Recommended minimum testing
Test . | Baseline . | Every 3 months . | Yearly . |
|---|---|---|---|
| Neurologic | |||
| Detailed neurologic history (numbness, pain, weakness, balance, orthostasis) and examination (including funduscopic exam) | X | X* | X |
| Electrophysiologic study (nerve conduction studies) | X | X* | X |
| Organomegaly/lymphadenopathy/extravascular volume overload | |||
| Physical exam documenting lymphadenopathy, organomegaly, ascites, pleural effusions, and edema | X | X | X |
| Computed tomography of chest/abdomen/pelvis: lymphadenopathy, organomegaly, ascites, and effusions | X | X† | X† |
| Endocrinopathy | |||
| History regarding menstrual and sexual function | X | X | X |
| Testosterone, estradiol, fasting glucose, glycosylated hemoglobin, thyroid stimulating hormone, and serum cortisol (morning and evening) | X | X† | X† |
| Lutenizing hormone, follicle stimulating hormone, prolactin, adrenocorticotropin hormone, parathyroid hormone, and Cortrosyn stimulation test | X‡ | X†‡ | X†‡ |
| Hematologic | |||
| Serum protein electrophoresis and immunofixation | X | X | X |
| Affected quantitative immunoglobulin | X | X | X |
| Complete blood count (hemoglobin, platelet) | X | X | X |
| 24-hour urine total protein, electrophoresis, and immunofixation | X | X | X |
| Bone marrow aspirate and biopsy (test for kappa/lambda by immunohistochemistry or flow cytometry or PCL1) | X | X | |
| Skin | |||
| History regarding skin pigment, thickening and texture, body hair quantity and texture, color of distal extremities, and development of cherry angiomata | X | X | X |
| Physical examination with attention to skin pigment, thickening and texture, body hair quantity and texture, color of distal extremities, and development of cherry angiomata | X | X | X |
| Sclerotic bone lesions | |||
| Skeletal radiographs | X | X | |
| Computed tomography of bones | X§ | ||
| Positron emission tomography | X‡ | X‡ | |
| Bone scan | X‡ | X‡ | |
| Pulmonary function | |||
| Pulmonary function tests including TLC, VC, FEV1, FVC, maximum inspiratory and expiratory pressures, and diffusing capacity of carbon monoxide | X | X† | X† |
| Echocardiography to assess right ventricular systolic and pulmonary artery pressures | X | X† | X† |
Test . | Baseline . | Every 3 months . | Yearly . |
|---|---|---|---|
| Neurologic | |||
| Detailed neurologic history (numbness, pain, weakness, balance, orthostasis) and examination (including funduscopic exam) | X | X* | X |
| Electrophysiologic study (nerve conduction studies) | X | X* | X |
| Organomegaly/lymphadenopathy/extravascular volume overload | |||
| Physical exam documenting lymphadenopathy, organomegaly, ascites, pleural effusions, and edema | X | X | X |
| Computed tomography of chest/abdomen/pelvis: lymphadenopathy, organomegaly, ascites, and effusions | X | X† | X† |
| Endocrinopathy | |||
| History regarding menstrual and sexual function | X | X | X |
| Testosterone, estradiol, fasting glucose, glycosylated hemoglobin, thyroid stimulating hormone, and serum cortisol (morning and evening) | X | X† | X† |
| Lutenizing hormone, follicle stimulating hormone, prolactin, adrenocorticotropin hormone, parathyroid hormone, and Cortrosyn stimulation test | X‡ | X†‡ | X†‡ |
| Hematologic | |||
| Serum protein electrophoresis and immunofixation | X | X | X |
| Affected quantitative immunoglobulin | X | X | X |
| Complete blood count (hemoglobin, platelet) | X | X | X |
| 24-hour urine total protein, electrophoresis, and immunofixation | X | X | X |
| Bone marrow aspirate and biopsy (test for kappa/lambda by immunohistochemistry or flow cytometry or PCL1) | X | X | |
| Skin | |||
| History regarding skin pigment, thickening and texture, body hair quantity and texture, color of distal extremities, and development of cherry angiomata | X | X | X |
| Physical examination with attention to skin pigment, thickening and texture, body hair quantity and texture, color of distal extremities, and development of cherry angiomata | X | X | X |
| Sclerotic bone lesions | |||
| Skeletal radiographs | X | X | |
| Computed tomography of bones | X§ | ||
| Positron emission tomography | X‡ | X‡ | |
| Bone scan | X‡ | X‡ | |
| Pulmonary function | |||
| Pulmonary function tests including TLC, VC, FEV1, FVC, maximum inspiratory and expiratory pressures, and diffusing capacity of carbon monoxide | X | X† | X† |
| Echocardiography to assess right ventricular systolic and pulmonary artery pressures | X | X† | X† |