Journal of Rheumatic Diseases Vol. 22, No. 6, December, 2015 http://dx.doi.org/10.4078/jrd.2015.22.6.405 Clinical Image

Cryoglobulinemia with a Digital Gangrene from of Undetermined Significance

Chan Hong Jeon Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, Korea

Type I cryoglobulin is composed of a monoclonal im- years. Concerning the complete count, the white munoglobulin (Ig) and can be found in patients with lym- blood cell count was 9,920/μL and the differential was phoproliferative disorders. Some frequent symptoms of normal. Hemoglobin was 11.5 g/dL; and the platelet type I are skin necrosis and Raynaud’s count was 361,000/μL. Erythrocyte sedimentation rate phenomenon [1]. Monoclonal gammopathy of undeter- was 120 mm/h. Total protein was 10.1 g/dL; and mined significance (MGUS) is a precursor lesion for mul- was 3.4 g/dL with a reversed albumin/ ratio of tiple myeloma [2] and one of the principal causes of cry- 0.5. Serum creatinine was 1.2 mg/dL; and the total cal- oglobulinemia [3]. cium was 8.7 mg/dL. The rheumatoid factor was 17 A 37 year-old-male was admitted with gangrene of the IU/mL; and the antinuclear test was positive but left second toe (Figure 1). The patient was diagnosed was in a low titer of 1:40. The test for anti-neutrophil cy- with primary Raynaud’s syndrome at a tertiary center and toplasmic antibody was negative; and both IgG and IgM has been under observation without treatment for 3 anticardiolipin were negative, and the lupus anticoagulant was also negative. However, cryoglobulin came out as positive. Furthermore, a peripheral blood smear showed marked rouleaux formation and numerous

Figure 1. There was gangrene of the left second toe. Other Figure 2. On peripheral blood smear, after incubation in toes also showed mild cyanotic change on their tips with a room temperature, extracellular pale pink amorphous materi- background of livedo reticularis. als were observed (Wright-Giemsa stain, ×200).

Received：August 4, 2015, Revised：(1st) August 19, 2015, (2nd) August 26, 2015, Accepted：August 26, 2015 Corresponding to：Chan Hong Jeon, Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, 170 Jomaru-ro, Wonmi-gu, Bucheon 14584, Korea. E-mail：[email protected] pISSN: 2093-940X, eISSN: 2233-4718 Copyright ⓒ 2015 by The Korean College of Rheumatology. All rights reserved. This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

405 Chan Hong Jeon

nosed with cryoglobulinemia from light-chain MGUS [2]. The digital gangrene was treated conservatively with anti- biotics and regular dressing. We didn’t plan any addi- tional management for cryoglobulinemia because there was no life-threatening complication, such as hyper- viscosity [1]. As for MGUS, since there was no evidence of or Waldenstrom’s , the patient is under periodic follow-up with serum pro- tein electrophoresis and a complete blood count [4].

CONFLICT OF INTEREST Figure 3. A bone marrow biopsy showed a normal myeloid to erythroid ratio and the usual maturation pattern but multifocal No potential conflict of interest relevant to this article aggregates of plasma cells were observed (Wright-Giemsa was reported. stain, ×400). REFERENCES pale pink amorphous deposits were observed (Figure 2). 1. Harel S, Mohr M, Jahn I, Aucouturier F, Galicier L, Asli B, et Because the serologic tests for and hu- al. Clinico-biological characteristics and treatment of type I man immunodeficiency virus were negative and cry- monoclonal cryoglobulinaemia: a study of 64 cases. Br J oglobulin from paraproteinemia was suspected, protein Haematol 2015;168:671-8. electrophoresis and immunofixation were performed. 2. Dispenzieri A, Katzmann JA, Kyle RA, Larson DR, Melton LJ 3rd, Colby CL, et al. Prevalence and risk of progression of The serum M protein was 1.32 g and monoclonal gamm- light-chain monoclonal gammopathy of undetermined sig- opathy of the IgG-lambda type was noted: the ratio of the nificance: a retrospective population-based cohort study. kappa to lambda free light chain was 0.16. On bone mar- Lancet 2010;375:1721-8. 3. Terrier B, Karras A, Kahn JE, Le Guenno G, Marie I, Benar- row biopsy, myeloid to erythroid ratio was 1.6:1 and ous L, et al. The spectrum of type I cryoglobulinemia vascu- showed the usual maturation pattern with multifocal ag- litis: new insights based on 64 cases. Medicine (Baltimore) gregates of plasma cells (Figure 3). The fraction of plasma 2013;92:61-8. cells was 8.8%. Chromosomal analysis showed no appa- 4. Kyle RA, Buadi F, Rajkumar SV. Management of mono- clonal gammopathy of undetermined significance (MGUS) rent cytogenetic abnormalities. On skeletal survey, no os- and smoldering multiple myeloma (SMM). (Wil- teolytic lesion was found. liston Park) 2011;25:578-86. Based on all the above findings, the patient was diag-

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