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At least 19 recordsLinked to original sources
PubMed1986

N-acetyl-beta-D-glucosaminidase enzymuria in leukaemia and myelomatosis: effect of treatment in acute myeloblastic leukaemia and myelomatosis in adults.

The activity of the lysosomal hydrolase N-acetyl-beta-D-glucosaminidase (NAG) was measured in the urine of patients with leukaemia or myeloma. Elevated pre-treatment enzymuria was noted in all patient groups with acute myeloblastic leukaemias (AML) FAB type M4 or 5 displaying higher activities than AML patients FAB types M1-3, which in turn were higher than those found in patients with myelomatosis and chronic lymphocytic leukaemia. The ratio of the major isoenzymes of NAG, A/B was reduced significantly only in patients with AML. Following treatment, AML patients who entered remission demonstrated NAG levels which approached normal values. In those AML patients who were either in relapse, in the terminal phase of their illness or treated with aminoglycoside antibiotics, NAG enzymuria was similar to pre-treatment values. A reduction in urinary NAG levels and both serum and urine beta 2 microglobulin concentrations was also observed following treatment in myeloma patients. The use of enzymuria both as a guide to progress towards remission in AML patients and for assessing prognosis and progress in myeloma patients is discussed.

Acetylglucosaminidase↗
PubMed1969

Younger age of presentation and extraosseous tumour in IgD myelomatosis.

A study of 14 personal patients and 16 others in the literature shows that (1) IgD myelomatosis often presents at a significantly younger age than other forms of myelomatosis, and (2) during life extraosseous tumour can be detected in about two-thirds of these patients.The IgD form represents 1.5% of myelomatosis and shows an increased incidence of osteolytic lesions, hypercalcaemia, and renal failure, together with heavy Bence Jones proteinuria (90% type L).Like only Bence Jones myelomatosis, the IgD form seems to behave clinically in a more vicious manner.

Adult↗
PubMed1992

Leptomeningeal myelomatosis presenting with mental status changes and other neurologic findings.

BACKGROUND: Leptomeningeal myelomatosis is a rare complication of multiple myeloma. METHODS: The authors identified and studied three patients with leptomeningeal myelomatosis and reviewed previous case reports of this condition. RESULTS: The patients described here had intermittent abnormalities in mental status or cranial nerve and brain stem abnormalities. Two of the patients responded dramatically, though transiently, to treatment. In one patient, the clinical findings correlated with lesions visualized by gadolinium-enhanced magnetic resonance imaging. CONCLUSIONS: These patients are typical of those reported previously. Patients with leptomeningeal myelomatosis often have a good response to treatment initially, but long-term survival is rare.

Aged↗
PubMed1976

Extraosseous myelomatosis. Clinical presentation with symptoms in lower urinary tract.

We believe this to be the first case reported of extraosseous myelomatosis seen clincally with lower urinary tract symptoms. Extraskeletal spread of myelomatosis which reportedly occurs in two thirds of cases is discovered postmortem. Immunoglobulin A prevails in this type of myelomatosis. Clinical course and autopsy findings are discussed.

Humans↗
PubMed1992

Combined chemotherapy with ABCM versus melphalan for treatment of myelomatosis. The Medical Research Council Working Party for Leukaemia in Adults.

Both melphalan and cyclophosphamide increase life expectancy in patients with myelomatosis, but few large randomised studies have compared combination chemotherapy regimens with these single agents. In the Vth MRC myelomatosis trial, the survival of 314 patients randomised to receive ABCM (adriamycin, BCNU, cyclophosphamide, and melphalan) as first-line treatment was significantly longer than that of 316 patients given intermittent melphalan (M7) (p = 0.0003). The 75%, median, and 25% survivals were 7, 24, and 42 months, respectively, with M7 and 10, 32, and 56 months, respectively, with ABCM. Stable disease with few symptoms (plateau) was achieved by 61% of patients given ABCM and 49% of those given M7 (p = 0.004). Myelotoxicity was comparable between regimens. Cross-trial analysis suggests that M7 is comparable to melphalan and prednisone or melphalan, prednisone, and vincristine; that the efficacy of ABCM in the Vth trial and VIth MRC trials is comparable; and that ABCM gave better survival than intermittent melphalan regimens in the prognostic groups analysed. The results indicate that ABCM is an acceptable regimen that is more effective than melphalan, with or without prednisone, for first-line treatment of myelomatosis.

