Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026764 (multiple myeloma)
 36,148 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From 1960 through 1972, 236 cases of amyloidosis with histologic proof were found. The amyloidosis was primary (without evidence of preceding or coexisting disease) in 132 cases (group 1) and associated with multiple myeloma in 61 (group 2). Secondary amyloidosis appeared in 19 cases (associated with rheumatoid arthritis or osteomyelitis in two-thirds of them). There were 22 patients with amyloid localized to a single organ (bladder, lung, skin, or larynx in more than half of them). Two patients had familial amyloidosis. In group 1 and group 2, the most common presenting symptoms were fatigue, weight loss, edema, dyspnea, light-headedness or syncope, and paresthesias. Symptoms of the carpal-tunnel syndrome were frequent. The liver was palpable in almost 50% of the series, but splenomegaly was an initial finding in less than 10%. Macroglossia was recorded in 26% of group 2 and in 12% of group 1. Enlargement of submandibular structures was noted in about 10% of cases; and purpura, particularly around the eyes, was a significant feature. Substantial numbers of the patients had carpal-tunnel syndrome, nephrotic syndrome, congestive heart failure, sprue, peripheral neuropathy, or orthostatic hypotension. Approximately 50% of patients had renal insufficiency at the time of diagnosis. Proteinuria was found in more than 90%. A monoclonal protein was found in the serum of 49% of group 1 and in 74% of group 2. Monoclonal proteins were found in the urine of 35% and 81%, respectively. Only 12% of patients in group 1 had no monoclonal protein when both serum and urine were analyzed, and all patients of group 2 had a monoclonal protein in the serum or urine when both were analyzed. Lambda light chains were more common than kappa. None of the patients in group 1 had more than 15% plasma cells in the marrow, whereas more than half of group 2 had more than 15% plasma cells. Roentgenograms showed no evidence of skeletal disease in 94% of group 1, but 50% of group 2 had skeletal abnormalities. Rectal biopsy was positive for amyloid in 84% of cases. Kidney, liver, and carpal-tunnel biopsies were positive in 90% or more. Follow-up of all 193 patients in groups 1 and 2 revealed that 80% of group 1 and 97% of group 2 had died. The median survival was 14.7 months in group 1 and 4 months in group 2. Cardiac failure was the most common cause of death, accounting for 30% of the fatalities. We also reclassified all cases by the method of Isobe and Osserman (105), which is based on clinical patterns: pattern I--principal involvement of tongue, heart, gastrointestinal tract, muscle, nerves, skin, and carpal ligaments; pattern II--principal involvement of liver, spleen, kidneys, and adrenals; and mixed pattern I and II. This analysis failed to reveal predictive value in the clinical pattern classification, and did not discern the survival differences between primary amyloidosis (group 1) and amyloidosis with myeloma (group 2). Consequently, for the present we prefer the classification used in this study.
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PMID:Amyloidosis: review of 236 cases. 115 71

Six months after a pneumonectomy for myeloma, which had preoperatively been indistinguishable from bronchial carcinoma, a 50-year-old man presented with shortage of breath, cyanosis and episodes of syncope on standing or walking, symptoms which improved on lying down (platypnea). On one occasion these symptoms necessitated controlled artificial ventilation, but even at an inspiratory oxygen saturation of 100%, blood gases only partially improved (pCO2 27 mm Hg, pO2 67 mm Hg, O2 saturation 93%). Right heart catheterization in recumbency revealed a right to left shunt at atrial level of 37% of systemic flow. Contrast medium injection into the inferior vena cava near the heart demonstrated cardiac displacement and rotation. Part of the inferior vena cava flow passed into the left atrium via a patent foramen ovale: it is likely that this shunt increased in the upright position. After surgical closure of the patent foramen ovale and partial relocation of the heart (with a vicryl net) the patient has now remained free of symptoms for 5 years.
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PMID:[Patent foramen ovale and platypnea after pneumonectomy]. 145 15

