VAD-PECC regimen in the treatment of advanced-stage multiple myeloma.

PURPOSE: This prospective study was undertaken to evaluate the efficacy of combination chemotherapy with alternating cycles of vincristine, doxorubicin, and dexamethasone (VAD) and prednisone, vindesine, carmustine, and cyclophosphamide (PECC) in poor-risk multiple myeloma (MM). PATIENTS AND METHODS: Forty-four patients were previously untreated; 36 had been pretreated with an alkylating agent-containing regimen and had refractory or relapsed MM. All previously untreated patients had a high tumor burden at inclusion (stage III according to the Durie and Salmon classification). Logistic regression and the Cox proportional hazards models were used to assess the association between patient characteristics and response rate and survival, respectively. RESULTS: The overall response rate was 68% for previously untreated patients, compared with 54% for previously treated patients (P = .16). The median survival time for all patients was 28 months: 53 months in previously untreated patients, and 18 months in previously treated patients. Univariate analysis showed that the predictive factors that had a significant affect on survival in the newly diagnosed patients were age, therapeutic response to VAD-PECC, low pretreatment Karnofsky score, high baseline serum beta 2-microglobulin (beta 2M) level, bone marrow impairment, and renal insufficiency at the start of treatment. When these parameters were used as continuous variables in multivariate analysis, three were found to correlate with survival: serum beta 2M, followed by therapeutic response and Karnofsky score. In the previously treated group, only Karnofsky score entered the Cox model. CONCLUSION: These results indicate that combination VAD-PECC chemotherapy is an effective treatment that results in high response rates and long-term survival in advanced MM.

CD5 negative B-cell chronic lymphocytic leukemia: clinical and biological features of 42 cases.

Chronic lymphocytic leukemia cell (CLL) usually (95%) express B-phenotype and the CD5 antigen which is usually present on the surface of normal T cells. However, among B CLL, 7 to 20% do not express CD5. The significance of the lack of CD5 expression remains unclear. We reviewed 42 consecutive CD5- B CLL seen in three French medical centers from 1985 to 1991 and compared them with 79 CD5+ B CLL. Immunophenotype studies were performed using indirect immunofluorescence under light microscopy as well as flow cytometry after 1988. B CLL was considered to be CD5 negative when less than 5% of mononuclear cells expressed CD5 after subtraction of the number of T-cells. Cases with CD5- B CLL had isolated splenomegaly more frequently (p = 2.10(-7)). They frequently expressed a higher level of surface immunoglobulin (S-Ig) or the switch mu/delta phenotype (p = 4.7 10(-2)). The median survival time was not reached but no significant difference between CD5 negative and positive B CLL was observed at the time of our data analysis (p = 0.97). Clinical presentation of CD5- B CLL seems to be different from other forms of B CLL. Although, no conclusion can be reached in terms of prognosis, CLL with low expression of CD5 should be regarded as a subtype of CLL with a different clinical presentation than CD5+ CLL.

[Metastasis of breast cancer to a uterine leiomyoma]. / Métastase d'un cancer du sein dans un léiomyome utérin.

The authors report a case of metastasis of cancer from the breast in a uterine leiomyoma. The metastases into the body of the uterus from extragenital cancers are rare. If they occur they are usually in the myometrium where they are asymptomatic and where the diagnosis is difficult, or in the endometrium where the diagnosis can be made by biopsy after curettage. A few rare cases of metastases in uterine leiomyoma have been reported in the literature. They can be the cause of very sudden increase in size of fibroids with compression of the pelvic organs.

[Erythroderma and multiple cutaneous necrosis revealing a dermatomyositis]. / Erythrodermie et nécroses cutanées multiples révélant une dermatomyosite.