Aged↗
PubMed1975

Treatment of osteolytic myelomatosis with mithramycin.

The treatment of rapidly progressive skeletal demineralisation in myelomatosis has been studied with the help of metabolic calcium balance in two patients; In one, osteoporosis accelerated during treatment with melphalan and prednisolone, although he remained normocalcaemic throughout, suggesting that osteoporosis was aggravated by corticosteroid therapy. In the other patient, who was initially hypercalcaemic, conventional treatment produced clinical remission before eventual relapse with more hypercalcaemia and skeletal dissolution. Both patients were then treated with mithramycin alone, and, although neither obtained haematological remission, bone pain was relieved, hypercalciuria and hypercalcaemia were abolished, and calcium balances proved that mithramycin was effective in restoring calcium equilibrium. The results indicate that mithramycin may abolish excessive bone resorption in myelomatosis and that severe bone dissolution may occur in the absence of hypercalcaemia. Regular determination of 24-hour urinary calcium excretion as well as of plasma-calcium is important in monitoring process. Mithramycin should be considered in the early treatment not only of hypercalcaemia but also of severe hypercalciuria, if these complications do not rapidly remit during the first course of conventional myeloma therapy, with or without steroids. Finally, these results add to evidence that a humoral factor may be responsible for osteoclast stimulation in myelomatosis.

Aged↗
PubMed1976

The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis.

From this study I suggest that extramedullary plasmacytoma (EMP) shows several important differences from myelomatosis and solitary myeloma of bone (SMB) which can be summarized as follows: 1. A marked preference for the primary tumor to present in a particular site, namely the upper air passages. 2. A high incidence of metastatic spread to soft tissues. 3. Spread to bone occurs frequently but shows no preference for bones containing active hematopoietic tissue and widespread bone-marrow involvement occurs only occasionally. 4. Prolonged survival may be achieved with therapy for local disease. 5. Vigorous treatment for disseminated disease can be given and may result in longer remissions than those usually seen in myelomatosis. Healing of bone lesions has been observed on several occasions. It is concluded also that SMB constitutes a rather unusual presentation of myelomatosis but is essentially the same disease process.

Adolescent↗
PubMed1982

Bone mineral content in myelomatosis.

Bone mineral content (BMC) was determined by photon absorptiometry on both forearms in 28 patients with myelomatosis. Nineteen patients had received intermittent treatment with cytostatic agents and prednisone. No significant reduction of BMC was found between the patients with myelomatosis and an age- and sex-matched normal population. Neither was any significant decrease in BMC with the duration of the disease demonstrated. In 16 patients, routine X-ray indicated osteoporosis, but their BMC was not reduced compared to the patients without osteoporosis on X-ray. However, the former patients were significantly older than the latter. Provided the term "generalized osteoporosis" implies reduced mineral content in all bones, the study indicates that patients with myelomatosis have not generalized osteoporosis.

Adult↗
PubMed1980

Amyloid arthropathy in myelomatosis--intracytoplasmic synovial deposition.

An unusual case is described of a West Indian man who presented with symptoms of an acute polyarthritis and who was found to have myelomatosis. At necropsy, generalized amyloidosis was found with particular involvement of synovial tissue. Myelomatosis presenting as an acute polyarthritis resembling rheumatoid arthritis is an uncommon, although well recognized entity, whereas an arthritic picture produced by amyloidosis is exceptional. The occurrence of joint pathology in myelomatosis and amyloidosis is discussed and the unique feature of intracytoplasmic amyloid deposition seen in this case is stressed.

Amyloid↗
PubMed1977

Vitamin B12 metabolism in myelomatosis.