A patient with multiple myeloma and amyloidosis was admitted to hospital following successful cardiopulmonary resuscitation at home. No disturbances in heart rhythm were seen during the first 48 hours of continuous telemetric ECG recording. The patient died from ventricular asystole due to complete atrioventricular block, while he was on a 24-hour Holter monitoring the fifth night in hospital. Patients with known cardiac amyloidosis and syncope should undergo long-term ECG recordings, preferably by telemetry. Repeated registrations may be necessary to discover disturbances in heart rhythm.
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PMID:Sudden death caused by heart block in a patient with multiple myeloma and cardiac amyloidosis. 336 19

Primary amyloidosis, due to amassing of fragments of light chains of IgG, often causes cardiac involvement. We describe a 65-year-old woman with multiple myeloma efficaciously treated with chemotherapy. Amyloidosis had been supported by myelic biopsy. The patient came to our observation because of right heart failure, hypotension and syncope: she was treated with a dopamine i.v. and was in cachectic status. She had a moderate pericardial effusion. ECG showed reduction of QRS amplitude, I degree atrioventricular block, posterior fascicular and right bundle branch block. Right cardiac catheterization showed a restrictive situation. After 1 week exitus occurred by asystole. In this case, there were other involvements by amyloidosis, besides the cardia one: that of autonomic nervous system and, probably, surrenal.
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PMID:[Case of cardiac amyloidosis associated with IgG-K multiple myeloma in the framework of restrictive myocardiopathy]. 1020 58

We conducted a clinical trial of thalidomide as initial therapy for asymptomatic smoldering (SMM) or indolent multiple myeloma (IMM). Sixteen patients were studied. Thalidomide was given orally at a dose of 200 mg/day for 2 weeks, and then increased as tolerated by 200 mg/day every 2 weeks to a maximum dose of 800 mg/day. Bone marrow microvessel density (MVD) and angiogenesis grading were estimated using CD34 immunostaining. Six patients had a confirmed response to therapy with at least 50% or greater reduction in serum and urine monoclonal (M) protein. When minor responses (25-49%) decrease in M protein concentration) were included, 11 of 16 patients (69%) responded to therapy. Major grade 3-4 toxicities included two patients with somnolence, and one patient each with syncope and neutropenia. Pre-treatment MVD was not a significant predictor of response to therapy, median MVD 4 and 12 in responders and non-responders respectively, P = 0.09. We conclude that thalidomide has significant activity in the treatment of newly diagnosed SMM/IMM. However, we do not recommend treatment with thalidomide at this stage since some patients with SMM/IMM can be stable for several months or years without any therapy. Additional randomized trials are needed to determine if thalidomide will delay progression to active multiple myeloma.
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PMID:Thalidomide for previously untreated indolent or smoldering multiple myeloma. 1148 May 71

Patients with unexplained heart failure, hepatomegaly, nephrotic syndrome, or peripheral neuropathy should be evaluated for primary systemic (amyloid light-chain, or AL) amyloidosis by first seeking evidence of a clonal plasma cell disorder with serum and urine immunofixation studies, as well as a bone marrow biopsy. Immunostaining of the marrow biopsy for lambda and kappa isotypes will usually demonstrate a dominant clonal population of plasma cells if immunofixation studies are negative (less than 10% of cases). Tissue diagnosis of amyloidosis should be sought by biopsy of the abdominal fat or an involved organ. In addition, patients with stable myeloma or monoclonal gammopathy of undetermined significance who develop such conditions or become progressively ill should be evaluated for amyloidosis. We recommend that newly diagnosed patients with AL amyloidosis, who meet criteria for autologous hematopoietic cell transplantation, be considered for high-dose melphalan with stem cell support. Criteria usually include adequate cardiac, pulmonary, and hepatic function. AL amyloidosis patients treated with autologous transplantation frequently achieve durable complete remissions of the plasma cell disease and marked improvement in amyloid-related organ dysfunction. AL amyloidosis patients with dominant cardiac amyloid, who are without symptomatic pleural effusions and have no history of cardiac syncope or symptomatic arrhythmias, may be considered for autologous transplantation but are at increased risk of peritransplant mortality. Autologous transplantation should not routinely be offered to patients with dominant cardiac amyloid with recurrent effusions or histories of syncope or arrhythmias or to patients older than 50 years of age with more than two major organ systems involved (eg, heart, kidneys, liver, and peripheral nerves). We recommend that AL patients with isolated advanced cardiac or hepatic amyloidosis be considered for solid organ replacement followed by autologous transplantation. Otherwise, AL patients who are elderly or ineligible for autologous transplantation may be treated with oral melphalan (Alkeran, GlaxoWellcome, Middlesex, UK) and prednisone; however, because the response rate is only about 25% and the prognosis poor, such patients might also be enrolled on clinical trials of emerging therapies.
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PMID:Primary systemic amyloidosis. 1205 64

