Abstract
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.
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Funding
This study is supported by the National Natural Science Foundation of China (Grant No. 81570195, for LJ), Beijing Natural Science Foundation (Grant No. 7182128, for LJ), the Capital Health Research and Development of Special Fund (No. 2018-2-4015, for LJ), the CAMS Innovation Fund for Medical Sciences (Grant No. 2016-12M-1-002, for LJ), and the National Key Research and Development Program of China (Grant No. 2016YFC0901503, for LJ).
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Zhang, Ll., Cao, Xx., Shen, Kn. et al. Clinical characteristics and treatment outcome of type I cryoglobulinemia in Chinese patients: a single-center study of 45 patients. Ann Hematol 99, 1735–1740 (2020). https://doi.org/10.1007/s00277-020-04123-1
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DOI: https://doi.org/10.1007/s00277-020-04123-1