Wang Patrick, Kanda Pushpinder, Wang Yao, Bal Manpartap
Department of Ophthalmology, Queen's University, Kingston, ON, Canada.
Department of Ophthalmology, The University of Ottawa Eye Institute, Ottawa, ON, Canada.
Int J Retina Vitreous. 2023 Mar 22;9(1):16. doi: 10.1186/s40942-022-00423-y.
Cryoglobulinemia, the presence of serum cryoglobulins which are immunoglobulins or complement components that precipitate at temperatures below 37 °C, commonly present with cutaneous manifestations initially, but are more rarely associated with ocular manifestations. To our knowledge, we report the first case of a patient presenting with sequential central retinal artery occlusion (CRAO) associated with cryoglobulinemia.
A 69-year-old female with a history of indolent B-cell lymphoma associated cryoglobulinemia, treated hepatitis B infection and CRAO in the left eye presented with acute vision loss and diffuse retinal whitening with a cherry red spot in her right eye, suggestive of sequential CRAO. Laboratory studies revealed a cryocrit of 55% (normal < 1%), elevated titres of cryoglobulin IgG at 1.98 g/L and cryoglobulin IgM at 3.78 g/L (normal < 0.3 g/L), and elevated kappa free light chain at 283.5 mg/L (normal < 0.06 g/L). Such elevated tires of cryoglobulins in the context of the patient's CRAO raised suspicion of cryoglobulinemia associated CRAO. The patient was promptly referred to rheumatology and oncology and was admitted for treatment including intravenous methylprednisone, rituximab and bendamustine chemotherapy.
We report a patient with a complex medical history presenting with significant vision loss due to a sequential CRAO likely associated with cryoglobulinemia. Although a direct relationship between cryoglobulinemia and CRAO cannot be confirmed in this case, it highlights the importance of considering cryoglobulinemia in high-risk patients with prior history of hematological malignancy or chronic hepatitis infection.
冷球蛋白血症是指血清冷球蛋白的存在，冷球蛋白是在温度低于37°C时沉淀的免疫球蛋白或补体成分，通常最初表现为皮肤症状，但较少与眼部症状相关。据我们所知，我们报告了首例与冷球蛋白血症相关的先后发生中央视网膜动脉阻塞（CRAO）的患者。
一名69岁女性，有惰性B细胞淋巴瘤相关冷球蛋白血症病史，曾接受过乙型肝炎感染治疗，左眼曾发生CRAO，此次因右眼急性视力丧失和视网膜弥漫性变白伴樱桃红斑就诊，提示先后发生CRAO。实验室检查显示冷球蛋白比容为55%（正常<1%），冷球蛋白IgG滴度升高至1.98 g/L，冷球蛋白IgM滴度升高至3.78 g/L（正常<0.3 g/L），游离κ轻链升高至283.5 mg/L（正常<0.06 g/L）。在该患者CRAO的情况下，如此升高的冷球蛋白滴度引发了对冷球蛋白血症相关CRAO的怀疑。该患者被迅速转诊至风湿科和肿瘤科，并入院接受包括静脉注射甲泼尼龙、利妥昔单抗和苯达莫司汀化疗在内的治疗。
我们报告了一名有复杂病史的患者，因先后发生的CRAO可能与冷球蛋白血症相关而出现严重视力丧失。尽管在该病例中不能证实冷球蛋白血症与CRAO之间存在直接关系，但它凸显了在有血液系统恶性肿瘤或慢性肝炎感染既往史的高危患者中考虑冷球蛋白血症的重要性。
