Heterogeneity of t(4;14) in multiple myeloma. Long-term follow-up of 100 cases treated with tandem transplantation in IFM99 trials.

One hundred de novo multiple myeloma patients with t(4;14) treated with double intensive therapy according to IFM99 protocols were retrospectively analyzed. The median overall survival (OS) and event-free survival (EFS) were 41.4 and 21 months, respectively, as compared to 65 and 37 for patients included in the IFM99 trials without t(4;14) (P<10(-7)). We identified a subgroup of patients presenting at diagnosis with both low beta(2)-microglobulin <4 mg/l and high hemoglobin (Hb) >/=10 g/l (46% of the cases) with a median OS of 54.6 months and a median EFS of 26 months, respectively, which benefits from high-dose therapy (HDT); conversely patients with one or both adverse prognostic factor (high beta(2)-microglobulin and/or low Hb) had a poor outcome. The achievement of either complete response or very good partial response after HDT was also a powerful independent prognostic factor for both OS and EFS.

[Yersinia enterocolitica septicemia, iron overload and deferoxamine]. / Septicémie à Yersinia enterocolitica, surcharge ferrique et déféroxamine.

Infections due to Yersinia enterocolitica are usually limited to the bowel. When infection is generalized, the role of iron overload and iron chelation has been discussed. We report the case of a 55 year-old patient with sideroblastic anemia who received repetitive transfusions and deferoxamine for 4 years and heme arginate for 2 months, and who was admitted in our institution for Yersinia enterocolitica sepsis. Treatment by third-generation cephalosporins and aminoglycosides has allowed favorable outcome.

[Acute drug-induced agranulocytosis. Clinical study apropos of 30 patients and evolution of etiologies over 2 decades]. / Agranulocytoses aiguës médicamenteuses. Etude clinique à propos de 30 patients et évolution des étiologies sur 2 décennies.

Between 1982 and 1993, 30 patients were treated for drug-induced agranulocytosis. They did not receive cytotoxic chemotherapy nor radiotherapy during the past 6 months. There is a higher incidence in women (21 females, nine males). Mean age is 59.3 years old. The drug could be found in 25 cases including noramidopyrine five cases, antithyroid drugs four cases, non steroidal anti-inflammatories drugs four cases. Five patients died of infection during agrulocytosis. Sepsis was documented in three cases. We used hematopoietic growth factors in two cases. Neutrophils rose up to 0.5.10(9)/l between 2 to 14 days after the diagnosis and 1.10(9)/l between 3 to 16 days. Time when absolute neutrophil count was less than 0.5.10(9)/l was shorter (p = 0.008) when bone marrow was rich with maturation arrest but with few or no mature forms rather than reduction of granulocytic precursors. By comparison with a similar study made in the same institution between 1971 and 1981, there were fewer cases each year. Drugs involved were not similar: phenicols were not found, reference to noramidopyrine is less frequent. Now antithyroid drugs is becoming one of the most important etiologies.

[Papilledema and POEMS syndrome. Apropos of a case]. / OEdème papillaire et syndrome POEMS. A propos d'une observation.

The POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin Changes) is often unrecognized by ophthalmologists. We report the case of a 51-year-old female suffering from a POEMS syndrome with a bilateral optic disc edema.

[Paraneoplastic opsoclonus-myoclonus syndrome revealing T-cell lymphoma]. / Syndrome opsoclonus-myoclonus paranéoplasique révélateur d'un lymphome T.

