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This study presents two cases of type II mixed cryoglobulinemia. One case is essential, while the other is presumably associated with hepatitis B virus (HBV) infection. Both patients tested positive for monoclonal IgMk, but negative for MyD88 mutation. They showed resistance to rituximab combined with a glucosteroid regimen, but responded positively to BTK inhibitors. These cases highlight the remarkable effectiveness of BTK inhibitors in treating refractory type II cryoglobulinemia without MyD88 mutation. The first patient achieved rapid complete remission of nephrotic syndrome within one month of starting ibrutinib, along with a significant reduction in cryoglobulin levels and abnormal clonal cells. The second patient had a rapid disappearance of rash within three days and accelerated wound healing within one week of initiating orelabrutinib, accompanied by a reduction in C-reactive protein. However, there was no reduction in cryoglobulin levels during the 12-month follow-up. These findings suggest varied mechanisms of action of BTK inhibitors in type II cryoglobulinemia through different mechanisms. [ABSTRACT FROM AUTHOR]

This study presents two cases of type II mixed cryoglobulinemia. One case is essential, while the other is presumably associated with hepatitis B virus (HBV) infection. Both patients tested positive for monoclonal IgMκ, but negative for MyD88 mutation. They showed resistance to rituximab combined with a glucosteroid regimen, but responded positively to BTK inhibitors. These cases highlight the remarkable effectiveness of BTK inhibitors in treating refractory type II cryoglobulinemia without MyD88 mutation. The first patient achieved rapid complete remission of nephrotic syndrome within one month of starting ibrutinib, along with a significant reduction in cryoglobulin levels and abnormal clonal cells. The second patient had a rapid disappearance of rash within three days and accelerated wound healing within one week of initiating orelabrutinib, accompanied by a reduction in C-reactive protein. However, there was no reduction in cryoglobulin levels during the 12-month follow-up. These findings suggest varied mechanisms of action of BTK inhibitors in type II cryoglobulinemia through different mechanisms., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Xu, Xu, Yuan, Shang, Chen, Liu, Li, Liu, Liu, Wang, Ding, Xie and Hao.)

Purpose: Macular perivenous retinal whitening results from hypoperfusion-induced ischemia of the middle retina that can occur in central retinal vein occlusion (CRVO). We describe an unusual case of recurrent CRVO with macular perivenous retinal whitening and retino--ciliary venous sparing in the setting of 2 prothrombotic diseases, antiphospholipid syndrome and Type II cryoglobulinemia. Methods: A 50-year-old man presented with intermittent loss of vision in his right eye related to a recurrent CRVO. Color photography, optical coherence tomography, and fluorescein angiography were performed and compared with those obtained during a previous CRVO that occurred 6 years earlier in the same eye. Results: On presentation, visual acuity was hand motion in the right eye, 20/30 in the left eye. Funduscopic examination of the right eye showed vascular tortuosity, scattered retinal hemorrhages, and retinal whitening in the macula. Optical coherence tomography showed hyperreflectivity of the middle layers of the retina that correlated with the areas of retinal whitening. A discrete area of retinal sparing was noted in the superonasal macula that, on fluorescein angiography, corresponded to the distribution of a single retino--ciliary vein. A review of retinal imaging obtained during the patient's previous CRVO showed similar but more subtle findings of retino--ciliary sparing. Laboratory testing revealed antiphospholipid syndrome and Type II cryoglobulinemia. As the patient's CRVO progressed and subsequently stabilized after treatment in the following months, this area of venous sparing remained the only functional, nonischemic retinal tissue in his macula. Presumably, this vein possessed privileged and uncompromised blood flow by circumventing the occluded venous circulation. Conclusion: Macular perivenous retinal whitening should be considered in the differential diagnosis of retinal whitening and occurs in CRVO secondary to hypoperfusion-induced middle retinal ischemia. To our knowledge, this case represents the first description of retino--ciliary venous sparing of the retina in CRVO. [ABSTRACT FROM AUTHOR]

Objective: To explore the clinical characteristics and outcome of patients with type Ⅱ cryoglobulinemia in our center. Methods: Clinical data of 61 patients diagnosed with type Ⅱ cryoglobulinemia in Peking Union Medical College hospital from May 2015 to January 2020 were retrospectively analyzed. Results: A total of 61 patients were enrolled in the study, including 26 (42.6%) males, with a median age of 53 (range 28-79) years. The primary diseases identified were hepatitis C virus infection (21.3%) , hepatitis B virus infection (21.3%) , autoimmune diseases (14.8%) , and hematological tumors (11.5%) . Idiopathic cryoglobulinemia patients accounted for 19 cases (31.1%) . The major symptoms presented were purpura, proteinuria, hematuria, renal failure, fever and arthralgia. The median concentration of cryoglobulin level was 215.9 (22.0-17 075.8) g/L, and the IgM-monoclonal component of cryoglobulin was identified in 54 patients (88.5%) . Rheumatoid factor increased in 93.2% of patients. C3 decreased in 57.6% of patients. C4 decreased in 61.0% of patients. Treatment was initiated in 49 patients (80.3%) , and the total clinical remission rate was 75.5%. The expected 3-year overall survival was 89.3%. Conclusion: Type Ⅱ cryoglobulinemia was a systemic disease with multi-organ involvement. Most type Ⅱ CGs were secondary to hepatitis virus infection. Early diagnosis and proper treatment could bring better outcome.