[French recommendations on control measures to reduce the infectious risk in immunocompromised patients]. / Quelles mesures pour maîtriser le risque infectieux chez les patients immunodéprimés ? Recommandations formalisées d'experts.

The increase use of immunosuppressive treatments in patients with solid cancer and/or inflammatory diseases requires revisiting our practices for the prevention of infectious risk in the care setting. A review of the literature by a multidisciplinary working group at the beginning of 2014 wished to answer the following 4 questions to improve healthcare immunocompromised patients: (I) How can we define immunocompromised patients with high, intermediate and low infectious risk, (II) which air treatment should be recommended for this specific population? (III) What additional precautions should be recommended for immunocompromised patients at risk for infection? (IV) Which global environmental control should be recommended? Based on data from the literature and using the GRADE method, we propose 15 recommendations that could help to reduce the risk of infection in these exposed populations.

[Efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) on the neuropathic manifestations in POEMS syndrome]. / Efficacité à moyen terme de l'autogreffe de cellules souches hématopoïétiques périphériques sur la polyneuropathie du syndrome POEMS: expérience chez 5 cas.

INTRODUCTION: POEMS syndrome (polyneuropathy, organomegaly, endocrynopathy, M-protein, and skin changes) is a rare multisystem disease associated with plasma cell dyscrasia. The efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) reported in case series has been mainly based on hematologic criteria and clinical recovery of peripheral neuropathy dysfunctions but has not been specifically evaluated. This retrospective study aimed to analyze the efficacy of auto-PBSCT on disability and electrophysiological patterns in patients with POEMS syndrome. METHODS: Five patients presenting with POEMS syndrome received auto-PBSCT. Disability was evaluated before treatment and at 6 and 12 months using the Overall Neuropathy Limitation Scale (ONLS) and MRC sumscore of 28 muscles. Nerve conduction studies were performed before and one year after treatment, on median, ulnar, fibular and tibial nerves. RESULTS: Mean age was 60.6 years (49-70). Disease duration between first symptoms and auto-PBSCT was 15.4 months (2-33). Before auto-PBSCT, mean ONLS score was 4.2 (1-10) and mean MRC sumscore 115.8/140 (74-140). At M6, mean ONLS score decreased and mean MRC sumscore increased; both were improved in all patients at M12: mean ONLS score 3 (range 0-8) at M6 and 2.2 (range 0-7) at M12; mean MRC sumscore 118/140 (77-140) at M6 and 122.4/140 (80-140) at M12. Significant recovery in electrophysiological patterns was observed in all patients on ulnar and median nerves: before-after treatment differences were observed for motor conduction velocities (34.41 vs. 45.47 m/s; P<0.001), distal CMAP amplitudes (5.04 vs. 5.96 mV; P=0.004), and sensory conduction velocities (43.20 vs. 49.20 m/s; P=0.001). Distal CMAP amplitude remained low in fibular and tibial nerves (0.41 vs. 0.17 mV). CONCLUSIONS: Clinical and electrophysiological improvement is obvious in POEMS syndrome peripheral neuropathy within one year after treatment with auto-PBSCT, undoubtedly resulting from extensive remyelinisation and axonal regeneration. Further studies are required to examine long-term outcome in patients with POEMS syndrome given auto-PBSCT.

Total genomic alteration as measured by SNP-array-based molecular karyotyping is predictive of overall survival in a cohort of MDS or AML patients treated with azacitidine.

Metaphase cytogenetics (MC) has a major role in the risk stratification of patients with myelodysplastic syndromes (MDSs) and can affect the choice of therapies. Azacitidine (AZA) has changed the outcome of patients with MDS or acute myeloid leukemia (AML) unfit for intensive chemotherapy. Identification of patients without the benefit of AZA would allow AZA combination or other drugs in first-line treatments. New whole-genome scanning technologies such as single nucleotide polymorphism microarray (SNP-A)-based molecular karyotyping (MK) improve the risk stratification in MDS and AML. Maintenance of genomic integrity is less than three megabases (Mbs) total disruption of the genome correlated with better overall survival (OS) in patients with lower-risk MDS. In this SNP-A study, we aimed at defining a cutoff value for total genomic copy number (CN) alterations (TGA) influencing the median OS in a cohort of 51 higher-risk MDS/AML patients treated with AZA. We observed that the relative risk of worse OS increased >100 Mb of TGA, as detected by SNP-A-based MK (8 and 15 months respectively, P=0.02). Our data suggest that precise measurement of TGA could provide predictive information in poor and very poor revised International Prognostic Scoring system (IPSS-R) patients treated with AZA.