INTRODUCTION: We report an original case of dermatomyositis associated with neoplasia, which initial clinical expression was erythroderma and multiple cutaneous necrosis. OBSERVATION: A 64-year-old patient was admitted at hospital for erythroderma. He had a diffuse and inflammatory erythema with thrill, periorbital oedema, periungueal telangiectasia and epidermal necrosis. Physical examination also revealed symmetric proximal muscle weakness as well as hepatomegaly. There were biological signs of myolysis. Complementary investigations revealed a liver carcinoma with lung metastasis. The patient first underwent topical corticosteroid treatment, which provided partial improvement of the clinical and biological signs of disease. Thereafter he was treated with prednisone and tamoxifen. Death occurred at home 4 months after the diagnosis. DISCUSSION: Diagnosis of dermatomyositis was definitely set according to the criteria of Bohan and Peter. Epidermal necrosis, present in our observation, occur classically in dermatomyositis where they are a predictive factor of association with neoplasia. Other recognized predictive factors are the age of the patient and persistent itching. Erythroderma linked to dermatomyositis is a very unusual event: 4 cases have been reported in the literature, only one of which was associated with cancer. On the other hand, numerous cases of diffuse erythema were reported, which are close to erythroderma. It is not possible to set out that this clinical form is a factor of bad prognosis of dermatomyositis. As a matter of fact, in the published cases, one does not find more frequent deceases, pejorative signs or increased frequency of association with neoplasia.

A case of association between hepatocellular carcinoma and porphyria variegata.

BACKGROUND: A correlation between acute intermittent porphyria or porphyria cutanea tarda and hepatocellular carcinoma (HCC) has been noted in several studies, but only one case of association between HCC and porphyria variegata has been reported. We therefore report another case of association between HCC and porphyria variegata. METHODS: A 54-year-old nurse with familial porphyria variegata who developed an HCC was studied. The diagnosis of porphyria variegata was made in the course of a familial survey by means of measuring lymphocyte protoporphyrinogen oxidase activity, at a time when the patient had no symptoms. Eighteen years later the patient presented with a firm enlargement of the liver. RESULTS: Histologic examination showed a well-differentiated HCC. The diagnosis was confirmed by positive immunostaining for alpha-foetoprotein antibodies. DISCUSSION: Sixteen months after surgical resection of the HCC the patient was still alive.

[Chronic arthritis in palmo-plantar pustulosis. Bacterid of Andrews]. / Arthrites chroniques des pustuloses palmo-plantaires. Bactérides d'Andrews.

Report of a 30 years old man story pustulosis palmaris et plantaris, spondylodiscitis L5-S1, right sterno-clavicular arthritis, right sacro-iliitis, and pubic symphysitis. Multiple bacteriologic investigations did not discover any infectious articular process. Histologic articular findings were inflammatory pattern, non steroid anti-inflammatory drugs corrected the arthritis. Anterior thoracic arthropathies are quasi-constant. Inflammatory spondylodiscitis are frequent, while sacro-iliac involvement is infrequent, this pattern distinguishing pustular arthro-osteitis from classic pelvispondylitis. Radiologic lesions are peculiar by predominance on one articular side at each site. Nosology is discussed. Slow infection by non virulent germs has been suspected , but seems improbable. This affection seems close seronegative spondylo-arthropathies and reactive arthritis, like acne conglobata arthritis for instance. Usual absence of HLA-B27 antigen must be noted.

[Geographic distribution of multiple myeloma in the Loiret region. 10-year retrospective epidemiological survey]. / Répartition géographique du myélome multiple dans le Loiret. Enquête épidémiologique rétrospective sur dix ans.

Suspecting the presence of a myeloma focus in a forest area of the Loiret department, we have undertaken a retrospective epidemiological survey on this disease. Three surveys were conducted, between 1972 and 1982: the first one among general practitioners; the second one was carried out from immuno-electrophoreses performed at the University Hospital in Orleans; the third survey was carried out from reports on the medical causes of deaths. The mean number of myelomas in the Loiret (0.029 p. cent) is comparable to the national mean (0.025 p. cent), with a sex ratio of 47.09 p. cent of men, also quite similar to the national sex ratio (46.7 p. cent). The different districts were classified according to the percentages observed in "neutral areas" (mean plus or minus a typical variation), "cold areas" (between one and two typical variations below the mean) and "hot areas" (between one and two typical variations above the mean). These results do not permit to ascertain the existence of a geographical focus of myelomas in Loiret but require a more in-depth study of "hot areas".

[Value of the measurement of intra-articular lactates. Contribution to the diagnosis of septic arthritis. Interpretation of 370 evaluations]. / Intérêt de la mesure des lactates intra-articulaires. Apport au diagnostic des arthrites septiques. Interprétation de 370 évaluations.

