Find information on thousands of medical conditions and prescription drugs.

Multiple myeloma

Multiple myeloma (also known as MM, myeloma, plasma cell myeloma, or as Kahler's disease after Otto Kahler) is a presently incurable hematological malignancy of plasma cells, the cells of the immune system that produce antibodies. Its prognosis despite therapy is generally poor, and treatment may involve chemotherapy and stem cell transplant. more...

Mac Ardle disease
Macular degeneration
Mad cow disease
Maghazaji syndrome
Mal de debarquement
Malignant hyperthermia
Mallory-Weiss syndrome
Malouf syndrome
Marburg fever
Marfan syndrome
MASA syndrome
Mast cell disease
MAT deficiency
Maturity onset diabetes...
McArdle disease
McCune-Albright syndrome
Mediterranean fever
Megaloblastic anemia
Meleda Disease
Meniere's disease
Mental retardation
Mercury (element)
Metabolic acidosis
Metabolic disorder
Methylmalonic acidemia
Microscopic polyangiitis
Microtia, meatal atresia...
Miller-Dieker syndrome
Mitochondrial Diseases
Mitral valve prolapse
Mobius syndrome
MODY syndrome
Moebius syndrome
Molluscum contagiosum
MOMO syndrome
Mondini Dysplasia
Mondor's disease
Monoclonal gammopathy of...
Morquio syndrome
Motor neuron disease
Moyamoya disease
MPO deficiency
Mullerian agenesis
Multiple chemical...
Multiple endocrine...
Multiple hereditary...
Multiple myeloma
Multiple organ failure
Multiple sclerosis
Multiple system atrophy
Muscular dystrophy
Myalgic encephalomyelitis
Myasthenia gravis
Mycosis fungoides
Myelodysplastic syndromes
Myeloperoxidase deficiency
Myoadenylate deaminase...
Myositis ossificans

Signs and symptoms

Symptoms can include: malaise, bone pain, anemia, infections (due to decreased immunity) and fractures (due to breakdown of bone by malignant cells, as well as a tendency to brittle bones). Often, the diagnosis of multiple myeloma is made incidentally during routine blood tests for other conditions. The antibody that is produced in excess may cause specific medical problems, such as amyloid, acute renal failure and chronic renal failure, polyneuropathy and other disorders.

A mnemonic doctors use to remember the common tetrad of multiple myeloma is CRAB - C = Calcium (elevated), R =Renal failure, A = Anemia, B = Bone lesions.



The existence of unexplained anemia, kidney dysfunction, a high erythrocyte sedimentation rate (ESR) and a high serum protein (especially raised globulin) may suggest further testing. A doctor will then order protein electrophoresis of the blood and urine, on which a paraprotein (monoclonal protein, or M protein) band can be noticed. A type of paraprotein is the Bence Jones protein which is paraprotein composed of free light chains (see below). Quantitative measurements of the paraprotein are necessary to determine the severity of the disease. The paraprotein is a deviant immunoglobulin produced by the tumor clone. Very rarely, the myeloma is nonsecretory (not producing immunoglobulins).

In theory, myeloma can produce all classes of immunoglobulin, but IgD, IgM and IgE myeloma are very rare compared to IgG and IgA. In addition, light and heavy chains (the building blocks of antibodies) may be secreted in isolation: κ- or λ-light chains or any of the five types of heavy chains (α-, γ-, δ-, ε- or μ-heavy chains).

Additional findings are: a raised calcium (when myeloma cells are breaking down bone, releasing calcium into the bloodstream) and decreased renal function, which may be due to paraprotein deposition in the kidney).


The workup of suspected multiple myeloma includes a skeletal survey. This is a series of X-rays of the skull, axial skeleton and proximal long bones. Myeloma deposits appear as "lytic lesions" (with local disappearance of normal bone due to resorption), and on the skull X-ray as "punched-out lesions". A CT scan may be performed to measure the size of soft tissue plasmacytomas.

A bone marrow biopsy is usually performed to estimate the percentage of bone marrow occupied by plasma cells. This percentage is used in the diagnostic criteria for myeloma. Immunohistochemistry (staining particular cell types using antibodies against surface proteins) can detect plasma cells which express immunoglobulin in the cytoplasm but usually not on the surface; myeloma cells are typically CD56, CD138 positive and CD19 negative. Cytogenetics may also performed in myeloma for prognostic purposes.


[List your site here Free!]

