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PubMedDate not supplied

Myelomatosis: clinical and laboratory features in Nigerians.

BACKGROUND: The objective was to investigate the factors influencing survival of Nigerians with Myelomatosis. MATERIALS AND METHODS: The pre-therapy clinical and laboratory features of patients managed at the Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, between June 1986 and May 2001 were studied. Diagnosis was based on history of bone pain, marrow plasmacytosis, osteolytic bone lesions, serum biochemical profile, monoclonal (M) band, and/or Bence-Jones proteinuria. RESULTS: Twenty-seven patients (22 males, 5 females) aged 15 to 81 (median, 60) years were managed within the study period. Of the 27, 5 (18.5%) were 40 years or younger while 14(51.8%) were 60 years or older. Multiple myeloma (MM) is the main sub-type seen (81.5%). Majority presented with bone pain, weakness, fatigue and inability to walk. Anaemia, high erythrocyte sedimentation rate and bone marrow plasmacytosis were the significant haematological findings. Serum protein electrophoresis showed M-band in 6 cases of MM, with 3 of them also having Bence-Jones proteinuria. Renal function impairment and hypercalcaemia occurred mainly in those with MM. Multiple osteolytic lesions and pathological fractures were recorded in 44% of those with MM and Solitary Myeloma of Bone. Twelve patients were already dead at the time of analysis, with renal failure being the cause in 8 and anaemia in the rest. The median survival was 1.2 months. CONCLUSIONS: We could conclude that the presenting features of Nigerian patients with myelomatosis are not different from reports elsewhere, but high default rate and short survival are particularly noted.

Adolescent↗
PubMed1991

[Meningeal myelomatosis as an exclusive form of recurrence in a case of IgA-lambda myeloma in complete systemic remission].

Meningeal myelomatosis is an extremely rare clinical presentation generally associated to terminal states of the disease and is more frequent in the presence of peripheric plasmocytosis or leukemia of the plasmatic cells. Diagnosis requires its demonstration in the cephalorhachidian liquid and its monoclonal secretion. A case of relapse of meningeal myelomatosis in a patient with multiple IgA-lambda myeloma is presented in which the systemic disease fulfilled the criteria for complete remission.

Aged↗
PubMed1988

Selective induction of light chain synthesis in cultures of blood lymphocytes from patients with IgG myelomatosis.

In the present study evidence is provided that neoplastic B cells from the blood from four of 24 patients with myelomatosis were activated selectively with polyclonal B cell mitogens. In three of these patients the activated cells produced light chains without heavy chains; of these, two patients had IgGK paraproteins and one had free lambda light chain disease. The ratio of kappa-expressing to lambda-expressing B cells in the initial blood B cell preparations was within the range for healthy controls for all four patients where neoplastic B cells were selectively activated. It is concluded that in some patients with myelomatosis the neoplastic clone is a mosaic of: (1) cells capable of synthesizing both light and heavy chains with (2) cells producing light chains only.

Adult↗
PubMed1985

Widespread crystallisation of paraprotein in myelomatosis.

Two patients with myelomatosis leading to acute renal failure were found at autopsy to have extensive crystalline deposits of paraprotein within the capsular space of the glomeruli, renal tubules and renal blood vessels. In one case, crystalline deposits in the renal arteries had caused infarction of both kidneys. Both patients had extensive paraprotein deposits in the heart and in other tissues, leading to myocardial infarction in one case. This patient also had extensive pulmonary deposits of paraprotein without a local plasma cell infiltrate, an appearance which has not been described before. Crystalline deposition does not appear to be specifically associated with a single class of paraprotein. This type of disease, affecting many organs, may be more common than has generally been appreciated and should be considered as a cause of otherwise unexplained organ failure in patients with myelomatosis.

Acute Kidney Injury↗
PubMed1981

[The treatment of bone lesions in myelomatosis (author's transl)].

