Sudden loss of vision: Purtscher retinopathy in multiple myeloma

Abstract

A 53-year-old man presented with acute, severe loss of vision in both eyes. Three days earlier, he had noticed a painless deterioration of his vision, which had progressed until he could no longer read a newspaper using either eye. On examination, the patient's best corrected visual acuity was 20/100 using both eyes. He had full visual fields by confrontation and the motility of extraocular muscles was normal. No afferent pupillary defect was evident. Examination of the posterior segment showed bilateral edema in the optic nerve head with multiple cotton wool spots and areas of retinal whitening (Purtscher flecken) (Figure 1). Intravenous fluroescein angiography showed severe ischemia of the nasal macular retinal arterioles with extensive secondary late leakage. Based on these findings, we diagnosed Purtscher disease or Purtscher-like angiopathic retinopathy.Pertinent laboratory findings included an elevated erythrocyte sedimentation rate of 83 (normal 0-20) mm/hr, elevated total protein of 92 (normal 60-80) g/L and a serum IgG immunoglobulin of 41.8 (normal 7-17) g/L. These results prompted a skeletal survey that showed small lytic lesions in the proximal humerus, skull and proximal femur. Serum protein electrophoresis showed a monoclonal M spike of 3.39 g, which was eventually determined by immunofixation to be IgG/Kappa type. His peak serum viscosity was elevated at 2.3 (normal 1.4-1.8) centipoises. A bone marrow biopsy was then performed and showed large aggregates of plasma cells comprising approximately 33% of the cell mass. Immunohistochemical stains showed that the plasma cells were kappa light-chain restricted, which was consistent with multiple myeloma, and involved about 70% of the marrow cellularity.Our patient presented with the classic appearance of Purtscher-like retinopathy as an initial manifestation of multiple myeloma. We considered the possibility that hyperviscosity from multiple myeloma was playing a role in our patient's visual loss. Symptoms of hyperviscosity usually appear when a normal serum viscosity climbs to 4-5 centipoises, which often corresponds to a serum IgG level of 40 g/L.1 The patient was treated with three cycles of plasmapheresis, resulting in a drop in the viscosity to a baseline of 1.3 centipoises. He was also given dexamethasone and lenalidomide. After eight months, his vision had improved to 20/400 (right eye) and 20/30 (left eye).Purtscher's retinopathy is rare and most commonly seen after trauma, acute pancreatitis, crush-type injury, long-bone fracture, orthopedic surgery and childbirth.2 Acute retinal signs often persist for at least one month after the initial injury and include Purtscher flecken, cotton wool spots and retinal hemorrhage.2 Spontaneous visual recovery of at least two Snellen lines occurs in half of patients.

Systematic review of Purtscher’s and Purtscher-like retinopathies

“…Other pathogenic processes that may be involved include capillary endothelial damage (supported by late leakage in IVFA 16 ), sudden increase of intracranial pressure (causing precapillary occlusion in lamina crivosa 43,46 ), sudden expansion of retinal veins, 7 or hyperviscosity (such as in multiple myeloma 47 ). A recent study used computer dynamic flow simulations and suggested an additional mechanism: an inner layer retinal posterior pole rheological event that results in vascular endothelial dysregulation.…”

Purtscher-like Retinopathy in Systemic Lupus Erythematosus

“…In addition, capillary endothelial damage and hyperviscosity have also been suggested to play roles in Purtscher-like retinopathy. 11,12 Since vasculitis-associated endothelial damage is not uncommon in SLE, which can result in thrombosis, we speculate that in SLE several mechanisms are involved, including precapillary arteriole occlusion followed by altered retinal microvascular permeability. Our observation suggests that SLE-associated antiphospholipid syndrome (APS) is not likely a main mechanism in Purtscher-like retinopathy, although APS can lead to thrombosis.…”

“…Lupus nephritis was defined as the presence of proteinuria or cellular casts containing red blood cells and hemoglobin. Disease activity was measured using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) developed in 2000, categorized as no activity (SLEDAI ¼ 0), mild activity (SLEDAI ¼ 1-5), moderate activity (SLEDAI ¼ 6-10), high activity (SLEDAI ¼ [11][12][13][14][15][16][17][18][19], and very high activity (SLEDAI > _20). [7][8][9] To evaluate the correlation between Purtscher-like retinopathy and other clinical characteristics of SLE, particularly CNS lupus, 240 control patients were randomly selected from all SLE patients without Purtscher-like retinopathy admitted during the study period, stratified by sex and 2 age categories (<18 years and > _18 years).…”

“…Cases with similar clinical manifestations have been reported in patients with acute pancreatitis, collagen vascular diseases, HELLP syndrome, and renal failure, as well as in patients with multiple myeloma and no relation to trauma. [10][11][12][13] These cases are grouped together as Purtscher-like retinopathy.…”

Reversal of threatening blindness after initiation of eculizumab in Purtscher-like retinopathy secondary to atypical hemolytic uremic syndrome

“…Uncontrolled complement activation has been proposed to play a major role in the pathogenesis of Purtscher-like retinopathy by mechanisms involving endothelial damage and activation of the clotting cascade [ 5 , 6 , 13 , 14 ] and development of leukocyte and platelet aggregates [ 1 , 4 ]. Other proposed mechanisms include intravascular volume surge such as in sudden expansion of retinal veins [ 1 ], hyperviscosity [ 15 ], intracephalic shock waves such as in sudden increase in intracranial pressure with resulting precapillary occlusion at the level of the lamina cribrosa [ 16 ], capillary endothelial damage [ 17 ] and vascular endothelial dysregulation and ensuing endothelin-induced vasculopathy [ 16 ]. Associated systemic entities include acute pancreatitis [ 18 – 25 ], pancreatic adenocarcinoma [ 26 ], systemic lupus erythematosus [ 27 ], renal failure [ 28 , 29 ], amniotic fluid embolization [ 12 ], thrombotic thrombocytopenic purpura [ 30 – 33 ], hemolytic uremic syndrome (HUS) [ 31 , 34 ] and cryoglobulinemia [ 35 – 37 ].…”