[Treatment of ITP and AIHA in CVID: A systematic literature review]. / Traitement du PTI et de l'AHAI au cours du DICV : revue systématique de la littérature.

INTRODUCTION: Ten to 15% of common variable immunodeficiencies (CVID) develop auto-immune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Treatment is based on immunosuppressants, which produce blocking effects in the CVID. Our objective was to assess their risk-benefit ratio in these immunocompromised patients. METHODS: We identified 17 articles detailing the treatment of AIHA and/or ITP in patients suffering from CVID through a systematic review of the MEDLINE database. RESULTS: The increased infectious risk with corticosteroids does not call into question their place in the first line of treatment of ITP and AIHA in CVID. High-doses immunoglobulin therapy remain reserved for ITP with a high risk of bleeding. In second-line treatment, rituximab appears to be effective, with a lower infectious risk than the splenectomy. Immunosuppressants (azathioprine, methotrexate, mycophenolate, cyclophosphamide, vincristine, ciclosporine) are moderately effective and often lead to severe infections, meaning that their use is justified only in resistant cases and steroid-sparing. Dapsone, danazol and anti-D immunoglobulins have an unfavorable risk-benefit ratio. The place of TPO receptor agonists is still to be defined. The establishment of immunoglobulin replacement in the place of immunosuppressants (except for short-term corticotherapy) or splenectomy appears to be essential to limit the risk of infections, including in the absence of previous infections. CONCLUSION: The presence of CVID does not mean that it is necessary to give up on corticosteroids as a first-line treatment and rituximab as a second-line treatment for AIHA and ITP, but it should be in addition to immunoglobulin replacement. A splenectomy should be reserved as a third-line treatment.

[Innate immunity primary immunodeficiencies and infections]. / Déficits immunitaires héréditaires de l'immunité innée et infections.

The diagnosis of primary immunodeficiency diseases (PIDs) is important for the early and adaptive care of patients and their families. Among the various known PIDs, a number of them concern the innate immune system, which involve a set of cells and mechanisms involved in the host defense by a nonspecific and fast response. The majority of patients with innate immunity defects have a predisposition to one isolated type of infection (bacterial, viral, or fungal), dependent on the genetic defect involved. This article describes the different PIDs involving innate immunity and the immunological investigations allowing for their diagnosis.

Clusterin facilitates apoptotic cell clearance and prevents apoptotic cell-induced autoimmune responses.

Clusterin (Clu), an extracellular chaperone, exhibits characteristics of soluble innate immunity receptors, as assessed by its ability to bind some bacteria strains. In this study, we report that Clu also binds specifically to late apoptotic cells but not to live, early apoptotic, or necrotic cells. Histones, which accumulate on blebs during the apoptotic process, represent privileged Clu-binding motifs at the surface of late apoptotic cells. As a consequence, Clu potentiates, both in vitro and in vivo, the phagocytosis of late apoptotic cells by macrophages. Moreover, the increased phagocytosis of late apoptotic cells induced by Clu favors the presentation and cross-presentation of apoptotic cell-associated antigens. Finally, we observed that, in a model of apoptotic cell-induced autoimmunity, and relative to control mice, Clu(-/-) mice develop symptoms of autoimmunity, including the generation of anti-dsDNA antibodies, deposition of immunoglobulins and complement components within kidneys, and splenomegaly. These results identify Clu as a new molecule partner involved in apoptotic cell efferocytosis and suggest a protective role for Clu in inflammation and autoimmune diseases.

[Interest of a sterilised anti-burning gel in radiation dermatitis: results of a comparative study]. / Intérêt d'un gel stérile anti-brûlure dans les radiodermites : résultats d'une étude comparative.

Trial objectives were to assess effectiveness and tolerance of sterilized Avène thermal spring water anti burning gel (ATSW gel) in prevention of radiation dermatitis in adults irradiated (6 weeks) for breast (n=61) or head and neck (n=8) cancer. Patients included in this open labelled, 2 parallel groups, multicentric study, were randomly assigned to apply five times daily for ten weeks either the Avène spring water gel (n=35) or trolamine cream (n=34). The median of emergence of the first objective radiation dermatitis signs was 31 days in the ATSW gel group and 29 days in the control group (p=0,924). The median incidence for pruritus in patients of the ETA gel group was 46 days versus 27 days (p=0,028) and 44 days versus 24 days for pain (p=0,426). Global efficacy was <> in 46,7 % of patients of the ETA gel group versus 17,2 % (p=0,029) and tolerance was <> in 65,5 % of the ETA gel group versus 40,7 % (p=0,107). No radionecrosis was observed. In conclusion, the efficacy of the Avène spring water gel was similar to the control product in prophylaxis of radiation dermatitis. Tolerance was better and the pruritus was significantly more delayed in patients who applied the Avène spring water gel than in controls.

