A review of systems and their implications in the workup of patients with WM
| Symptom . | Implications . | Action . |
|---|---|---|
| Energy level/changes in activities of daily life | Anemia, fatigue without anemia | Evaluate for anemia, underlying etiology, including iron deficiency, hemolytic anemia (warm and cold antibodies); consider amyloidosis; exclude other medical causes of anemia |
| Constitutional symptoms | Tumor-related fever, chills, night sweats | |
| Recurrent sinus and bronchial infections | Chronic sinusitis, usually on the basis of IgA and IgG hypogammaglobulinemia | Antibiotic support, if refractory to multiple antibiotic courses, hospitalizations, or life-threatening, strongly consider intravenous immunoglobulin replacement |
| Headaches, blurry vision or visual loss, confusional episodes, epistaxis | Hyperviscosity | Funduscopic examination for hyperviscosity-related changes, obtain serum IgM and viscosity levels; consider emergency plasmapheresis for symptomatic hyperviscosity; strongly consider in patients with serum viscosity > 4.0 cp given high risk of hyperviscosity-related events |
| Easy bruising, bleeding diathesis | Thrombocytopenia, acquired von Willebrand disorder | Complete blood count, evaluate for immune thrombocytopenia or hypersplenism if indicated; consider evaluation for von Willebrand disorder; consider amyloidosis |
| Progressive symmetric numbness, tingling, burning, pain in feet and hands; unsteady gait, deficits in motor function | IgM-related neuropathy or myopathy; amyloidosis | Obtain anti-MAG, anti-GM1, anti-sulfatidyl IgM antibody studies; if myopathy is present, consider obtaining antidecorin antibodies; consider obtaining fat pad biopsy and stain for amyloid; consider EMG studies |
| Raynaud-like symptoms, acrocyanosis, ulcerations on extremities | Cryoglobulinemia, cold agglutinemia | Obtain cryoglobulins, cold agglutinins, in patients suspected of having cryoglobulins, all studies, including quantitative immunoglobulins, should be obtained in a warm bath to avoid cryoprecipitation and false lowering of serum IgM levels |
| Diarrhea, gastrointestinal cramping | Malabsorption, secondary to amyloidosis, IgM deposition, tumor involvement; rarely, autonomic neuropathy on basis of autoantibody or amyloidosis | Endoscopy to evaluate small bowel, biopsy to evaluate for amyloidosis, IgM deposition, tumor involvement |
| Hearing loss | Hyperviscosity, sensorineural hearing loss, amyloid or tumor deposition, thrombus formation | Consider evaluation for anti-Hu and anti-hsp 70 antibodies, MRI to assess for amyloidoma, tumor deposition; evaluate for hyperviscosity syndrome (as earlier in Table 3); assess for IgM antiphospholipid antibodies |
| Thrombotic events | Antiphospholipid antibody syndrome | Assess for IgM antiphospholipid antibodies |
| Urticaria, papules, dermatitis | Schnitzler syndrome (nonpruritic urticaria), IgM or tumor cell infiltration, amyloid deposition | Skin biopsy, histologic examination for tumor cell infiltration, stain for IgM, Congo red for amyloid |
| Symptom . | Implications . | Action . |
|---|---|---|
| Energy level/changes in activities of daily life | Anemia, fatigue without anemia | Evaluate for anemia, underlying etiology, including iron deficiency, hemolytic anemia (warm and cold antibodies); consider amyloidosis; exclude other medical causes of anemia |
| Constitutional symptoms | Tumor-related fever, chills, night sweats | |
| Recurrent sinus and bronchial infections | Chronic sinusitis, usually on the basis of IgA and IgG hypogammaglobulinemia | Antibiotic support, if refractory to multiple antibiotic courses, hospitalizations, or life-threatening, strongly consider intravenous immunoglobulin replacement |
| Headaches, blurry vision or visual loss, confusional episodes, epistaxis | Hyperviscosity | Funduscopic examination for hyperviscosity-related changes, obtain serum IgM and viscosity levels; consider emergency plasmapheresis for symptomatic hyperviscosity; strongly consider in patients with serum viscosity > 4.0 cp given high risk of hyperviscosity-related events |
| Easy bruising, bleeding diathesis | Thrombocytopenia, acquired von Willebrand disorder | Complete blood count, evaluate for immune thrombocytopenia or hypersplenism if indicated; consider evaluation for von Willebrand disorder; consider amyloidosis |
| Progressive symmetric numbness, tingling, burning, pain in feet and hands; unsteady gait, deficits in motor function | IgM-related neuropathy or myopathy; amyloidosis | Obtain anti-MAG, anti-GM1, anti-sulfatidyl IgM antibody studies; if myopathy is present, consider obtaining antidecorin antibodies; consider obtaining fat pad biopsy and stain for amyloid; consider EMG studies |
| Raynaud-like symptoms, acrocyanosis, ulcerations on extremities | Cryoglobulinemia, cold agglutinemia | Obtain cryoglobulins, cold agglutinins, in patients suspected of having cryoglobulins, all studies, including quantitative immunoglobulins, should be obtained in a warm bath to avoid cryoprecipitation and false lowering of serum IgM levels |
| Diarrhea, gastrointestinal cramping | Malabsorption, secondary to amyloidosis, IgM deposition, tumor involvement; rarely, autonomic neuropathy on basis of autoantibody or amyloidosis | Endoscopy to evaluate small bowel, biopsy to evaluate for amyloidosis, IgM deposition, tumor involvement |
| Hearing loss | Hyperviscosity, sensorineural hearing loss, amyloid or tumor deposition, thrombus formation | Consider evaluation for anti-Hu and anti-hsp 70 antibodies, MRI to assess for amyloidoma, tumor deposition; evaluate for hyperviscosity syndrome (as earlier in Table 3); assess for IgM antiphospholipid antibodies |
| Thrombotic events | Antiphospholipid antibody syndrome | Assess for IgM antiphospholipid antibodies |
| Urticaria, papules, dermatitis | Schnitzler syndrome (nonpruritic urticaria), IgM or tumor cell infiltration, amyloid deposition | Skin biopsy, histologic examination for tumor cell infiltration, stain for IgM, Congo red for amyloid |