Shogo Minomo1, Yu Fujiwara2,3, Shota Sakashita4, Akito Takamura5, Kaoru Nagata6. 1. Department of General Internal Medicine, Musashino Red Cross Hospital, 1-26-1, Kyonancho, Musashino-shi, Tokyo, 1808610, Japan. shogo189@gmail.com. 2. Department of Medicine, Icahn School of Medicine at Mount Sinai, Mount Sinai Beth Israel, 281 First Avenue, New York, NY, 10003, USA. 3. Department of Medical Oncology, Musashino Red Cross Hospital, 1-26-1, Kyonancho, Musashino-shi, Tokyo, 1808610, Japan. 4. Department of Nephrology, Musashino Red Cross Hospital, 1-26-1, Kyonancho, Musashino-shi, Tokyo, 1808610, Japan. 5. Department of Rheumatology and Collagen Disease, Musashino Red Cross Hospital, 1-26-1, Kyonancho, Musashino-shi, Tokyo, 1808610, Japan. 6. Department of General Internal Medicine, Musashino Red Cross Hospital, 1-26-1, Kyonancho, Musashino-shi, Tokyo, 1808610, Japan.
Abstract
BACKGROUND: TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly) syndrome is a recently recognized disease with a variety of presentations of variable severity. In acute settings, this disease also involves organ dysfunction because of the associated systemic inflammation. However, cases of TAFRO syndrome with myocardial and/or skeletal muscle calcification have never been reported. CASE PRESENTATION: A 24-year-old healthy young Asian man was admitted with intermittent epigastric pain and fever for 2 weeks. Computed tomography revealed pleural effusion, ascites and systemic lymphadenopathy. Laboratory tests showed thrombocytopenia, elevated C-reactive protein, hypoalbuminemia, anemia and renal dysfunction. Based on these findings and bone marrow biopsy, we diagnosed his disease as TAFRO syndrome and commenced hemodialysis for the renal dysfunction. However, he developed refractory hypocalcemia with unstable vital signs, for which we administered calcium gluconate hydrate. Thereafter, myocardial and skeletal muscle calcification was revealed radiologically, with the myocardial calcification causing sick sinus syndrome. He was treated with tocilizumab and finally discharged in an ambulatory condition after prolonged hospitalization, with residual calcific lesions. CONCLUSION: This is the first report of a patient with TAFRO syndrome and the complication of organ calcification. The etiology of calcification in this case is not clear. Systemic inflammation with possible hypercytokinemia might have been involved in the unexpected complication of systemic calcification. It is important to carefully handle the general management of TAFRO syndrome because of the possibility of various complications.
BACKGROUND:TAFRO (thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly) syndrome is a recently recognized disease with a variety of presentations of variable severity. In acute settings, this disease also involves organ dysfunction because of the associated systemic inflammation. However, cases of TAFRO syndrome with myocardial and/or skeletal muscle calcification have never been reported. CASE PRESENTATION: A 24-year-old healthy young Asian man was admitted with intermittent epigastric pain and fever for 2 weeks. Computed tomography revealed pleural effusion, ascites and systemic lymphadenopathy. Laboratory tests showed thrombocytopenia, elevated C-reactive protein, hypoalbuminemia, anemia and renal dysfunction. Based on these findings and bone marrow biopsy, we diagnosed his disease as TAFRO syndrome and commenced hemodialysis for the renal dysfunction. However, he developed refractory hypocalcemia with unstable vital signs, for which we administered calcium gluconate hydrate. Thereafter, myocardial and skeletal muscle calcification was revealed radiologically, with the myocardial calcification causing sick sinus syndrome. He was treated with tocilizumab and finally discharged in an ambulatory condition after prolonged hospitalization, with residual calcific lesions. CONCLUSION: This is the first report of a patient with TAFRO syndrome and the complication of organ calcification. The etiology of calcification in this case is not clear. Systemic inflammation with possible hypercytokinemia might have been involved in the unexpected complication of systemic calcification. It is important to carefully handle the general management of TAFRO syndrome because of the possibility of various complications.
Authors: Frits van Rhee; Peter Voorhees; Angela Dispenzieri; Alexander Fosså; Gordan Srkalovic; Makoto Ide; Nikhil Munshi; Stephen Schey; Matthew Streetly; Sheila K Pierson; Helen L Partridge; Sudipto Mukherjee; Dustin Shilling; Katie Stone; Amy Greenway; Jason Ruth; Mary Jo Lechowicz; Shanmuganathan Chandrakasan; Raj Jayanthan; Elaine S Jaffe; Heather Leitch; Naveen Pemmaraju; Amy Chadburn; Megan S Lim; Kojo S Elenitoba-Johnson; Vera Krymskaya; Aaron Goodman; Christian Hoffmann; Pier Luigi Zinzani; Simone Ferrero; Louis Terriou; Yasuharu Sato; David Simpson; Raymond Wong; Jean-Francois Rossi; Sunita Nasta; Kazuyuki Yoshizaki; Razelle Kurzrock; Thomas S Uldrick; Corey Casper; Eric Oksenhendler; David C Fajgenbaum Journal: Blood Date: 2018-09-04 Impact factor: 25.476