This retrospective study interprets the results of 370 evaluations of the lactate level of joint fluid. 5 groups were established: joint fluid from cases of mechanical (114) and microcrystalline (67) joint diseases, from inflammatory arthritis (149) and from septic arthritis (40). There was a significant difference between the lactate level in fluid from septic joints and that in the other groups. However, there was no significant difference between the rheumatoid joint fluid and the septic joint fluid. The increased level of intra-articular lactate can therefore be considered to be an argument in favour of the diagnosis of septic arthritis, but there is a certain rate of false positives (particularly in the case of rheumatoid arthritis) and false negatives, which require a very careful interpretation of the test. In cases of inflammatory arthritis with a cell count of more than 10,000 per mm3, there is a correlation between the white cell count and the lactate level. This relation is not observed in the case of septic arthritis. The authors did not detect any difference in the levels of intra-articular lactate according to the nature of the infecting organism. They studied 4 specimens of joint fluid from gonococcal arthritis and the levels of lactate were similar to those of other types of septic arthritis.

[Calcium oxalate microcrystalline arthropathy in primary oxalosis]. / Arthropathie microcristalline à oxalate de calcium au cours d'une oxalose primitive.

This paper dealt with the case of a 53 years old man, affected by a chronic renal failure as the initial symptom of a primary oxalosis and treated by hemodialysis three years ago. Two years after the onset of renal failure, the left knee was painful and swollen but no cartilage or bone joint lesion was observed. Presence of intra synovial calcium oxalate crystals suggests that this arthropathy may be related to the primary oxalosis. However the role of other calcium salts under identification evidenced by synovial electron microscopy (apatite ? pyrophosphate ?) is discussed.

[Lyme disease. Clinical, biological and developmental aspects. 29 cases in the Orléans region]. / Maladie de Lyme. Aspects cliniques, biologiques et évolutifs. 29 cas en région orléanaise.

The description of Lyme's disease (LD) in 3 stages (like syphilis), has now become classical. 29 cases of LD, between June 1981 and November 1986, have been recorded at the Hospital in Orleans. The first twelve patients that were clinically diagnosed before the serology was introduced in France, have been recalled in order to search for possible late forms and assay their antibody level of anti-Borrelia burgdorferi. The preponderance of neurological forms (22 cases) and the scarcity of peripheral and/or arthralgic arthritis (8 cases) are emphasized by this study. Radiculalgias (18 cases in 22 meningo-radiculitis), especially in the lower extremities (14 cases), required hospitalization in rheumatology in 21 instances. Spinal algias (16 cases), were only isolated, at the time of hospitalization, in one case. The intense and nocturnal nature of the radiculalgias and spinal algias was noted in 17 of 18 cases, and 16 of 16 cases, respectively. Two atrio-ventricular blocks required hospitalization in cardiology. The long-term follow-up (3 to 5 years) of 12 patients revealed only 2 cases of severe asthenia and swelling at the puncture site in one case. There were no entesopathies nor chronic arthritis. The search for anti-Borrelia burgdorferi antibodies in the serum, was positive 24 times in 28. In the 4 cases where the serology was negative, the samplings were taken after a follow-up of more than three years in 3 instances.(ABSTRACT TRUNCATED AT 250 WORDS)

Multiple myeloma in elderly patients: presenting features and outcome.

Few studies have been performed regarding multiple myeloma (MM) in elderly patients. We report a retrospective series of 130 unselected patients with MM aged 75 yr or more at diagnosis. Presenting features were identical to those reported in younger patients, except for a higher rate of infection. Heavy comorbidity was characteristic of unselected geriatric patients. Ninety-four patients received conventional chemotherapy. The response rate was 62%. Treatment toxicity was mild. Median survival was 22 months. Durie-Salmon (DS) clinical stages II and III MM were severe and often led to death, while significantly more patients with DS stage I MM died from unrelated causes (p<0.0001). Univariate analysis showed that age > or = 85 yr, performance status > or = 2, creatinine level > or = 120 micromol/l, beta 2 microglobulin level > 4 mg/l, C-reactive protein level > 6 mg/l, platelet count < 100 x 10(9)/l, presence of infection and lack of response to chemotherapy were adverse prognostic factors for survival. In Cox multivariate regression analysis, age > or = 85 yr (p<0.0001), performance status > or = 2 (p<0.0001) and creatinine level > or = 120 micromol/l (p<0.0001) were independent factors in predicting short survival. This study provides evidence that in patients with symptomatic MM age should not be considered as a major obstacle to active treatment. Prospective clinical trials are needed in this population of patients and should include an assessment of quality of life.