Review of systems and considerations for their use in working up patients with WM
| Symptom/complaint . | 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. Hepcidin-related iron deficiency is common in WM. Check iron saturation levels.31 Patients with low iron saturation levels < 10-12% may benefit with parenteral iron infusions32 |
| Constitutional complaints | 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 patient is refractory to multiple antibiotic courses, required hospitalizations, or infections were life threatening, strongly consider IVIG replacement |
| Headaches, blurry vision or visual loss, confusional episodes, epistaxis | Hyperviscosity | Funduscopic examination for hyperviscosity-related changes, obtain serum IgM and serum viscosity levels.62,63 Serum viscosity levels may be slow to be resulted, not reproducible or lack correlation to serum IgM levels. Consider emergent plasmapheresis for symptomatic hyperviscosity |
| Easy bruising, bleeding diathesis | Thrombocytopenia, acquired von Willebrand disorder (VWD) | Complete blood count, evaluate for immune thrombocytopenia or hypersplenism if indicated; consider evaluation for VWD; consider amyloidosis |
| Progressive symmetrical numbness, tingling, burning, pain feet and hands. Unsteady gait, deficits in motor function | IgM-related neuropathy or myopathy; amyloidosis | Obtain anti-MAG, and if negative then anti-GM1, anti-sulfatide IgM antibody studies. Consider obtaining fat pad biopsy and Congo-red stain to evaluate for amyloidosis. Obtain EMG studies and neurology consult |
| 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 auto-antibody 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 | Evaluate for anti-hu antibody, MRI to assess for amyloidoma, tumor deposition. Evaluate for hyperviscosity syndrome (as above). Assess for IgM anti-phsopholipid antibodies |
| Thrombotic events | Antiphospholipid antibody syndrome | Assess for IgM anti-phospholipid antibodies |
| Urticaria, papules, dermatitis | Schnitzler’s syndrome (nonpruritic urticaria), IgM or tumor cell infiltration, amyloid deposition | Skin biopsy, histological examination for tumor cell infiltration, stain for IgM, Congo-red staining for amyloid |
| Symptom/complaint . | 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. Hepcidin-related iron deficiency is common in WM. Check iron saturation levels.31 Patients with low iron saturation levels < 10-12% may benefit with parenteral iron infusions32 |
| Constitutional complaints | 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 patient is refractory to multiple antibiotic courses, required hospitalizations, or infections were life threatening, strongly consider IVIG replacement |
| Headaches, blurry vision or visual loss, confusional episodes, epistaxis | Hyperviscosity | Funduscopic examination for hyperviscosity-related changes, obtain serum IgM and serum viscosity levels.62,63 Serum viscosity levels may be slow to be resulted, not reproducible or lack correlation to serum IgM levels. Consider emergent plasmapheresis for symptomatic hyperviscosity |
| Easy bruising, bleeding diathesis | Thrombocytopenia, acquired von Willebrand disorder (VWD) | Complete blood count, evaluate for immune thrombocytopenia or hypersplenism if indicated; consider evaluation for VWD; consider amyloidosis |
| Progressive symmetrical numbness, tingling, burning, pain feet and hands. Unsteady gait, deficits in motor function | IgM-related neuropathy or myopathy; amyloidosis | Obtain anti-MAG, and if negative then anti-GM1, anti-sulfatide IgM antibody studies. Consider obtaining fat pad biopsy and Congo-red stain to evaluate for amyloidosis. Obtain EMG studies and neurology consult |
| 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 auto-antibody 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 | Evaluate for anti-hu antibody, MRI to assess for amyloidoma, tumor deposition. Evaluate for hyperviscosity syndrome (as above). Assess for IgM anti-phsopholipid antibodies |
| Thrombotic events | Antiphospholipid antibody syndrome | Assess for IgM anti-phospholipid antibodies |
| Urticaria, papules, dermatitis | Schnitzler’s syndrome (nonpruritic urticaria), IgM or tumor cell infiltration, amyloid deposition | Skin biopsy, histological examination for tumor cell infiltration, stain for IgM, Congo-red staining for amyloid |