[Six cases of pulmonary MALT lymphoma: a heterogeneous therapeutic management]. / Six cas de lymphomes pulmonaires de type MALT : une prise en charge thérapeutique hétérogène.

Pulmonary mucosa-associated lymphoid tissue lymphomas (PMALT) account for around 1% of lymphomas. Clinical and radiological presentations, and the treatment of six PMALT were collected from 1993 to 2008. All patients received chemotherapy before disease progression. Two patients had a lobectomy and one received thoracic radiotherapy. In 2008, all the patients were alive and three were in remission. A "watch and wait" strategy is widely accepted for stable, asymptomatic patients and patients with low tumour mass. Surgery may be proposed for symptomatic patients who have localised PMALT. When a chemotherapy treatment is to be suggested, chlorambucil-based chemotherapy is preferred. There may be room for rituximab alone or in combination, but this remains to be precisely defined. Several larger studies are currently ongoing to assess the role of monoclonal antibodies and chemotherapy in MALT lymphomas. Subgroup analysis should help us to define the optimal treatment for PMALT.

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.

Human trichinellosis due to Trichinella britovi in southern France after consumption of frozen wild boar meat.

Six patients were infected with Trichinella britovi in southern France following consumption of frozen wild boar meat, which had been frozen at -35 degrees C for 7 days. Microscopic examination of a sample of frozen wild boar muscle revealed the presence of rare encapsulated Trichinella larvae, identified as T. britovi. People eating wild boar must follow individual prophylactic rules such as efficient cooking of meat (at least 65 degrees C at the core for 1 minute) as recommended by the International Commission on Trichinellosis, or freezing exceeding four weeks at -20 degrees C.

In the era of highly active antiretroviral therapy, why are HIV-infected patients still admitted to hospital for an inaugural opportunistic infection?

OBJECTIVES: To identify factors related to delayed testing, and delayed or interrupted care-seeking or treatment uptake, among HIV-infected patients. DESIGN: HIV-infected patients hospitalized for an opportunistic infection (OI) cases were included in a prospective study and compared with controls matched by age and sex who had regular follow-up and treatment. Patients were asked to complete a questionnaire about their therapeutic itinerary and their socioeconomic, psychological and medical characteristics. RESULTS: Seventy patients were matched with 140 controls. According to their therapeutic itinerary prior to admission, cases were subdivided into four groups among which three will be more particularly studied: nontested patients (NT) (24%; n=17), known HIV-infected patients with no medical follow-up (NF) (30%; n=21); and noncompliant patients (NC) (36%, n=25). Characteristics of NT and NF patients included a predominantly sexual mode of contamination (P=0.01), continuing occupational activity (P=0.01) despite a low mean Karnofsky index (P=0.001) and unfavourable virological and immunological parameters. NT patients displayed a low degree of anxiety, and lacked awareness concerning risk of contamination and HIV-related symptoms. HIV-status announcement (P=0.04) and the benefits of medical follow-up (P=0.05) were less favourably perceived by NF patients than by controls, and were associated with a high degree of anxiety in NF patients. NC patients had a weaker commitment to follow-up and treatment, and more frequent treatment discontinuation associated with a higher rate of interruption of follow-up in a context of social difficulties. CONCLUSIONS: Patients ignorant of their HIV status, patients NF and NC have very specific characteristics. More appropriate approaches are needed regarding screening and access to care in order to reduce the incidence of delayed care-seeking.

[Assessment of the habits of physicians involved in the management of HIV-infected patients. A clinical audit on the biological follow-up]. / Pratiques professionnelles de médecins suivant des patients infectés par le VIH. Un audit clinique sur le suive biologique.