Multiple myeloma in a patient with hoarseness, dysphagia, aspiration, and cervical lymphadenopathy
From Ear, Nose & Throat Journal, 4/1/04 by James K. Fortson


Multiple myeloma, which primarily affects the elderly, is rare in the head and neck. We report the case of a 71-year-old man who came to us with hoarseness, dysphagia, intermittent aspiration, and cervical lymphadenopathy. Our work-up included laboratory tests, radiographic examinations, analysis of bone marrow aspiration, and histopathologic evaluations. Cervical lymph node biopsy confirmed a diagnosis of multiple myeloma. Despite treatment with chemotherapy and radiation, the patient died of his disease 6 months later.


Multiple myeloma, a rare malignancy of plasma cells, was first described in 1950 as an abnormal production of immunoglobulins. (1) It accounts for approximately 10 to 15% of all hematologic malignancies, 1% of all malignant neoplasms in whites and 2% in blacks, and 2% of deaths from all malignancies. (1-3)

Multiple myeloma represents fewer than 2% of all head and neck cancers. (1-4) When it has been reported in the head and neck, it involved the skull base, orbit, scalp, forehead, paranasal sinuses, mandible, palate, tongue, larynx, and thyroid gland. (2-5)

The etiology of multiple myeloma is unclear. Race, genetics, geography, and exposure to radiation and occupational substances have all been implicated in its development. (1-3) Occupational risk factors include exposure to wood, metal, rubber, textiles, and petroleum. (1) Population-based studies have shown that blacks experience both a higher incidence of this malignancy and higher mortality from it. (1) Multiple myeloma is rarely seen in patients younger than 40 years; its peak incidence occurs in the sixth and seventh decades of life. (1,3)

The clinical manifestations of multiple myeloma include bone pain, anemia, hypercalcemia, and renal disease. Amyloidosis has also been seen. (3) Initial symptoms in the head and neck may include headache, confusion, irritability, diplopia, blurred vision, hearing loss, vertigo, hoarseness, dysphagia, and cranial nerve deficits. (1,3)

Case report

A 71-year-old black man came to us with a 3-month history of progressive hoarseness and dysphagia and intermittent aspiration. He denied using any medications, alcohol, or tobacco. He reported that he often applied a commercially available topical analgesic cream to relieve persistent left shoulder pain. His medical and surgical histories were unremarkable.

Physical examination revealed that he was a well-developed, well-nourished man with mild hoarseness. Findings on head, eyes, and ENT examination were normal except for the fact that his tongue deviated to the left upon protrusion and his gag reflex was diminished on the left. Indirect laryngoscopy revealed left vocal fold paralysis. Cervical adenopathy of the left posterior triangle was noted.

Laboratory testing revealed moderate normochromic, normocytic anemia (hemoglobin: 10.5 g/dl; mean corpuscular volume: 81.9 [micro][m.sup.3]; mean corpuscular hemoglobin: 26.9 g/dl; and white blood cell count: 1 1.4 x [10.sup.3]/[micro]l). His platelet count was normal. The differential count revealed 85% polymorphonuclear leukocytes, 9% lymphocytes, 3% monocytes, 3% bands, and 4+ rouleaux forms. Bence Jones proteinuria was absent. Subsequent urine immunoelectrophoresis revealed the presence of M subclass proteinuria.

Findings on fine-needle aspiration biopsy (FNAB) of the neck mass were equivocal, demonstrating only abnormal cells. Computed tomography suggested fullness at the left base of the tongue. Magnetic resonance imaging (MRI) revealed the presence of a mass in the left skull base that involved the clivus and the temporal bone (figure 1, A). Its epicenter was located in the area of the jugular foramen. The mass was approximately 5 cm wide, and its anteroposterior diameter was 2.5 cm. The lesion obscured the region of the left hypoglossal canal and abutted the left carotid artery. The mass indented the left cerebellar hemisphere posteriorly and extended laterally to the mastoid air cells (figure 1, B). The differential diagnosis suggested by these findings included meningioma, plasmacytoma, lymphoma, and metastatic carcinoma.


Direct laryngoscopy, esophagoscopy, and bronchoscopy were performed with the patient under general anesthesia. Random biopsies were taken from the nasopharynx, base of the tongue, tonsillar fossa, and pyriform sinus. The left vocal fold paralysis was noted, as well. A posterior cervical lymph node was excised and sent for frozen-section analysis. Examination of the specimen revealed sheets of atypical plasma cells with foci of normal lymphocytes (figure 2, A). Immunoperoxidase stains of the plasma cells for B- or T-cell markers were negative. Kappa and lambda preparations were inadequate for interpretation.