In 38 patients with myelomatosis the serum cobalamin varied from 34 pmol/1 to 404 pmol/1, median 181.5 pmol/1, which is significantly lower than the levels in 22 control persons with range 173-535 pmol/1, median 265 pmol/1. In spite of low serum cobalamin no symptoms of vitamin B12 deficiency could be demonstrated in any of the patients, except for the one patient who had a serum cobalamin of 34 pmol/1. Mean values for Hb, MCV, PCV, serum lactate-dehydrogenase, adjested red cell folate and nucleated neutrophil count were similar in a group of patients with a serum cobalamin below 160 pmol/1 and a group of patients with higher serum cobalamin values. The decrease in serum cobalamin is due in part to a reduction in the major cobalamin binder (TC-I) in serum. Measuring serum cobalamin in relationship to gastric acis secretion, we found a significantly higher frequency of hypo- and achlorhydria in patients with serum cobalamin below 160 pmol/1 although the intestinal absorption of vitamin B12 was normal by a Schilling test. Although our finding of low saturation of TC-I in serum seems to demonstrate decreased vitamin B12 content in the body in myelomatosis, the lack of evidence for a functional vitamin B12 deficiency speaks against giving a supplement to patients with myelomatosis.

Aged↗
PubMed1984

Decrease of Fc gamma and C3b receptor-bearing granulocytes and of T lymphocytes in myelomatosis.

18 patients with myelomatosis had lower percentages of granulocytes bearing receptors for Fc gamma (47.6%) and C3b (43.0%) than controls (80.4% and 75.0%). The percentage of T lymphocytes was decreased in patients when untreated sheep erythrocytes were used as indicator cells. Patients with high serum IgG levels had lower percentages of T lymphocytes. There was no significant difference in receptor profile between treated and untreated patients. The decrease in Fc gamma and C3b receptor-bearing granulocytes in myelomatosis is probably not due to serum or plasma factors since (a) the distribution of receptor-bearing lymphocytes was not different from that of the controls; (b) extra washings of cells or overnight incubation did not enhance the percentage of receptor positive cells; and (c) incubation of normal granulocytes in sera or plasma from myelomatosis patients did not cause a more pronounced reduction in the proportions of Fc gamma of C3b receptor-bearing cells than incubation in normal sera or plasma.

Aged↗
PubMed1992

Abnormal bone remodelling in patients with myelomatosis and normal biochemical indices of bone resorption.

We studied bone biopsies from 26 patients with myelomatosis with apparently normal skeletal metabolism. Quantitative histomorphometric measurements suggested that skeletal disease was progressive despite normocalcaemia and normal urinary excretion rates of calcium and hydroxyproline. When biopsies were divided according to the involvement of marrow by plasma cells, bone resorption--as judged by the eroded surface--increased significantly the greater plasma cell burden. Osteoclasts were frequent with moderate tumour burdens, but there was no further increase in the number of osteoclasts when plasma cell infiltration increased by more than 50% of bone marrow. Contrary to expectation, the numbers of osteoblasts and bone formation rates were increased with bone biopsies with moderate tumour burden, but were markedly lower when plasma cell infiltration occupied more than 50% of bone marrow, due to a decreased functional capacity of osteoblasts. We conclude that skeletal bone disease in myeloma is commonly progressive despite apparently stable bone disease as judged by biochemical measurements. The major mechanism of bone loss in myelomatosis is increased osteoclastic resorption but decreased bone formation contributes to bone loss with heavy plasma cell burdens. Urinary excretion of calcium and hydroxyproline provide insensitive indices of bone resorption in myelomatosis.

Alkaline Phosphatase↗
PubMed1974

The kidney and intravascular coagulation in myelomatosis.

In 15 out of 35 patients with myelomatosis histological examination showed intravascular fibrin within the glomeruli, and this was associated with proliferation of the mesangial complex in 12. The presence of intravascular fibrin and mesangial proliferation was not associated with any specific immunoglobulin abnormality or with the presence or absence of Bence Jones proteinuria. In addition to fibrin being present within glomerular capillaries it was also shown in intertubular capillaries in three cases of myelomatosis with acute tubular necrosis. It is suggested that intraglomerular coagulation and fibrin deposition may contribute to the genesis of renal failure in myelomatosis.

Acute Kidney Injury↗
PubMed1992

The role of interferon-alpha in the management of myelomatosis.

