Morbidities mediated by the monoclonal IgM protein in WM
Property of IgM monoclonal protein . | Diagnostic condition . | Clinical manifestations . |
---|---|---|
Pentameric structure | Hyperviscosity | Headaches, blurred vision, epistaxis, retinal hemorrhages, leg cramps, impaired mentation, intracranial hemorrhage |
Precipitation on cooling | Cryoglobulinemia (type I) | Raynaud phenomenon, acrocyanosis, ulcers, purpura, cold urticaria |
Autoantibody activity to myelin-associated glycoprotein (MAG), ganglioside M1 (GM1), sulfatide moieties on peripheral nerve sheaths | Peripheral neuropathies | Sensorimotor neuropathies, painful neuropathies, ataxic gait, bilateral foot drop |
Autoantibody activity to IgG | Cryoglobulinemia (type II) | Purpura, arthralgias, renal failure, sensorimotor neuropathies |
Autoantibody activity to red blood cell antigens | Cold agglutinins | Hemolytic anemia, Raynaud phenomenon, acrocyanosis, livedo reticularis |
Tissue deposition as amorphous aggregates | Organ dysfunction | Skin: bullous skin disease, papules, Schnitzler syndrome; GI: diarrhea, malabsorption, bleeding; kidney: proteinuria, renal failure (light chain component) |
Tissue deposition as amyloid fibrils (light chain component most commonly) | Organ dysfunction | Fatigue, weight loss, edema, hepatomegaly, macroglossia, organ dysfunction of involved organs: heart, kidney, liver, and peripheral sensory and autonomic nerves |
Property of IgM monoclonal protein . | Diagnostic condition . | Clinical manifestations . |
---|---|---|
Pentameric structure | Hyperviscosity | Headaches, blurred vision, epistaxis, retinal hemorrhages, leg cramps, impaired mentation, intracranial hemorrhage |
Precipitation on cooling | Cryoglobulinemia (type I) | Raynaud phenomenon, acrocyanosis, ulcers, purpura, cold urticaria |
Autoantibody activity to myelin-associated glycoprotein (MAG), ganglioside M1 (GM1), sulfatide moieties on peripheral nerve sheaths | Peripheral neuropathies | Sensorimotor neuropathies, painful neuropathies, ataxic gait, bilateral foot drop |
Autoantibody activity to IgG | Cryoglobulinemia (type II) | Purpura, arthralgias, renal failure, sensorimotor neuropathies |
Autoantibody activity to red blood cell antigens | Cold agglutinins | Hemolytic anemia, Raynaud phenomenon, acrocyanosis, livedo reticularis |
Tissue deposition as amorphous aggregates | Organ dysfunction | Skin: bullous skin disease, papules, Schnitzler syndrome; GI: diarrhea, malabsorption, bleeding; kidney: proteinuria, renal failure (light chain component) |
Tissue deposition as amyloid fibrils (light chain component most commonly) | Organ dysfunction | Fatigue, weight loss, edema, hepatomegaly, macroglossia, organ dysfunction of involved organs: heart, kidney, liver, and peripheral sensory and autonomic nerves |