Combined chemotherapy-radiotherapy in advanced Hodgkin's disease: results of a prospective clinical trial with 70 stage IIIB-IV patients.

PURPOSE: To evaluate two regimens of chemotherapy followed by high dose total or subtotal nodal irradiation in advanced Stages of Hodgkin's disease. METHODS AND MATERIALS: From October 1980 to September 1985, 70 patients with Hodgkin's disease, with clinical Stages IIIB (35 cases) and IV (35 cases) were treated with combined modality therapy. Patients were randomly assigned to receive four cycles of chemotherapy, mechlorethamine, vincristine, procarbazine and prednisone (MOPP) versus the same regimen alternating with adriamycin, bleomycin, vinblastine and dacarbazine, ABVD-derived regimen, followed by high-dose (40 Gy) total or subtotal nodal irradiation. Because of partial response, 13 patients (18.5%) got additional chemotherapy (1-4 cycles). RESULTS: After chemotherapy, 49 patients (70%) achieved complete remission or good partial response and 15 patients (21.5%) partial response. Five primary failures (7%) and one death (1.5%) occurred. After combined modality therapy, 59 patients (84%) achieved complete remission, one patient partial response (1.5%) and eight patients (11.5%) failed to primary treatment. Two toxic deaths (3%) were observed during initial treatment. There was no significant difference in response rates between MOPP/radiotherapy and MOPP/ABVD/radiotherapy. Nine patients relapsed (15%). A total of 21 patients died, 13 because of Hodgkin's disease and eight from other causes. High dose total or subtotal nodal irradiation following four courses of chemotherapy was feasible, although hematological toxicity grade > or = 2 (World Health Organization) was observed in one-third of the patients, particularly in patients aged over 40. The median duration of follow-up was 75 months. Actuarial survival curves indicate a 8 years disease-free survival and survival of 70% and 65% respectively, without any significant difference between the two regimens. Because of hematological toxicity, the percentage of planned full treatment was lower in MOPP/radiotherapy regimen. CONCLUSION: These results lead to recommend the alternating regimen. Patients restaged as poor responders after initial chemotherapy did not survive for long. More intensive treatment is now proposed for this subgroup of patients.

Hodgkin's disease, clinical stages I, II A-B and IIIA. Results of brief chemotherapy followed by irradiation.

From October 1980 to September 1985, 152 patients with Hodgkin's disease (HD) in clinical stages (CS) I, II A-B and IIIA were treated with combined modality therapy (CMT): brief chemotherapy (CT) followed by radiotherapy (RT). CS IA and IIA cases received 3 cycles of MOP, while CS IB, IIB and IIIA cases were randomly assigned to receive MOP or MOP alternating with ABVD (4 cycles). Irradiation was delivered according to the areas initially involved and response to CT. CS I and II subjects in complete remission (CR) received localized fields, whereas CS I, II and IIIA subjects in partial remission (PR) received extended fields, with subtotal nodal RT for CS I and II and CS IIIA without pelvic involvement and total nodal RT for CS I and II below diaphragm and CS IIIA with pelvic involvement. Following CT, CR was 60% (IA-IIA: 71.5%, IB-IIB: 37%, IIIA: 55.5%), PR 35.5% and failure 4.5%. After CT and RT, CR was 98% (IA-IIA: 100%, IB-IIB: 95.5%, IIIA: 94.5%). Responses were similar using MOP and MOP/ABVD regimens. Fifteen patients relapsed (10%) and 15 died, 11 due to HD and 4 due to other causes, while after 7 years overall survival and relapse free survival were respectively 87% and 82% (IA-IIA 90% and 85%, IB-IIB: 80% and 80%, IIIA: 87% and 62%). Results were equivalent irrespective of CT regimen.(ABSTRACT TRUNCATED AT 250 WORDS)

Radiation-induced lung injuries: a survey by computed tomography and pulmonary function tests in 18 cases of Hodgkin's disease.

Eighteen patients with Hodgkin's disease received chemotherapy and 40 Gy mantle-field irradiation. Radiation-induced lung injuries were studied 5 times during one year for each patient by chest x-ray, CT examination of the thorax and pulmonary function tests. Homogeneous and inhomogeneous densities developed within the radiations ports. They were more often and more easily detected by CT than by chest x-ray (39% vs 11% at the end of the irradiation). CT changes suggested that homogeneous lung density increase and lung nodules corresponded to the radiation pneumonitis phase, also that linear aspects and/or lung condensation aspects corresponded to irradiation-induced lung fibrosis. The displacement of the vessels and the fissures were seen more precisely by CT than by chest x-ray. There was a highly significant correlation between the number of modified areas and the increase in the coefficient of retraction (p less than 0.001).