41 patients suffering from a bone lesion due to myelomatosis were treated (32 multiple myelomata and 9 solitary myelomata). Diagnosis was established by biopsy in 9 cases. In 8 cases there was compression of the spinal cord or of a spinal root. 15 pathological fractures were treated by fixation and bony union was always obtained. In 7 other cases, fixation was used to prevent fracture and 2 patients had a total joint prosthesis. It is concluded that the treatment of the bony lesions of myelomatosis is comparable to that of bony metastases but the average survival is much longer.

Adult↗
PubMed1982

Peptichemio induction therapy in myelomatosis.

Fifteen patients with multiple myeloma, two of whom had plasma cell leukemia, were treated between May 1974 and December 1978. Peptichemio was administered intravenously at doses of 40-80 mg/48 h, courses including 4-17 administrations in association with moderate doses of prednisone (15-50 mg/day) and androstanes at high dosages (250 mg weekly). In two patients PTC was associated with vincristine (VCR) administered on the first day of the course. Eight patients were previously untreated, four had been resistant to melphalan (MPH) and/or cyclophosphamide (CTX), and three had been treated irregularly with one or both of these alkylating agents. The criteria of response to therapy are reported. Out of a total of 15 PTC courses administered we obtained 13 responses, eight complete and five partial; no response was achieved in the other two patients. In the four patients who were resistant to MPH and/or CTX we obtained three responses, which were maintained with the same alkylating agent to which they had been resistant previously. The time needed to obtain a response in 90% of the patients was 6 weeks. Peptichemio was shown to be effective in patients in an advanced stage of the disease, in patients with light-chain myeloma and in those with plasma cell leukemia. The association of VCR potentiated the antitumor effect, but also increased the myelotoxicity. The PTC treatment was well tolerated. It is suggested that PTC be used in induction treatment of myelomatosis and in patients resistant to traditional alkylating agents.

Adult↗
PubMed1982

Renal plasma clearance: a valuable marker in myelomatosis.

Forty-two consecutive patients with untreated myelomatosis (MM) formed the basis of settling the validity of measuring the renal plasma clearance (RPC), either indirectly using the serum creatinine or directly using the glomerular filtration rate (GFR) when studying anaemia, calcium metabolism, proteins in serum and urine, and prognosis. Patients without light chain excretion in the urine had a higher GFR (P less than 0.01) than patients with light chain excretion. The haemoglobin concentration (Hb) was strongly correlated (P less than 0.001) to both, serum creatinine and GFR. Patients with normal serum concentrations of the physiological immunoglobulins had higher Hb (P less than 0.01) than patients with reduced serum immunoglobulins. Patients with serum calcium greater than 3.00 mmol/1 had additional reduced GFR compared with the other myeloma patients. The serum parathyroid hormone was decreased (P less than 0.01) and inversely correlated to the GFR. Patients with increased serum creatinine, reduced GFR or with osteolytic bone lesions had a decreased survival rate. The study shows that the major factor in prediction of Hb and prognosis in patients with MM is the RPC expressed either as the serum creatinine or the GFR. In addition, the significant correlations between the GFR and the other variables in MM assessed the RPC to be a useful and valuable marker in studies of anaemia, protein and calcium metabolism and prognosis in MM.

Aged↗
PubMed1990

Atypical type III hyperlipoproteinemia in a patient with Ig A myelomatosis.

We studied a 58-year-old woman with severe therapy-refractory hyperlipidemia, xanthomatosis, and multiple myeloma (immunoglobulin A, lambda light chain). The lipid disorder became evident about half a year prior to the expression of myelomatosis. Clinical symptoms were similar to those found in classical type III hyperlipoproteinemia but the underlying metabolic defect was different from the one described in this primary dyslipoproteinemia. The patient has the heterozygous apolipoprotein E3/2 phenotype and her VLDL-cholesterol/serum-triglyceride ratio is unusually low at 0.05. Evidence is given that the hyperlipoproteinemia is due to an impaired catabolism of intermediate density lipoproteins probably because of a reduced hepatic triglyceride lipase activity.