Bone marrow aspiration cytology revealed normal cellularity, with myeloid, erythroid, and megakaryocyte cell lines present. There were areas of patchy plasmacytosis, with increased numbers of plasma cells that had abnormal shapes and abnormal nuclei (figure 2, B). Serum electrophoresis demonstrated a monoclonal spike in the gamma globulin region. The findings of this spike and the bone marrow plasmacytosis strongly suggested a diagnosis of multiple myeloma. Except for the skull and the temporal bone, the results of a bone scan were negative.

The patient was started on six cycles of vincristine, carmustine, and cyclophosphamide on the first day of treatment. After 12 weeks, he was placed on six cycles of maintenance treatment with 1 mg/kg of melphalan for 10 days and 60 mg of prednisone tapered over 5 days. He also received 4,000 cGy of radiation therapy to the neck over a 4-week period. Two months after the start of treatment, bone marrow aspiration revealed minimal change. Vincristine, doxorubicin, and dexamethasone were then given over a 5-week period. However, the patient's symptoms progressed, and he died 6 months after the initial diagnosis.


Multiple myeloma is characterized by a proliferation of plasma cells that produce M proteins and an overproduction of myeloma protein and immunoglobulin. (1,3) A thyroid mass may arise as an enlarging neck mass with cervical lymph node involvement. Thyroid masses and cervical lymphadenopathy can be evaluated by FNAB. FNAB is also indicated for lesions in the nasal cavity, oral cavity, oropharynx, and larynx. Open biopsy may be indicated for neck and thyroid masses when FNAB is nondiagnostic. The differential diagnosis includes glomus jugular tumors, cerebellopontine angle tumor, malignant melanoma, esthesioneuroblastoma, undifferentiated carcinoma, lymphoma, and plasma cell granuloma.

The head and neck work-up includes a thorough examination, laboratory studies, and radiographic evaluation. MRI is useful in detecting dissemination of disease in the temporal bone and skull base.

The treatment of choice for previously untreated multiple myeloma is chemotherapy. The gold standard for chemotherapy is melphalan and prednisone. Even so, the long-term prognosis remains dismal, although improved survival rates have been observed with this combination. (1,2) Vincristine, doxorubicin, and dexamethasone are used to treat resistant disease.

Other treatment modalities include interferon, radiation therapy, and bone marrow transplantation. Clinical trials of interferon have demonstrated that it is effective in decreasing the production of myeloma plasma cells. (1,2) Improved response rates have been reported when interferon is combined with chemotherapy. (1,2) Radiation therapy is primarily used for palliation since multiple myeloma is not a radiosensitive disease. (1) Bone marrow transplantation has been useful in selected cases, but its application is limited. Surgery plays a limited role in multiple myeloma, although it may be useful as a means of cytoreduction, managing airway obstructions, and obtaining cervical lymph node biopsies. (5)

The prognosis of multiple myeloma is poor, and most patients die within 2 years of their diagnosis; 3-year survival is only about 10%. (1,2,5) The average survival for patients with systemic multiple myeloma is less than 1 year. (1,2,5)


(1.) Sheridan CA. Multiple myeloma. Semin Oncol Nurs 1996;12: 59-69.

(2.) Nofsinger YC, Mirza N, Rowan PT, et al. Head and neck manifestations of plasma cell neoplasms. Laryngoscope 1997:107:741-6.

(3.) Kearns GJ, Pogrel MA, Hanks DK, Macintosh RB. Simultaneous masses of the palate and body of mandible. J Oral Maxillofac Surg 1993;51:783-6.

(4.) Schiller VL, Deutsch AL, Turner RR. Multiple myeloma presenting as extramedullary plasmacytoma of the thyroid, advanced grade II-III plasmablastic type. Skeletal Radiol 1995;24:314-16.

(5.) Reinish EI, Raviv M, Srolovitz H, Gornitsky M. Tongue, primary amyloidosis, and multiple myeloma. Oral Surg Oral Med Oral Pathol 1994;77:121-5.

From the Department of Surgery, Morehouse School of Medicine, Atlanta.

Reprint requests: J.K. Fortson, MD, Chief of Otolaryngology, Department of Surgery, Morehouse School of Medicine, 21136 Cleveland Ave., #300, Atlanta, GA 30344. Phone: (404) 768-9350; fax: (404) 768-2530; e-mail:

Originally presented at the annual meeting of the American Academy of Otolaryngology-Head and Neck Surgery; Sept. 13-16, 1998; San Antonio, Tex.

COPYRIGHT 2004 Medquest Communications, LLC
COPYRIGHT 2004 Gale Group

Return to Multiple myeloma
Home Contact Resources Exchange Links ebay