Successful use of tocilizumab (TCZ) to treat TAFRO syndrome has recently been reported. In those cases, TCZ was used with steroid. We present herein the case of a 59-year-old man with very severe TAFRO syndrome who was successfully treated using TCZ without steroid. He showed rapidly progressive anasarca, acute renal failure and very severe thrombocytopenia. We initially used steroid, but its efficacy was limited. Moreover, steroid use had to be stopped as soon as possible, because hemorrhagic shock developed due to severe duodenal ulcer. After overcoming infections (about 40 days after stopping steroid), administration of TCZ was started and the patient was discharged in clinical remission.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5438817PMC
http://dx.doi.org/10.1007/s13730-017-0253-6DOI Listing

Publication Analysis

Top Keywords

tafro syndrome
12
tcz steroid
8
steroid
6
successful treatment
4
treatment tocilizumab
4
tocilizumab steroid
4
severe
4
steroid severe
4
severe case
4
case tafro
4

Similar Publications

Computed tomography findings of idiopathic multicentric Castleman disease subtypes.

J Clin Exp Hematop

December 2024

Department of Radiology, Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan.

This study retrospectively evaluated the computed tomography (CT) findings of idiopathic multicentric Castleman disease (iMCD) at a single center and compared the CT findings of iMCD-TAFRO with those of iMCD-non-TAFRO. CT images obtained within 30 days before diagnostic confirmation were reviewed for 20 patients with iMCD (8 men and 12 women, mean age 52.8 ± 12.

View Article and Find Full Text PDF

POEMS syndrome is a complex and rare hematological disease involving multiple physiological systems. According to the currently accepted diagnostic criteria for POEMS syndrome, polyneuropathy is one of the primary mandatory criteria. A patient presented with M protein, Castleman disease (CD), elevated vascular endothelial growth factor (VEGF), extravascular volume overload, and endocrinopathy.

View Article and Find Full Text PDF

The majority of multicentric Castleman disease (MCD) patients in China are of the idiopathic subtype (iMCD) with systemic manifestations. However, the impact of iMCD on life quality, mental and psychological status, social function, and caregiving burden is poorly understood. To address this gap, a cross-sectional web-based survey was conducted with 178 iMCD patients and 82 caregivers, including 42 patient-caregiver dyads.

View Article and Find Full Text PDF

Identifying Castleman disease from non-clonal inflammatory causes of generalized lymphadenopathy.

Hematology Am Soc Hematol Educ Program

December 2024

Raymond and Ruth Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

Idiopathic multicentric Castleman disease (iMCD) is a rare, life-threatening subtype of Castleman disease (CD), which describes a group of rare, polyclonal lymphoproliferative disorders that demonstrate characteristic histopathology and variable symptomatology. iMCD involves a cytokine storm that occurs due to an unknown cause. Rapid diagnosis is required to initiate appropriate, potentially life-saving therapy, but diagnosis is challenging and impeded by clinical overlap with a wide spectrum of inflammatory, neoplastic, and infectious causes of generalized lymphadenopathy.

View Article and Find Full Text PDF

[Research Advances in Pathogenesis of Idiopathic Multicentric Castleman Disease].

Zhongguo Yi Xue Ke Xue Yuan Xue Bao

October 2024

Department of Hematology, PUMC Hospital,CAMS and PUMC,Beijing 100730,China.

Idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening lymphoproliferative disorder involving systemic inflammatory symptoms,polyclonal lymphoproliferation,cytopenia,and multiple organ dysfunction.Although interleukin-6-mediated cytokine storm is thought to be the key driver of iMCD,more than 50% of the patients fail to respond to the treatments targeting interleukin-6 or its receptors.This underscores the urgent need to identify other cytokines and pathogenic pathways that may underlie iMCD.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!