Cholesterol, VLDL↗
PubMed1979

Interferon therapy in myelomatosis.

Four patients with myelomatosis (one IgG-chi-type, two IgA-chi, and one Bence Jones-x) were given human leucocyte interferon as the only treatment for from 3 to 19 months. Remission was complete in two patients and partial in the other two. Normal bone-marrow haematopoiesis was slightly inhibited. The disease has not so far progressed in any of the patients.

Aged↗
PubMed1980

Serum beta 2-microglobulin in myelomatosis: potential value in stratification and monitoring.

In a longitudinal study of the evolution of serum beta 2-microglobulin (beta 2-m) levels in 37 patients with myelomatosis, those patients with a level of < 4 mg/l at first presentation had a median survival of 46 months, whereas those with an initial level of > mg/l had a median survival of 15 months. THe beta 2-m appeared to be independent of the level of the paraprotein and its class, as seen in a vertical study of 129 patients. Analysis of the influence of a rising serum creatinine on the serum beta 2-m indicates that beta 2-m production is excessive in advanced disease with or without renal failure. Practical application of the measurement of serum beta 2-m in the stratification and monitoring of patients is suggested.

Beta-Globulins↗
PubMed1980

Treatment comparisons in the third MRC myelomatosis trial. Medical Research Council's Working Party on Leukaemia in Adults.

Results after an average follow-up of 3 years are presented on 485 patients in the 3rd MRC therapeutic trial in myelomatosis. The 353 non-azotaemic patients (199 now dead) were randomized between i.v. cyclophosphamide (CY) and oral melphalan with prednisone (M & P). THose treated with M & P fared slightly, but non-significantly, better. The 132 azotaemic patients (111 now dead) were randomized between i.v. CY and a 4-drug regimen, and both groups fared equally badly. Finally, after one year of the allocated treatment, 297 survivors (126 now dead) were randomized either to stop all treatment until evidence of relapse was obtained, or to continue treatment with azathioprine and vincristine, interrupted every 3 months for a course of the first-allocated treatment. The overall results suggested that maintenance therapy was beneficial, though the results were not statistically significant. Most of the difference was found among the few patients with unfavourable prognostic features who survived one year and were eligible for this randomization. In this, as in the two previous MRC trials, no striking differences have emerged between the therapeutic effects of different schedules of melphalan and/or CY. Consequently, a regimen of intermittent oral melphalan (with or without prednisone) seems satisfactory, because it is among the least toxic and most convenient. The 4th myeloma trial, now beginning, seeks to discover whether the addition of vincristine to the regimen can improve these results.

Aged↗
PubMed1980

Prognostic features in the third MRC myelomatosis trial. Medical Research Council's Working Party on Leukaemia in Adults.

This paper reports the prognostic significance of clinical and laboratory features recorded at presentation in 485 patients entered into the Medical Research Council's 3rd therapeutic trial in myelomatosis between July 1975 and August 1978. The data were complete up to 1 January 1980, with a median follow-up time of 36 months. The 3 major determinants of prognosis were the blood urea concentration (BUC), the haemoglobin concentration ([Hb]), and the clinical performance status. Three prognostic groups based on these determinants were specified. The groups contained 22%, 56% and 22% of the patients and gave 2-year survival probabilities of 76%, 50% and 9% respectively. Patients in the good-prognosis group had a BUC less than or equal to 8 mM. [Hb] greater than or equal to 100 g/l, and no or minimal symptoms. Those in the poor-prognosis group had either [Hb] less than or equal to 75 g/l or a BUC greater than 10 mM and restricted clinical activity. Patients who had combinations of the 3 determinant features which excluded them from these 2 groups were classified into an intermediate prognosis group.

Aged↗
PubMed1982

Anaemia in patients with myelomatosis.

