Fatal case of TAFRO syndrome with unilateral adrenal hemorrhage in early-stage disease
Autor: | Fumihiko Nakamura, Toshiya Nakatani, Hidekazu Azumi, Shinya Kobayashi, Shinichi Ochi, Hideo Yagi, Yuki Motokawa, Tomoya Okamoto |
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Jazyk: | angličtina |
Rok vydání: | 2021 |
Předmět: |
medicine.medical_specialty
Case Report Hemorrhage Anasarca Gastroenterology Organomegaly chemistry.chemical_compound Tocilizumab Bone Marrow Internal medicine Adrenal Glands Medicine Humans adrenal hemorrhage Stage (cooking) Aged TAFRO business.industry Castleman Disease Gamma globulin General Medicine iMCD chemistry Female medicine.symptom business Early stage disease Adrenal Hemorrhage Rare disease |
Zdroj: | Journal of Clinical and Experimental Hematopathology : JCEH |
ISSN: | 1880-9952 1346-4280 |
Popis: | Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly comprise TAFRO syndrome, which was proposed as a distinct clinical entity from iMCD without TAFRO syndrome (iMCD-NOS) due to its aggressive clinical course, refractoriness to corticosteroids, presence of thrombocytopenia, increased level of alkaline phosphatase, and normal level of gammaglobulin. However, diagnosing TAFRO syndrome in its early stages is challenging because it is rare and its diagnostic criteria are complicated. We describe a patient with TAFRO syndrome and adrenal hemorrhage who demonstrated a rapid decline in her clinical condition and did not respond to steroid pulse therapy, resulting in a fatal outcome. In the early stage of her clinical course, she developed unilateral adrenal hemorrhage with mild thrombocytopenia and normal clotting times, suggesting adrenal hemorrhage as a unique manifestation of TAFRO syndrome. In general, patients with TAFRO syndrome exhibit a more aggressive clinical course and poorer outcome than those with iMCD-NOS. To ameliorate this poor prognosis, it is important to diagnose the disease early and immediately start powerful immunosuppressive agents such as tocilizumab. Based on this case, adrenal hemorrhage may suggest TAFRO syndrome, and facilitate the rapid diagnosis of this complicated and rare disease. |
Databáze: | OpenAIRE |
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