Ryo Kurokawa1, Wataru Gonoi2, Hajime Yokota3, Saiko Isshiki4, Kenji Ohira5, Hideaki Mizuno6, Takao Kiguchi7, Shohei Inui1,8, Mariko Kurokawa9,10, Shimpei Kato1,11, Mitsuru Matsuki12, Taro Takeda13, Kota Yokoyama14, Yoshiaki Ota15, Yudai Nakai1, Eriko Maeda1, Harushi Mori1, Osamu Abe1. 1. Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. 2. Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. watapi-tky@umin.net. 3. Department of Diagnostic Radiology and Radiation Oncology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba-shi, Chiba, 260-8677, Japan. 4. Department of Radiology, Nippon Medical School Musashikosugi Hospital, 1-396 Kosugi-machi, Nakahara-ku, Kawasaki-shi, Kanagawa, 211-8533, Japan. 5. Department of Radiology, Chiba University Hospita, 1-8-1 Inohana, Chuo-ku, Chiba-shi, Chiba, 260-8677, Japan. 6. Department of Hematology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan. 7. Department of Diagnostic Radiology, Ichinomiya Nishi Hospital, 1-Hira Kaimei, Ichinomiya City, Aichi, 494-0001, Japan. 8. Department of Radiology, Teikyo University Hospital, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8606, Japan. 9. Department of Radiology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan. 10. Department of Radiology, Osaka General Medical Center, 3-1-56 Mandai-Higashi, Sumiyoshi-ku, Osaka, 558-8558, Japan. 11. Department of Radiology, Juntendo University, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan. 12. Department of Radiology, Kindai University Faculty of Medicine, 377-2 Ohnohigashi, Osaka-Sayama City, Osaka, 589-8511, Japan. 13. Department of Radiology, Matsunami General Hospital, 185-1 Dendai, Kasamatsu-cho, Hashima-gun, Gifu, 501-6062, Japan. 14. Department of Radiology, Tokyo Medical and Dental University Hospital, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan. 15. Division of Neuroradiology, Department of Radiology, Michigan Medicine, 1500E Medical Center Drive, Ann Arbor, MI, 48109, USA.
Abstract
OBJECTIVES: To compare CT findings of early (within 3 weeks post-onset)- and later (within 1 month before or after diagnostic criteria were satisfied, and later than 3 weeks post-onset) stage thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome. METHODS: Between 2014 and 2019, 13 patients with TAFRO syndrome (8 men and 5 women; mean age, 54.9 years) from nine hospitals were enrolled. The number of the following CT findings (CT factors) was recorded: the presence of anasarca, organomegaly, adrenal ischaemia, anterior mediastinal lesion, bony lesion, and lymphadenopathy. Records of adrenal disorders (adrenomegaly, ischaemia, and haemorrhage) throughout the disease course were also collected. Differences in CT factors at each stage were statistically compared between remission and deceased groups. RESULTS: Para-aortic oedema and mild lymphadenopathy were observed in all patients, whereas pleural effusion, ascites, and subcutaneous oedema were found in 5/13, 7/13, and 7/13 cases, respectively, at the early stage. CT factors at the early stage were significantly higher in the deceased than in the remission group (mean, 11 vs 6.5; p = 0.04), while they were nonsignificant at the later stage. Adrenal disorders were present in 7/13 cases throughout the course including 6 of adrenomegaly and 4 of ischaemia at the early stage. CONCLUSIONS: Para-aortic oedema and mild lymphadenopathy are most common at the early stage. Anasarca, organomegaly, lymphadenopathy, and adrenal disorders on early-stage CT are useful for unfavourable prognosis prediction. Moreover, adrenal disorders are frequent even at the early stage and are useful for early diagnosis of TAFRO syndrome. KEY POINTS: • CT findings facilitate early diagnosis and prognosis prediction in TAFRO syndrome. • Adrenal disorders are frequently observed in TAFRO syndrome. • Adrenal disorders are useful for differential diagnosis of TAFRO syndrome.
OBJECTIVES: To compare CT findings of early (within 3 weeks post-onset)- and later (within 1 month before or after diagnostic criteria were satisfied, and later than 3 weeks post-onset) stage thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome. METHODS: Between 2014 and 2019, 13 patients with TAFRO syndrome (8 men and 5 women; mean age, 54.9 years) from nine hospitals were enrolled. The number of the following CT findings (CT factors) was recorded: the presence of anasarca, organomegaly, adrenal ischaemia, anterior mediastinal lesion, bony lesion, and lymphadenopathy. Records of adrenal disorders (adrenomegaly, ischaemia, and haemorrhage) throughout the disease course were also collected. Differences in CT factors at each stage were statistically compared between remission and deceased groups. RESULTS:Para-aortic oedema and mild lymphadenopathy were observed in all patients, whereas pleural effusion, ascites, and subcutaneous oedema were found in 5/13, 7/13, and 7/13 cases, respectively, at the early stage. CT factors at the early stage were significantly higher in the deceased than in the remission group (mean, 11 vs 6.5; p = 0.04), while they were nonsignificant at the later stage. Adrenal disorders were present in 7/13 cases throughout the course including 6 of adrenomegaly and 4 of ischaemia at the early stage. CONCLUSIONS:Para-aortic oedema and mild lymphadenopathy are most common at the early stage. Anasarca, organomegaly, lymphadenopathy, and adrenal disorders on early-stage CT are useful for unfavourable prognosis prediction. Moreover, adrenal disorders are frequent even at the early stage and are useful for early diagnosis of TAFRO syndrome. KEY POINTS: • CT findings facilitate early diagnosis and prognosis prediction in TAFRO syndrome. • Adrenal disorders are frequently observed in TAFRO syndrome. • Adrenal disorders are useful for differential diagnosis of TAFRO syndrome.
Authors: Amanallah Montazeripouragha; Christine M Campbell; James Russell; Nadia Medvedev; Daniel R Owen; Alison Harris; Fergal Donnellan; Iain McCormick; David C Fajgenbaum; Luke Y C Chen Journal: Am J Hematol Date: 2022-07-19 Impact factor: 13.265