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
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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
- ^ PMID 17433850.
- ^ Meneshian A, Giaccone G, Olivier KR. "Clinical presentation and management of thymoma and thymic carcinoma". UpToDate.
- S2CID 26959211.
- PMID 7832281.