BACKGROUND: Nonrhythmic involuntary ocular oscillations and axial and segmentary myoclonia are associated in the opsoclonus-myoclonus syndrome. In adults, a paraneoplastic origin is generally found. We report the first of opsoclonus-myoclonus associated with non-Hodgkin's lymphoma. CASE REPORT: A 66-year-old woman rapidly developed a typical opsoclonus-myoclonus syndrome within a few hours, presenting vertigo, cerebellous ataxia, multidirectional involuntary ocular movements and non-rhythmic axial and segmentary myoclonia. Brain computed tomography and magnetic resonance imaging demonstrated discrete diffuse anomalies of the white substance predominating in the pons. The cerebrospinal fluid showed discrete lymphocytosis. Antineuron antibodies were negative. No cause could be identified until the development 11 months later of pleomorphic T-cell mediastino-cervical lymphoma. The patient responded moderately to a CHOP regimen which had no effect on the opsoclonus-myoclonus syndrome. Death occurred after a 16-month course due to pulmonary complications. DISCUSSION: Neuroblastoma and infectious causes predominate in opsoclonus-myoclonus syndromes observed in children; in adults, the predominant cause is cancer. Antineuron, anti-Ri and anti-Hu antibodies can be evidenced in some cases, arguing in favor of a paraneoplastic mechanism. Recent reports have evidenced MRI anomalies in the pons and the cerebellum, anatomically well correlated with the opsoclonus-myoclonus syndrome. Besides small-cell bronchogenic anaplastic cancer, the possibility of cancer of the breast and uterus, and both non-Hodgkin and Hodgkin lymphoma should be explored, knowing the cancer develops several month after the opsoclonus-myoclonus syndrome.

[Large crusted ulceration of the scalp: first manifestation of cryptococcosis in an AIDS patient]. / Ulcération étendue du cuir chevelu révélant une cryptococcose chez un malade VIH positif.

BACKGROUND: Cryptococcosis is an infection caused by the yeast-like fungus Cryptococcus neoformans. It primarily occurs in immunocompromised hosts. Crytptococcosis is usually a systemic disease and may be serious if the lungs or the central nervous system are involved. CASE REPORT: We describe a case of cutaneous cryptococcosis without systemic involvement occurring as the first manifestation of AIDS. The skin lesion in our patient consisted of a large crusted ulceration on the scalp. Fluconazole was successful. DISCUSSION: This case is a new observation of primary cutaneous cryptococcosis in a patient with AIDS. Clinical appearance may be misleading; skin lesions may be the primary sign in immunocompromised patients. Rapid treatment and secondary prophylaxis are required.

Impact of high-risk cytogenetics and prior therapy on outcomes in patients with advanced relapsed or refractory multiple myeloma treated with lenalidomide plus dexaméthasone.

This retrospective analysis investigated the prognostic value of del(13) and t(4;14) abnormalities and the impact of prior treatment on outcomes in 207 heavily pretreated patients with relapsed or refractory multiple myeloma (MM) treated with lenalidomide plus dexamethasone. Patients with relapsed or refractory MM who had either earlier received thalidomide or bortezomib, or for whom continuation of these agents was contraindicated, and who had fluorescence in situ hybridization data available were included in the analysis. Patients with relapsed or refractory MM who received treatment with lenalidomide plus dexamethasone in the presence of del(13) and t(4;14) chromosomal abnormalities had lower overall response rates (ORRs) and shorter median progression-free survival (PFS) and overall survival (OS) compared with those who did not have these abnormalities. The results also showed that prior treatment with bortezomib was associated with shorter median PFS and OS. Progression during thalidomide therapy was the only significant independent predictor for OS and that the presence of del(13) and hemoglobin levels <10 g per 100 ml were prognostic factors for ORR and PFS, but not OS, in these heavily pretreated relapsed or refractory MM patients treated with lenalidomide plus dexamethasone.

Tolerability of postexposure prophylaxis with zidovudine, lamivudine, and nelfinavir for human immunodeficiency virus infection.

Tolerability of the combination of zidovudine, lamivudine, and nelfinavir used as postexposure prophylaxis (PEP) for HIV infection was prospectively evaluated among 185 patients at 11 hospitals in eastern France. After exclusion of the 106 persons who discontinued PEP either because the source patient subsequently tested HIV seronegative or because the injury was reassessed as resulting in a low risk for transmission of HIV, 67 (85%) of the patients who received such PEP experienced adverse effects, which led to withdrawal of nelfinavir in 28 (35%) of these patients.