High-dose chemotherapy with autologous bone marrow transplantation for refractory metastatic gestational trophoblastic disease.

A patient with gestational trophoblastic disease failed to respond to all standard multiple-agent chemotherapy and had progressive uterine disease. Total hysterectomy also proved ineffective. We therefore administered high-dose cyclophosphamide, etoposide, and melphalan with autologous bone marrow support. Severe marrow suppression followed but a complete remission was achieved and the patient remains free of disease 3 years after the completion of therapy. This result seems to confirm the value of using high-dose multiagent therapy to optimize the dose-effect of therapy regimens on drug-refractory gestational trophoblastic disease.

[Endomyocardial biopsy in the monitoring of treatment with anthracyclines. Value of the assay of lactate dehydrogenase and its isoenzymes]. / La biopsie endomyocardique dans la surveillance des traitements par anthracyclines. Intérêt du dosage de la lacticodéshydrogénase et de ses isoenzymes.

Determination of intramyocardial lactate dehydrogenase (LDH) and its isoenzymes (LDH 1 to 5) has been conducted on biopsy material obtained from the right ventricle in 18 patients who had received the theoretical maximal dose of anthracyclines. Total LDH activity, expressed in mU/mg of myocardial protein is higher (852.61 +/- 87.98) than in subjects with good left ventricular function (334 +/- 208), p less than 0.02. The activity of LDH 1 is decreased in the treated group, whereas that of LDH 3,4 and 5 is raised: LDH 1 = 41.95 +/- 7.25 per cent instead of 47.76 +/- 7.93 per cent (p less than 0.03); LDH 3 = 15.34 +/- 5.09 per cent instead of 9.18 +/- 4.56 per cent (p less than 0.05). The H/M ratio (H = fractions of heart isoenzymes; M = fractions of muscle isoenzymes of LDH) is decreased in the treated group: H/M = 4.06 +/- 1.13 instead of 5.30 +/- 1.80 (p less than 0.01). When the histological lesions are minimal (stage 1 or 1.5 of Billingham), the H/M ratio is not altered: 4.92 +/- 1.02. When the lesions attain or exceed stage 2, the H/M ratio is significantly decreased: 3.74 +/- 0.82. These results, which indicate an increase in anaerobic metabolism, add quantitative information to the histological study.

[Right intraventricular biopsy. Indications and results. 116 cases]. / Biopsie endoventriculaire droite. Indications et résultats. Cent seize observations.

The indications for endomyocardial biopsy were evaluated from 116 consecutive cases. The diagnostic value of this invasive but well tolerated procedure was in agreement with data from the literature. An accurate diagnosis, unforseeable in 8% of the patients, was established in 12%. The diagnosis of apparently primary myocardiopathy with ventricular dilatation was confirmed in 45 out of 59 cases; there were 3 cases of myocarditis, 3 cases of restrictive cardiopathy (haemochromatosis, fibroplastic endocarditis) and 1 case of hypertrophic cardiopathy. No tissue abnormality was noted in 6 cases. An accurate diagnosis was obtained by biopsy in 1 case of "eosinophilic lung" without overt cardiac involvement. In malignant diseases treated with anthracyclines in doses reaching maximal theoretical total dosage (30 patients), severe tissue lesions were present in 10% of the cases, incipient haemochromatosis in 16.6% and subendocardial fibrosis in 3.3%. However, total doses of up to 600 mg/m2 could be administered to 90% of the patients. Myocardial lesions could be demonstrated in 1 of 2 patients with collagen disease. Endomyocardial biopsy therefore seems to be justified in myocardiopathies with ventricular dilatation, in some collagen diseases with a tendency to cardiac involvement and to monitor treatment with anthracyclines in total doses higher than the theoretical maximum dosage.

[The hemophilic femoropatellar joint]. / L'articulation fémoro-patellaire hémophilique.

The knee is the joint most frequently involved in haemophilia. Femoro-tibial damage in chronic arthropathies has been perfectly described. By contrast, lesions of the patella (with the exception of "squaring") have been reported in only a few scanty publications. An attempt was made to define the characteristics by clinical and radiological study (including in addition to lateral films, an analysis of 30, 60 and 90 degrees axial views, even though these were often made difficult by flexion deformity and joint limitation). Femoro-patellar involvement was seen to be very frequent with various lesions: rarely "squaring", and more often patellar erosions or femoro-patellar incongruence (either pre-existing dysplasia or secondary to haemarthrosis) and sometimes actual incarceration (described as "locked patella" by anglo-saxon authors). These concepts are left to have practical consequences in the management of physiotherapy, synoviorthesis and surgery.

Serum beta-2-microglobulin in multiple myeloma: relation to presenting features and clinical status.

Serum beta-2-microglobulin (B2m) levels were measured in 78 patients with multiple myeloma (MM) and were compared with values for normal individuals and patients with benign monoclonal gammopathies (BMG). Serum B2m levels and values corrected for renal function were significantly higher in patients with MM at time of diagnosis than in normal individuals (P less than 0.001) and were highly correlated with the total body burden of myeloma cells as derived from the staging system of Durie and Salmon. However there were no significant differences between values for BMG and low-mass MM. For patients evaluated following induction chemotherapy, there was also a clear correlation between serum B2m levels and the magnitude of tumor regression or progression (P less than 0.05). During the plateau-phase, serum B2m levels remained very stable and highly correlated with the residual tumor mass (P less than 0.001). It was concluded that (1) B2m was not a reliable marker to distinguish between BMG and low-mass MM and (2) B2m was a valuable marker for assessing initial tumor mass of patients with MM and response to chemotherapy (especially the plateau-phase), above all in patients with urine or low-serum monoclonal component levels.

[Bloom's syndrome. Discussion of the diagnosis concerning two cases of terminal leukemia in a sibship (author's transl)]. / Le syndrome de Bloom. Discussion du diagnostic à propos de deux cas dans une fratrie avec leucémie terminale.

Two brothers developed acute leukemia, one at the age of 7 months and the other at the age of 14 months. Both suffered from a staturoponderal retardation and the same malformation syndrome. The karyotype carried out only on the second child revealed breaks and chromatid changes. A diagnosis of Fanconi's anaemia can be discarbed since no blood cytopenia preceded the leukemia. Finally, the diagnosis of Bloom's syndrome prevailed despite the absence of telangiectatic erythema and the atypical chromosomal anomalies.