Clinical characteristics and treatment outcome of type I cryoglobulinemia in Chinese patients: a single-center study of 45 patients

• Published in: Annals of hematology Vol. 99; no. 8; pp. 1735 - 1740
• Main Authors: Zhang, Le-le, Cao, Xin-xin, Shen, Kai-ni, Han, Hong-xiao, Zhang, Cong-Li, Qiu, Yu, Zhao, Hao, Gao, Xue-min, Feng, Jun, Zhang, Lu, Zhou, Dao-bin, Li, Jian
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.08.2020; Springer Nature B.V
• Subjects: Adult; Aged; Aged, 80 and over; Asian Continental Ancestry Group; Blood diseases; Bortezomib - administration & dosage; China - epidemiology; Cryoglobulinemia - blood; Cryoglobulinemia - drug therapy; Cryoglobulinemia - mortality; Cryoglobulinemia - pathology; Disease-Free Survival; Female; Hematology; Humans; Immunotherapy; Inhibitor drugs; Male; Medicine; Medicine & Public Health; Middle Aged; Monoclonal antibodies; Oncology; Original Article; Peripheral neuropathy; Rituximab - administration & dosage; Survival Rate; Targeted cancer therapy; China; Rituximab; Cryoglobulinemia; Bortezomib; Monoclonal gammopathy of unknown significance
• ISSN: 0939-5555; 1432-0584
• DOI: 10.1007/s00277-020-04123-1

To explore the clinical characteristics and outcomes in Chinese patients with type I cryoglobulinemia (CG), we retrospectively analyzed the clinical data, management, and outcomes of 45 patients diagnosed with type I CG in our hospital from January 2015 to March 2019. In our study, all type I CGs were secondary to hematologic diseases, and monoclonal gammopathy of unknown significance was the most common primary disease, accounting for 48.9% ( n = 22). Additionally, B cell non-Hodgkin lymphoma, Waldenström’s macroglobulinemia, and multiple myeloma accounted for 24.4% ( n = 11), 20.0% ( n = 9), and 6.7% ( n = 3), respectively. In patients with type I CG, skin damage was the most common symptom, presenting in 57.8% of the patients, followed by peripheral neuropathy (22.2%) and renal involvement (15.6%). Treatment was initiated in 29 patients (64.4%), and the most common choice was a rituximab-based regimen in 13 patients (44.8%), followed by bortezomib-based regimen in 11 patients (37.9%). Clinical symptoms were significantly improved after treatment, and the clinical remission rate was 86.2%, including 34.5% of complete clinical remission, while the laboratory response rate was 88.9%, including 33.3% of complete response and 55.6% of partial response. The expected 1-year overall survival was 97.8%. In conclusion, for patients with multisystemic involvement, such as skin damage, kidney damage, or peripheral neuropathy, the diagnosis of type I CG should be considered, and the underlying disease needs to be explored. Symptoms and primary diseases should be taken into consideration before choosing initial management.