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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Multiple myeloma has many unusual manifestations, which may obscure the true cause and delay proper treatment. Physicians in all specialties should be alert to this possibility, since myeloma may be at the root of such diverse disorders as carpal tunnel syndrome, peripheral neuropathy, solitary lytic bone lesions, hypercalcemia, and hyperviscosity syndrome.
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PMID:Unusual presentations of multiple myeloma. 85 84

A case report of a patient with amyloidosis presented in which macroglossia and bilateral carpal tunnel syndrome were the initial features and myeloma was the probable underlying cause.
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PMID:Amyloidosis, macroglossia, and carpal tunnel syndrome associated with myeloma. 105 92

Thirty-five patients seen at the Mayo Clinic from 1968 to 1977 who had carpal tunnel syndrome and local deposition of amyloid without evidence of systemic amyloidosis were identified. The unlabeled immunoperoxidase method was used with antisera against purified amyloid proteins of the AA, A kappa, A lambda, AF/ASC1 (prealbumin) (transthyretin), and AB (beta 2-microglobulin) types. In 33 of the 35 patients, amyloid stained with antisera to transthyretin; in the remaining 2 patients, the amyloid did not stain with any antisera. Nine of the 35 patients had a monoclonal protein in the serum, and 2 had a monoclonal light chain in the urine. Systemic amyloidosis or multiple myeloma did not develop in any of these 11 patients. During follow-up, systemic amyloidosis developed in only 2 of the 35 patients: 1 had senile systemic amyloidosis and 1 had tissue that was inadequate for immunohistochemical staining. Amyloid localized to the tenosynovium consists of transthyretin, and systemic amyloidosis rarely develops.
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PMID:Amyloid localized to tenosynovium at carpal tunnel release. Immunohistochemical identification of amyloid type. 137 47

Primary amyloidosis and myeloma associated amyloidosis causes neuropathy in 10% of the cases, and hemodialysis associated amyloidosis causes carpal tunnel syndrome. However, most severe amyloid neuropathy is observed in familial amyloidotic polyneuropathy (FAP). FAP type I is an autosomal dominant systemic amyloidosis characterized by sensory dominant systemic amyloidosis characterized by dissociated sensory disturbance and autonomic dysfunction. Amyloid deposition is noted universally in endoneurium of peripheral nerve, especially prominent in sural, sciatic nerve, dorsal root ganglia and sympathetic ganglia. Moderate deposition was also noted in dorsal spinal root. Amyloid was absent in CNS. The number of small myelinated fibers is decreased. Unmyelinated axons are severely depleted and amyloid fibrils are observed around the wall of small vessels. Amyloid fibril protein consists of variant transthyretin (TTR:prealbumin) with one amino acid substitution of methionine-for-valine at position 30. Other types of FAP show another one point mutation in TTR molecule. Transgenic mouse model of FAP was produced by microinjecting cloned human variant TTR gene into mice. Amyloid were demonstrated in thyroid, kidney and intestine and so on, but not in peripheral nerve in these mice. Pathogenesis of neuropathy of FAP is not known, but mechanical compression to the nerve, ischemia and metabolic abnormality may play some role combined to cause of nerve fiber damage. The effect of deposition on physiochemical functions of the neuron and mechanism to accumulate in nervous system of the TTR remain to be elucidated.
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PMID:[Amyloid neuropathy]. 196 18

A number of different combination regimens including high-dose corticosteroids (HDCS) have been widely used in an attempt to achieve better results for relapsed or alkylating agent-resistant multiple myeloma (MM). A major complication of these regimens is commonly said to be infection. In addition, we have had occasion to point out that a rapid progression of systemic amyloidosis can be one of serious complications of HDCS therapy in MM. The patient, born in 1940, was diagnosed of having Bence Jones (BJ) type MM in 1987. The conventional therapy including alkylating agents and interferon-alpha induced a partial remission of 22 months' duration. After the relapse, 2 courses of vincristine, adriamycin plus high-dose dexamethasone resulted in a reduction of the excreted amount of urinary BJ proteins with symptomatic improvement. However, the following clinical features indicating systemic amyloidosis occurred in succession within 2 months after beginning the therapy: submandibular swelling, giant tongue, shoulder pad sign, carpal tunnel syndrome, low voltage on ECG and recurrent diarrhea. The biopsy specimens from the submandibular gland revealed amyloid deposition. In the present case, a rapid progression of systemic amyloidosis is supposed to be attributable to the HDCS therapy. The possible mechanism of enhancement of amyloidosis by HDCS therapy is discussed.
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PMID:[Rapid progression of systemic amyloidosis after high-dose corticosteroid therapy in multiple myeloma]. 225 65

The term, "monoclonal gammopathy of undetermined significance (MGUS)" is used because it is not known at the time of recognition whether the M-component will remain stable or will develop into multiple myeloma (MM) or related disorders. Recently, we have encountered a case of MGUS in which a diagnosis of primary amyloidosis (PA) was made more than 10 years after the recognition of an M-component in the serum. A 64-year-old man presented in 1979 for evaluation of monoclonal gammopathy. The level of M-component (IgG-lambda) in the serum was 1.6 g/dl. The urinary Bence Jones proteins (BJP) were negative. Bone marrow aspirate contained 9.8% plasma cells. Skeletal surveys were normal. A diagnosis of MGUS was made. In 1982, a trace amount of BJP was detected in the urine. Since 1988, carpal tunnel syndrome, angina pectoris and congestive heart failure developed in succession. In November 1989, the patient was admitted to Kyoto University Hospital for examination. Serum electrophoretic pattern remained unchanged. The excreted amount of urinary BJP was less than 0.3 g/day. Bone marrow aspirate contained 5.4% plasma cells. Histologic studies of bone marrow biopsy specimens revealed amyloid deposition. An echocardiogram was thought to reveal amyloidosis. Significant uptake of Tc-99m (V) DMSA was found in carpal regions, kidneys and heart. A diagnosis of PA was made. It is noteworthy that the development of PA did not accompany an increase in the serum M-component. An early diagnosis of PA as well as MM should be kept in mind in the follow-up study of patients with MGUS.
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PMID:[A long-term observation of development from monoclonal gammopathy of undetermined significance (MGUS) into primary amyloidosis]. 225 66