Treatments effective against multiple myeloma may be useful in primary systemic amyloidosis (AL). Thalidomide is active in myeloma. Results of the first 12 patients enrolled on a phase II trial of thalidomide for AL are presented. Progressive edema, cognitive difficulties, and constipation occurred in approximately 75%; dyspnea, dizziness and rash in 50%. Five developed progressive renal insufficiency. Deep venous thrombosis and syncope each occurred in two. Median time on the study was 72 days, range was 25 to 333 days. All 12 have withdrawn from the study (side-effects, 6; progression, 4; and death, 2 patients). AL patients do not tolerate high dose thalidomide.
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PMID:Poor tolerance to high doses of thalidomide in patients with primary systemic amyloidosis. 1498 85

We present a case of a 76-year-old Japanese man with hypertension and multiple myeloma (MM) presented with syncope and sinus bradycardia. Thalidomide therapy for MM was added to longstanding atenolol therapy one month prior to presentation. His heart rate (HR) was around 70 beats per minute (bpm) before addition of Thalidomide. His HR on presentation was less than 30 bpm. He was treated with intravenous atropine followed by temporary pacemaker and taken off atenolol. His HR returned to around 70 bpm few days after discontinuation of atenolol, even though he was still taking thalidomide, permitting outpatient management without a pacemaker. Both thalidomide and atenolol have been reported to cause bradycardia. Neither agent caused bradycardia when used alone in this patient, but simultaneous use caused symptomatic bradycardia. As thalidomide is prescribed more frequently, clinicians should be aware of the possibility of drug-induced sinus bradycardia due to the interaction of thalidomide and beta-blockers.
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PMID:Syncope and sinus bradycardia from combined use of thalidomide and beta-blocker. 1861 49

Bortezomib is a new chemotherapeutic agent approved for the treatment of relapsed/refractory and newly diagnosed multiple myeloma. One of the major side effects of bortezomib is a peripheral length-dependent sensory axonal neuropathy and, less frequently, a small fiber neuropathy. Autonomic symptoms like postural dizziness, syncope, diarrhoea, ileus, impotence and urinary disturbances have been reported, nevertheless, autonomic neuropathy has never been characterized. We describe by means of immunofluorescence, the involvement of autonomic skin nerve fibers in three patients with small fiber neuropathy induced by bortezomib treatment.
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PMID:Somatic and autonomic small fiber neuropathy induced by bortezomib therapy: an immunofluorescence study. 2129 Jan 60

A 52-year-old woman underwent vertebroplasty for fractures of the T10, T11, and L2 vertebrae secondary to multiple myeloma. She was discharged uneventfully within a week. Nine months later, she was readmitted for syncope. Echocardiography revealed a mass in the right atrium. Magnetic resonance imaging (MRI) revealed a low signal intensity irregular mass (8x7 mm) and a comma-shaped mass (12 mm) in the right atrium. She was prophylactically anticoagulated for the probable emboli. Repeat cardiac MRI performed 2 weeks later showed that the size of the masses remained unchanged. Surgical intervention was not suggested by the cardiothoracic surgeons. Three months later, the patient remained uneventful.
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PMID:Asymptomatic cement embolism in the right atrium after vertebroplasty using high-viscosity cement: a case report. 2516 66


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