2002). "Localized and disseminated histiocytic sarcoma of dendritic cell origin in dogs". Vet Pathol. 39 (1): 74–83. doi:10.1354/vp.39-1-74. PMID   12102221. S2CID   17366063.
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