Multiple myeloma, also known as malignant plasmacytoma, plasma cell myeloma, and myelomatosis, is a disseminated malignant neoplasm of marrow plasma cells that infiltrates bone to produce osteolytic lesions throughout the skeleton (flat bones, vertebrae, skull, pelvis, ribs). In late stages, it infiltrates the body organs (liver, spleen, lymph nodes, lungs, adrenal glands, kidneys, skin, and GI tract). Prognosis is usually poor because diagnosis is commonly made after the disease has already infiltrated the vertebrae, pelvis, skull, ribs, clavicles, and sternum. By then, skeletal destruction is widespread and, without treatment, leads to vertebral collapse; 52% of patients die within 3 months of diagnosis, 90% within 2 years. Early diagnosis and treatment prolong the lives of many patients by 3 to 5 years. Death usually follows complications, such as infection, renal failure, hematologic imbalance, fractures, hypercalcemia, hyperuricemia, or dehydration.
Multiple myeloma mainly affects older adults, but its causes and other risk factors are unknown. It's rare, with a yearly incidence of 3 new cases in 100,000 people.
The earliest indication of multiple myeloma is severe, constant back and rib pain that increases with exercise and may be worse at night. Arthritic symptoms may also occur: achiness, joint swelling, and tenderness, possibly from vertebral compression. Other effects include fever, malaise, slight evidence of peripheral neuropathy (such as peripheral paresthesia), and pathologic fractures. As multiple myeloma progresses, symptoms of vertebral compression may become acute, accompanied by anemia, weight loss, thoracic deformities (ballooning), and loss of body height (5 " [12.7 cm] or more) due to vertebral collapse. Renal complications such as pyelonephritis (caused by tubular damage from large amounts of Bence Jones protein, hypercalcemia, and hyperuricemia) may occur. Severe, recurrent infection such as pneumonia may follow damage to nerves associated with respiratory function.
❑Bone marrow aspiration and biopsy detects myelomatosis cells (abnormal number of immature plasma cells).
❑Urine studies may show Bence Jones protein and hypercalciuria. Absence of Bence Jones protein doesn't rule out multiple myeloma; however, its presence almost invariably confirms the disease. (See Bence Jones protein.)
❑Complete blood count shows moderate or severe anemia. The differential may show 40% to 50% lymphocytes but seldom more than 3% plasma cells. Rouleau formation (usually the first clue) seen on differential smear results from elevation of the red cell sedimentation rate.
❑Serum electrophoresis shows elevated globulin spike that's electrophoretically and immunologically abnormal.
❑ X-rays during early stages may show only diffuse osteoporosis. Eventually, they show multiple, sharply circumscribed osteolytic (punched out) lesions, particularly on the skull, pelvis, and spine — the characteristic lesions of multiple myeloma.
❑ Excretory urography can assess renal involvement. To avoid precipitation of Bence Jones protein, iothalamate or diatrizoate is used instead of the usual contrast medium and, although oral fluid restriction is usually the standard procedure before excretory urography, patients with multiple myeloma receive large quantities of fluid, generally orally but sometimes I.V., before excretory urography is done.
Long-term treatment of multiple myeloma consists mainly of chemotherapy to suppress plasma cell growth and control pain. Commonly used combinations include cyclophosphamide, doxorubicin, and prednisone as well as carmustine, doxorubicin, and prednisone. Adjuvant local radiation reduces acute lesions, such as collapsed vertebrae, and relieves localized pain. Other treatments usually include a melphalan-prednisone combination in high intermittent doses or low continuous daily doses and analgesics for pain. Oral thalidomide (with or without steroids) has shown promise in relapsed multiple myeloma, and velcade, a proteasome inhibitor, is a newer agent that has shown promise in myeloma treatment. For spinal cord compression, the patient may require a laminectomy; for renal complications, dialysis.
Clinical trials are currently under way to evaluate the role of biological response modifiers (interferon) in the management of multiple myeloma. In addition, high-dose chemotherapy and radiotherapy with peripheral stem cell rescue have been helpful in select cases.
Because the patient may have bone demineralization and may lose large amounts of calcium into blood and urine, he's a prime candidate for renal calculi, nephrocalcinosis and, eventually, renal failure due to hypercalcemia. Hypercalcemia is managed with hydration, diuretics, cortico-steroids, oral phosphate, mithramycin I.V., or bisphosphonates I.V. (such as pamidronate or zoledronic acid) to decrease serum calcium levels.
❑Push fluids; encourage the patient to drink 101.5 to 135 oz (3,000 to 4,000 ml) of fluids daily, particularly before his excretory urography. Monitor fluid intake and output (daily output shouldn't be less than 1,500 ml).
❑Encourage the patient to walk (immobilization increases bone demineralization and vulnerability to pneumonia), and give analgesics, as ordered, to lessen pain. Never allow the patient to walk unaccompanied; make sure that he uses a walker or other supportive aid to prevent falls. Because the patient is particularly vulnerable to pathologic fractures, he may be fearful. Give reassurance, and allow him to move at his own pace.
❑Prevent complications by watching for fever or malaise, which may signal the onset of infection, and for signs of other problems, such as severe anemia and fractures. If the patient is bedridden, change his position every 2 hours. Give passive range-of-motion and deep-breathing exercises. When he can tolerate them, promote active exercises.
❑If the patient is taking melphalan (a phenylalanine derivative of nitrogen mustard that depresses bone marrow), make sure his blood count (platelet and white blood cell) is taken before each treatment. If he's taking prednisone, watch closely for infection because this drug commonly masks it.
❑Whenever possible, get the patient out of bed within 24 hours after laminectomy. Check for hemorrhage, motor or sensory deficits, and loss of bowel or bladder function. Position the patient as ordered, maintain alignment, and logroll when turning.
❑Provide much-needed emotional support for the patient and his family, as they're likely to be anxious. Help relieve their anxiety by truthfully informing them about diagnostic tests (including painful procedures, such as bone marrow aspiration and biopsy), treatment, and prognosis. If needed, refer them to an appropriate community resource for additional support.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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Title: Professional Guide to Diseases (Eighth Edition)
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2005
ISBN: 1-58255-370-X
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