Table 3

Morbidities mediated by the IgM monoclonal protein in patients with WM

Properties of IgM monoclonal proteinDiagnostic conditionClinical manifestations
Pentameric Structure Hyperviscosity Headaches, blurred vision, epistaxis, retinal hemorrhages, leg cramps, impaired mentation, intracranial hemorrhage. 
Precipitation on cooling Cryoglobulinemia (type I) Raynaud-like phenomena, acrocyanosis, ulcers, purpura, cold urticaria. 
Auto-antibody 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. 
Auto-antibody activity to IgG Cryoglobulinemia (type II) Purpura, arthralgias, renal failure, sensorimotor neuropathies. 
Auto-antibody activity to red blood cell antigens Cold agglutinins Hemolytic anemia, Raynaud’s phenomenom, acrocyanosis, livedo reticularis. 
Tissue deposition as amorphous aggregates Organ dysfunction Skin: bullous skin disease, papules, Schnitzler’s syndrome. 
GI: diarrhea, malabsorption, bleeding. 
Kidney: proteinuria, renal failure (light chain component). 
Tissue deposition as amyloid fibrils (light chain component most common) Organ dysfunction Fatigue, weight loss, edema, hepatomegaly, macroglossia, organ dysfunction of involved organs: heart, kidney, liver, peripheral sensory and autonomic nerves. 
Properties of IgM monoclonal proteinDiagnostic conditionClinical manifestations
Pentameric Structure Hyperviscosity Headaches, blurred vision, epistaxis, retinal hemorrhages, leg cramps, impaired mentation, intracranial hemorrhage. 
Precipitation on cooling Cryoglobulinemia (type I) Raynaud-like phenomena, acrocyanosis, ulcers, purpura, cold urticaria. 
Auto-antibody 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. 
Auto-antibody activity to IgG Cryoglobulinemia (type II) Purpura, arthralgias, renal failure, sensorimotor neuropathies. 
Auto-antibody activity to red blood cell antigens Cold agglutinins Hemolytic anemia, Raynaud’s phenomenom, acrocyanosis, livedo reticularis. 
Tissue deposition as amorphous aggregates Organ dysfunction Skin: bullous skin disease, papules, Schnitzler’s syndrome. 
GI: diarrhea, malabsorption, bleeding. 
Kidney: proteinuria, renal failure (light chain component). 
Tissue deposition as amyloid fibrils (light chain component most common) Organ dysfunction Fatigue, weight loss, edema, hepatomegaly, macroglossia, organ dysfunction of involved organs: heart, kidney, liver, peripheral sensory and autonomic nerves. 
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