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".
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]
Pathophysiologyedit
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]
Diagnosisedit
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Treatmentedit
Patients often have a refractory disease course but some patients may respond to phototherapy.[3]
Historyedit
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]
Referencesedit
^ abcWadhera A, Maverakis E, Mitsiades N, Lara PN, Fung MA, Lynch PJ (Oct 2007). "Thymoma-associated multiorgan autoimmunity: a graft-versus-host-like disease". J Am Acad Dermatol. 57 (4): 683–9. doi:10.1016/j.jaad.2007.02.027. PMID 17433850.
^Meneshian A, Giaccone G, Olivier KR. "Clinical presentation and management of thymoma and thymic carcinoma". UpToDate.
^Nakayama M, Itoh M, Kikuchi S, Tanito K, Nakagawa H (Apr 2016). "Thymoma-associated cutaneous graft-versus-host-like disease possibly treated with Narrow-band UVB phototherapy". Eur J Dermatol. 26 (2): 208–9. doi:10.1684/ejd.2015.2716. PMID 27018042. S2CID 26959211.
^ Kornacki S, Hansen FC, Lazenby A (1995). "Graft-versus-host-like colitis associated with malignant thymoma". Am J Surg Pathol. 19 (2): 224–8. doi:10.1097/00000478-199502000-00011. PMID 7832281.
External linksedit
April 10, 2024
thymoma, associated, multiorgan, autoimmunity, tama, severe, often, fatal, disease, that, presents, some, patients, with, thymoma, also, been, referred, medical, literature, thymoma, associated, graft, versus, host, like, disease, tama, contents, presentation,. 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 Thymoma associated multiorgan autoimmunity TAMA Contents 1 Presentation 2 Pathophysiology 3 Diagnosis 4 Treatment 5 History 6 References 7 External linksPresentation editPatients 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 editThymoma 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 editThis section is empty You can help by adding to it October 2017 Treatment editPatients often have a refractory disease course but some patients may respond to phototherapy 3 History editThis 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 edit a b c Wadhera A Maverakis E Mitsiades N Lara PN Fung MA Lynch PJ Oct 2007 Thymoma associated multiorgan autoimmunity a graft versus host like disease J Am Acad Dermatol 57 4 683 9 doi 10 1016 j jaad 2007 02 027 PMID 17433850 Meneshian A Giaccone G Olivier KR Clinical presentation and management of thymoma and thymic carcinoma UpToDate Nakayama M Itoh M Kikuchi S Tanito K Nakagawa H Apr 2016 Thymoma associated cutaneous graft versus host like disease possibly treated with Narrow band UVB phototherapy Eur J Dermatol 26 2 208 9 doi 10 1684 ejd 2015 2716 PMID 27018042 S2CID 26959211 Kornacki S Hansen FC Lazenby A 1995 Graft versus host like colitis associated with malignant thymoma Am J Surg Pathol 19 2 224 8 doi 10 1097 00000478 199502000 00011 PMID 7832281 External links edit Retrieved from https en wikipedia org w index php title Thymoma associated multiorgan autoimmunity amp oldid 996494181, wikipedia, wiki, book, books, library,