The relationship between monoclonal gammapathies and peripheral nerve involvement is well known. The pathogenesis of neuropathy has been ascribed to infiltrative, compressive, ischemic or haemorrhagic phenomena, to an amyloid storage or to antibody autoaggression toward neural antigens. Forty-five patients suffering from monoclonal gammapathy of uncertain significance (MGUS), multiple myeloma (MM), MG IgM, were submitted to a neurophysiological examination. 10 pts. were excluded because of neurotoxic drugs, diabetes or alcohol abuse. The investigation included SCV of median, ulnar, and sural n., MCV and F wave of peroneal n., H reflex, and computerized analysis of recruitment pattern. A subclinical neuropathy was found in 11 of 35 pts. (30%) without any neurological symptom. A CTS was found in 4 patients. The high rate of subclinical neuropathy in asymptomatic pts. is underlined by the Authors, while the presence of subclinical neuropathy in cases of MGUS needs further studies.
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PMID:[Subclinical neuropathies and paraproteinemias]. 254 May 13

Prominent crystalline inclusions were noted in the glomeruli of a 57-year-old man with a 6-month history of swelling and pain in the hands, slight proteinuria, and elevated serum creatinine and BUN. The crystalline inclusions were most prominent in the visceral epithelium but were also noted in endothelial and mesangial cells, in the parietal epithelium, and in the epithelial cells of the proximal tubules. On electron microscopy the crystalline structures were of various geometric shapes but had no definite substructure. Immunofluorescence was negative. The patient was considered to have a hitherto undescribed metabolic disease. Several months later the patient underwent an operation for carpal tunnel syndrome and amyloid deposits, crystalline inclusions similar to those noted in the kidney were observed in the synovial tissue. Shortly after this the patient was found to have multiple myeloma of IgG, kappa type with Bence Jones proteinuria. Lymphoplasmacytic cells in the bone marrow contained the same crystalline inclusions noted in the kidney and synovium. This case therefore seems to represent a new and very rare form of glomerular involvement in multiple myeloma.
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PMID:Crystalline glomerular inclusions in multiple myeloma. 254 97

One hundred fifty-two patients with amyloid in the tenosynovium who had carpal tunnel release were identified. Twenty-eight patients were excluded because of systemic amyloidosis: primary systemic amyloidosis (AL) in 24, secondary amyloidosis (AA) in 3, and familial amyloidosis (AF) in 1. The remaining 124 patients (82%) had carpal tunnel syndrome with local deposition of amyloid and no evidence of systemic amyloidosis. Median survival of the 124 patients from diagnosis of amyloidosis was 12 years. Only two patients had systemic amyloidosis develop--9 and 10 years after recognition of tenosynovial amyloid. Of particular interest were 12 patients who had an M-protein in the serum or urine. None of the 12 patients have had evidence of systemic amyloidosis or multiple myeloma during the median follow-up of 14 years. The authors conclude that amyloid may be localized to the tenosynovium and that systemic amyloidosis rarely develops during long-term follow-up.
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PMID:Amyloid localized to tenosynovium at carpal tunnel release. Natural history of 124 cases. 264

Cutaneous lesions are present in up to 40% of patients with primary and myeloma-associated systemic amyloidosis and occur as a result of tissue deposition of immunoglobulin light chain material derived from a circulating paraprotein. The occurrence of waxy, purpuric mucocutaneous lesions provides a crucial early pointer to underlying occult plasma cell dyscrasia; the combination of the symptoms of the carpal tunnel syndrome, macroglossia, and specific mucocutaneous lesions is highly characteristic. Although secondary systemic (reactive) amyloidosis rarely gives rise to clinically evident cutaneous lesions, it may be etiologically related to a number of chronic dermatoses. Lesions of nodular primary localized cutaneous amyloidosis are indistinguishable from those of primary and myeloma-associated systemic amyloidosis, and they result from local plasma cell infiltration. Macular and papular (lichen amyloidosus) variants of primary localized cutaneous amyloidosis may have a familial or racial basis and are characterized by a tendency for keratinocytes to undergo filamentous degeneration and apoptosis. The prognosis of patients with plasma cell dyscrasia-related systemic amyloidosis remains poor, since there is little response to therapy with cytotoxic agents, colchicine, or dimethylsulfoxide. Colchicine is the drug of choice in the prevention and treatment of the renal amyloidosis associated with familial Mediterranean fever, and dimethylsulfoxide may be useful in the management of patients with secondary systemic amyloidosis. Macular amyloid and lichen amyloidosus generally follow a chronic course with intractable pruritus; there have been isolated reports of the beneficial effect of dermabrasion, topical dimethylsulfoxide, and therapy with the aromatic retinoid, etretinate.
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PMID:Amyloid and amyloidosis. 327 77


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