BACKGROUND: The aim of this study was to assess the habits of hospital and community-based physicians involved in the management of HIV-infected patients and to measure the gap between their practice and follow-up guidelines. METHOD: The guidelines considered as reference were the 1998 Dormont report. Data were prospectively collected from the medical files of the first 10 HIV-infected patients who presented for an out-patient visit (laboratory tests at initial consultation, type and frequency of follow-up during the previous year, relation between biological data and treatment strategy). RESULTS: 22 physicians (14 hospital-based physicians (HP) and 8 community-based general practitioners (GP) participated in the survey. Initial biological data were available for 211 patients; 45% had tests strictly conforming to the recommendations (HP: 57%, GP 23%; p<0.001). Among patients followed by a GP, the initial biological assessment was adequate in 7% of cases when an opiate substitute was prescribed versus 33% in the absence of opiate substitute prescription (p=0.05). For all patients, syphilis serology was the test most frequently lacking (38%). Among 78 patients with HIV-RNA levels>5,000 copies/ml, 18% did not benefit from a change in treatment. Among the patients treated by a GP, 15% had a three-fold increase in HIV-RNA, compared to their initial measurement. Of these, 3/4 were redirected to a hospital out-patient unit. CONCLUSION: This study highlights the discrepancy between initial laboratory testing and expert recommendations, particularly concerning patients attended by a GP. Improvement in data collection is essential. However, recommendations concerning patients' biological follow-up are applied, with the exception of the delay between the initial prescription or treatment modification and HIV-RNA measurement, which should be shortened.

[Necrobiotic xanthogranuloma , a cutaneous disorder associated with monoclonal gammopathy]. / La xanthogranulomatose nécrobiotique, une histiocytose non langerhansienne à expression cutanée associée à une gammapathie monoclonale.

INTRODUCTION: Necrobiotic xanthogranuloma is a rare cutaneous disorder usually associated with monoclonal gammapathy. We describe two new cases. EXEGESIS: A 70-year-old patient was affected by a monoclonal gammopathy. She presented with a diplopia related to a retro-orbital tumor. The biopsy showed inflammatory lesions. Five years later, inflammatory xanthomatous skin lesions appeared. Biopsy specimens gave the diagnosis of necrobiotic xanthogranuloma. A 70-year-old woman was referred for inflammatory cutaneous lesions. Clinical, biological investigations and skin biopsies led to the diagnosis of cutaneous sarcoidosis associated with monoclonal gammopathy. Four years later, she developed a nephrotic syndrome. New skin biopsy specimens showed a necrobiotic xanthogranuloma. CONCLUSION: Necrobiotic xanthogranuloma is a systemic disease. It is a rare non-Langerhans cell histiocytosis characterized by frequent cutaneous and ophthalmologic lesions and associated with monoclonal gammopathy. To our knowledge, retro-orbital involvement has never been reported in necrobiotic xanthogranuloma. Treatment is difficult.

[Cutaneous Waldenström's macroglobulinemia]. / Localisations cutanées de la maladie de Waldenström.

BACKGROUND: We report the case of a patient in whom the first manifestation of Waldenström' s macroglobulinemia was specific skin lesions, treated with chlorambucil chemotherapy. CASE REPORT: A 76-years old woman was referred to us because of chronic red nodular lesions on her face. A biopsy specimen showed a dense lymphocytic dermal infiltrate and immunohistochemistry identified a monoclonal B lymphoid population with an IgM-kappa phenotype. The patient's disease was diagnosed as Waldenström's macroglobulinemia with cutaneous localization, on the basis of a high level of circulating macroglobulinemia and a lymphoplasmocytic infiltrate in the bone marrow expressing the same monoclonal IgM-kappa as in blood and skin. Treatment with radiotherapy (12 Grays) was unsuccessful. Chlorambucil (16 mg per day, 7 days per month) was then introduced with rapid disappearance of the skin lesions. Neutropenia led to withdrawal of this treatment after 4 courses. The skin lesions relapsed 18 months later and were cured with chlorambucil at a lower dose. DISCUSSION: Specific skin infiltrates have been rarely described during Waldenström's macroglobulinemia. Review of the literature showed eight cases of such lesions treated by chemotherapy with only two successes with oral cyclophosphamide and polychemotherapy (cyclophosphamide, vincristine and CCNU). Chlorambucil was used unsuccessfully three times. We hypothesize that primary resistance to alkylating-agent and the small number of cases of cutaneous Waldenström's macroglobulinemia may explain the poor response to systemic chemotherapy previously reported.