06 April 2020
: Case report
Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with Bilateral Adrenal Hemorrhage in Two Caucasian Patients
Unusual clinical course
Grégoire Ducoux1ABCDEF*, Arthur Guerber1ABCDEF, Cécile-Audrey Durel1ACDEF, Bouchra Asli1DE, Jehane Fadlallah2ABCDEFG, Arnaud Hot1DEDOI: 10.12659/AJCR.919536
Am J Case Rep 2020; 21:e919536
Abstract
BACKGROUND: Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (TAFRO) syndrome is a variant of idiopathic multicentric Castleman disease. Adrenal hemorrhage has rarely been reported in TAFRO syndrome, and previous cases have mainly been Asian patients. This report is of two Caucasian patients with TAFRO syndrome presenting with acute adrenal insufficiency due to bilateral adrenal hemorrhage.
CASE REPORT: Case 1 was a 19-year-old Caucasian man with no significant past medical history who was admitted with acute abdominal pain, vomiting, anorexia, and moderate weight loss. Case 2 was a 31-year-old Caucasian woman with no past medical history who was admitted to hospital with fever, dyspnea, thoracic and abdominal pain, polyarthralgia, and hypotension. Both patients had splenomegaly, mild lymphadenopathy, thrombocytopenia, acute kidney injury, and myelofibrosis. In both cases, lymph node biopsy histology showed mixed-type idiopathic multicentric Castleman disease. In both patients, a diagnosis of TAFRO was made, and they developed bilateral adrenal hemorrhage with adrenal insufficiency. Case 1 was treated with high-dose steroids, followed by tocilizumab infusion. Due to persistent thrombocytopenia, second-line treatment commenced with rituximab, but the patient relapsed two months later. Tocilizumab treatment was recommenced, which was followed by an immuno-allergic adverse event. He then had a good response to sirolimus. Case 2 died nine months after diagnosis due to acute respiratory distress.
CONCLUSIONS: Two cases of TAFRO syndrome presented with acute adrenal insufficiency due to bilateral adrenal hemorrhage. The symptoms were only partially controlled with tocilizumab, rituximab, and tacrolimus. Adrenal hemorrhage may be a specific manifestation of TAFRO syndrome.
Keywords: adrenal insufficiency, Edema, Giant Lymph Node Hyperplasia, primary myelofibrosis, renal insufficiency, Thrombocytopenia, Adrenal Gland Diseases, Fatal Outcome, Hemorrhage, young adult
SARS-CoV-2/COVID-19
04 July 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.934468
29 June 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.936896
16 June 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.936498
13 June 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.936889
In Press
04 Jul 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.936836
04 Jul 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.934468
04 Jul 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.936448
01 Jul 2022 : Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.936536
Most Viewed Current Articles
23 Feb 2022 : Case report
DOI :10.12659/AJCR.935250
Am J Case Rep 2022; 23:e935250
17 Feb 2022 : Case report
DOI :10.12659/AJCR.934399
Am J Case Rep 2022; 23:e934399
06 Dec 2021 : Case report
DOI :10.12659/AJCR.934406
Am J Case Rep 2021; 22:e934406
09 Feb 2022 : Case report
DOI :10.12659/AJCR.934744
Am J Case Rep 2022; 23:e934744