Twenty-four untreated patients with myelomatosis were studied in order to characterize their anaemia, using standard haematological and ferrokinetic techniques, together with measurements of circulating erythropoietin, erythropoietin sensitivity of marrow cultures and in vitro measurements of haem synthesis. There is a reduction in total erythroid output by the marrow, together with a minor degree of plasma expansion. In patients with normal renal function there is an appropriate increase in erythropoietin in response to anaemia, but in a few cases there may be reduced response of CFU-E to the hormone in vitro. No abnormality of iron status or haem synthesis was found. One case of folate deficiency was discovered.

Adult↗
PubMed1985

Objective evaluation of the role of vincristine in induction and maintenance therapy for myelomatosis. Medical Research Council Working Party on Leukaemia in Adults.

In the Medical Research Council's IVth trial in Myelomatosis the possible benefit of adding vincristine to first line treatment with intermittent melphalan and prednisone has been assessed. This was analysed in 530 patients who were randomly allocated to receive vincristine or not. Survival was not improved by the addition of vincristine. A total of 268 patients reached plateau phase on first line therapy. Of these 226 patients were rerandomised either to continue receiving first line therapy for a further year or to cease therapy. At the present time there is a slight but not significant survival advantage in the group which received no further treatment on reaching plateau.

Adult↗
PubMed1985

Urinary pseudouridine excretion in myelomatosis.

Urinary psi excretion is independent of the main indices of tumour activity in myelomatosis (serum paraprotein, serum beta 2-microglobulin, serum creatinine and urinary light chain production). The mean (+/- s.d.) psi at presentation was 40.7 +/- 22.6 nmol. mumol ucr-1, compared to 25.4 +/- 4.8 nmol. mumol ucr-1 in controls. Urinary psi levels at presentation are significantly related to prognosis, the higher the level the poorer the prognosis. However, when these levels have been stratified according to the corresponding level of serum beta 2m, the level adds little as a prognostic factor.

Adult↗
PubMed1987

A comparison of the incidence of the myelodysplastic syndrome and acute myeloid leukaemia following melphalan and cyclophosphamide treatment for myelomatosis. A report to the Medical Research Council's working party on leukaemia in adults.

Twelve of 648 patients in the Medical Research Council's first two trials in myelomatosis have developed myelodysplasia or acute leukaemia. This corresponds to a 5-year actuarial prevalence of 3% and an 8-year prevalence of 10%. Patients were randomised to treatment with either melphalan or cyclophosphamide and the relative capabilities of these two drugs to cause these conditions were examined as a function of duration of treatment. A significant relationship with length of melphalan treatment was found but no relationship was observed for cyclophosphamide treatment. The amount of melphalan treatment given in various intervals before diagnosis of myelodysplasia or leukaemia was studied and it was found that the amount of treatment in the most recent 3-year period was the most important determinant of risk (P = 0.0001). It is estimated that the risk of haemopoietic neoplasia after 10 years of follow-up is about 3% for each year of melphalan treatment and that much of this risk will occur within three years of the last treatment.

Aged↗
PubMed1981

Radiation-induced myelomatosis.

It is well known that radiation can cause myeloid leukemia. However, no excess of chronic lymphocytic leukemia has been observed. Myelomatosis, like chronic lymphocytic leukemia, is a tumor of B lymphocytes. To determine whether this disease has a radiogenic origin, we surveyed all cohorts of persons exposed to radiation for which data on cancer-related mortality are available. An excess of myeloma was found in most cohorts. However, a striking deficit was found in two groups irradiated intensely for uterine neoplasms (three cases observed, 10.71 expected; P = 0.012). All other groups combined had a highly significant excess (50 observed, 22.21 expected; P = 2 X 10(-7)). The largest relative risk appeared among persons receiving internal doses of alpha-particles (14 observed, 3.24 expected; P = 2 X 10(-5)), but a significant excess (13 observed, 6.33 expected; P = 0.026) was also found in patients receiving only therapeutic or diagnostic gamma-rays or x-rays. Most cases occurred 15 to 25 years after exposure.

England↗