Medicine:Thymoma-associated multiorgan autoimmunity
| Thymoma-associated multiorgan autoimmunity (TAMA) | |
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Thymoma-associated multiorgan autoimmunity (TAMA) is a severe often fatal disease that presents in some patients with thymoma.[1] It has also been referred to in the medical literature as "thymoma-associated graft-versus-host-like disease".
Presentation
Patients with TAMA present with variable combinations of a morbilliform skin eruption, chronic diarrhea, and abnormal liver enzymes. The histopathology of the skin, liver, or bowel mucosa resembles GVHD.[2]
Pathophysiology
Thymoma is a common neoplasm arising from the thymus, the primary lymphoid organ where T cells become educated to distinguish "self" from "non self". In the setting of thymoma, abnormal thymic education occurs as a result of subtle differences in antigen processing. In TAMA these differences result in autoreactive T cells escaping from the thymus. This results in a condition similar to graft-versus-host disease.[1]
Diagnosis
Treatment
Patients often have a refractory disease course but some patients may respond to phototherapy.[3]
History
This disease name was coined by Emanual Maverakis and described in detail in 2007 but case reports of graft-versus-host-like disease in the setting of thymoma date back to at least the mid 1990s.[1][4]
References
- ↑ 1.0 1.1 1.2 "Thymoma-associated multiorgan autoimmunity: a graft-versus-host-like disease". J Am Acad Dermatol 57 (4): 683–9. Oct 2007. doi:10.1016/j.jaad.2007.02.027. PMID 17433850.
- ↑ "Clinical presentation and management of thymoma and thymic carcinoma.". UpToDate. https://www.uptodate.com/contents/clinical-presentation-and-management-of-thymoma-and-thymic-carcinoma?source=search_result&search=Thymoma%20associated%20multiorgan%20autoimmunity&selectedTitle=1~1.
- ↑ "Thymoma-associated cutaneous graft-versus-host-like disease possibly treated with Narrow-band UVB phototherapy". Eur J Dermatol 26 (2): 208–9. Apr 2016. doi:10.1684/ejd.2015.2716. PMID 27018042.
- ↑ "Graft-versus-host-like colitis associated with malignant thymoma". Am J Surg Pathol 19 (2): 224–8. 1995. doi:10.1097/00000478-199502000-00011. PMID 7832281.
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