The data provided in the medical literature suggest that alpha-IFN is a useful agent in the management of myelomatosis. In particular, its combination with conventional induction therapies of previously untreated MM patients may improve the overall response rate and probably increase the number of complete responders. On the other hand, even though alpha-IFN alone, in some patients with previously untreated MM, may induce good objective responses, it certainly remains less effective than conventional chemotherapy. Moreover, in a small proportion of advanced MM patients, alpha-IFN alone has induced objective responses. It is therefore possible that alpha-IFN should be combined with other therapeutic modalities to improve the observed response rate in these patients. Finally, alpha-IFN maintenance treatment seems to be one of the most promising therapies for patients with myelomatosis. However, the achievement of a "true" plateau phase after the induction treatment is certainly necessary to permit alpha-IFN maintenance treatment to prolong the response duration. As for the prolongation of survival duration observed in the Italian study, it requires confirmation by the other ongoing randomized studies. In the future, a better understanding of the mechanisms of action of alpha-IFN, as well as the increasing use of other biologic response modifiers, will improve the therapeutic modalities utilized to treat myelomatosis and therefore lead to better control of this so-far-incurable disease.

Humans↗
PubMed1982

Multiple cranial nerve palsies as the presenting feature of meningeal myelomatosis.

The case of a 69-year-old man with IgGK myeloma in whom meningeal myelomatosis with multiple cranial nerve palsies developed, is reported. Review of previously reported cases of clinically apparent meningeal myelomatosis indicates this rare complication frequently presents with cranial nerve palsies; currently available treatment is ineffective.

Adult↗
PubMed1980

Amyloid casts within renal tubules: a singular finding in myelomatosis.

This study was carried out in order to investigate a possible relationship between multiple myeloma and the occurrence of material exhibiting the properties of amyloid within renal tubules. Two groups of autopsied patients, with myelomatosis and benign monoclonal gammopathy were examined for the presence of amyloid deposits in renal and extra-renal sites. Urines were analysed for the presence and amount of Bence Jones protein and the pattern of the associated proteinuria was characterized. Renal tubular casts exhibiting the histochemical characteristics of immuno-amyloid were found exclusively in myeloma patients with Bence Jones proteinuria but without the renal lesions classically described as "myeloma kidney". This finding was independent of the occurrence of immuno-amyloid deposits in other renal and extra-renal sites, suggesting involvement of local factors in the pathogenesis of amyloid formation and deposition within renal tubular lumina. The results of present study suggest the conclusion that the presence of amyloid intratubular casts is to be regarded as a peculiar finding in myelomatosis.

Aged↗
PubMed1980

Enzyme-induced modification of the surface properties of lymphoid cells in malignant disease. I. Effect of trypsin on rosette formation by lymphocytes in myelomatosis.

The surface properties of blood lymphocytes from treated myeloma patients and healthy controls were studied in vitro. The patients were tested 6 weeks after the last treatment to allow time for cells to recovery from possible drug toxicity. Peripheral-blood lymphocytes were tested for rosette formation with unsensitized sheep erythrocytes (E rosettes) and with complement and antibody-coated erythrocytes (EAC rosettes). The tests were duplicated using lymphocytes pretreated with trypsin. As others have noted, myelomatosis is associated with increased blood levels of EAC-rosette-forming cells and a marked reduction in E-rosette-forming cells. E-rosette formation was significantly increased by pretreatment of myeloma lymphocytes with trypsin. By contrast, enzyme-treated cells showed no significant change in EAC-rosette formation. These results suggest that the absolute number of circulating T cells is probably not reduced in myelomatosis, but that the surface of T cells is somehow modified so that a proportion of them lose the ability to form E rosettes.

B-Lymphocytes↗
PubMed1983

Evaluation of serum beta 2-microglobulin as a prognostic indicator in myelomatosis.

Serum beta 2-microglobulin (beta 2-m) is frequently increased in patients with myelomatosis. The possibility that it could provide a biochemical indicator of prognosis was tested in a group of 129 patients from 3 centres, all serum analyses being carried out in one laboratory by radioimmunoassay. A strong association between the pretreatment serum beta 2-m level and survival was demonstrated, the data for the 2 main subgroups being very similar. In further detailed analyses of 64 patients, serum beta 2-m proved to be a stronger indicator of prognosis than current "standard" clinical and laboratory data, including stage determined by the method of Durie and Salmon and the combination of haemoglobin level and blood urea. The association between serum beta 2-m and survival remained close after treatment as indicated by the findings at one year. The serum beta 2-m in myeloma reflects the tumour mass and also reduced glomerular filtration when renal failure supervenes. It is concluded that the serum beta 2-m is a powerful prognostic indicator in myelomatosis and of considerable value in the investigation of patients with the disease.

Beta-Globulins↗