[Early post-radiation pericardial changes in Hodgkin's disease. Value of x-ray computed tomography]. / Modifications péricardiques post-radiques précoces au cours de la maladie de Hodgkin. Intérêt de la tomodensitométrie.

Eighteen patients with mediastinal Hodgkin's disease treated with chemotherapy first, then irradiation were investigated during 1 year by means of 5 CT and radiological examinations of the chest. Four months after irradiation pericardial thickening was detected in 60 p. cent of the patients. The authors underline the high frequency, early occurrence and usually spontaneous resolution of radiation-related pericarditis without clinical or radiological signs.

[Supratentorial glioblastoma in adults. Therapeutic results]. / Glioblastomes sus-tentoriels de l'adulte. Résultats thérapeutiques.

This retrospective study concerns 55 adult patients with supratentorial glioblastoma. The tumours were treated by complete or partial surgical excision whenever possible (31 cases), radiotherapy (22 cases) in doses of 60 Gy over 6 to 7 weeks (40 Gy with telecobalt and 20 Gy with superimposed electrons) and multiple chemotherapy (10 cases) with VM26 and CCNU or BCNU. Although the number of patients in some categories was too small for statistical evaluation, the results obtained were in agreement with those found in the literature and indicative of what can be expected. In patients with inoperable tumours the mean survival was increased from 2 to 8 months by radiotherapy or chemotherapy given separately, and from 2 to 9 or 10 months only when these two methods were combined. The mean survival of patients with partial tumoral excision was 2.5 months extended to 10 months after post-operative radiotherapy; one patient in this group who received both radiotherapy and chemotherapy is still alive after more than 5 years. In patients with macroscopically satisfactory excision, the 12 months good quality survival obtained by surgery was apparently prolonged to 22 months with radiotherapy; 3 of these patients who had both radiotherapy and chemotherapy after surgery are alive and in good condition after 15, 16 and 28 months respectively.

[An unusual form of neoplastic lymphangitis in an irradiated field]. / Une forme particulière de lymphangite néoplasique en zone irradiée.

The authors describe an exceptional form of neoplastic lymphangitis occurring in old people some years after surgery and irradiation of a cancer (parotid, breast), the special character of which is that it draws exactly the fields of irradiation. This lymphangitis is at first smooth and later covered with neoplastic nodes which join together. Evolution is in any case the death after some weeks or months. Different pathogenetic hypotheses are discussed, involving the mechanisms of biology of cancer and radiations.

[Effects of mantle-field irradiation and chemotherapy on respiratory function (author's transl)]. / Conséquences de l'irradiation médiastinale et de la chimiothérapie sur la fonction respiratoire.

In 15 patients with mediastinal Hodgkin's disease treated with chemotherapy followed by mantle-field irradiation respiratory function tests performed before and after irradiation showed a decrease in vital capacity and FEV1 as compared with pretreatment values. The decrease was maximum at 4 months and regressed over one year. No significant changes were observed in blood gas measurements. Respiratory symptoms, which were frequent after irradiation, almost completely disappeared within one year. The therapeutic combination had no cumulative adverse effect on respiratory function.

[Results of the radiotherapy in the non Hodgkin's lymphomas treatment (author's transl)]. / Résultats de la radiothérapie dans le traitement des lymphomes non hodgkiniens (ex-lympho-réticulo-sarcomes).

In our 98 patients all treated by association chemotherapy + cobaltherapy, 37 cases were stages III and IV and 71 cases were of diffuse pathology classification. Meanwhile the results were not so poor: 66% at 5 years survival (all stages) and 69% for stages I and II. The curves of survival rate generally stabilize at 7 years. The prognostic depend of the pathology class: diffuse LBS and diffuse HBS have an evolution similar to acute leukemia (44.5% and 19% at 7 years survival), on the opposite nodular LBS, diffuse LCS have 100% survival at 7 years. Diffuse cases have visceral relapses (liver, bone marrow, Waldeyer ring) or generalized lymphatic involvement; nodular cases have generally localized and only lymph-nodes relapses. The best results by large fields irradiation suggest a contiguous extension of the disease, but there is also distant lymph nodes relapses. In the nodular cases, relapses can be generally cured by localized radiotherapy. In the diffuse case, we do not know if it is better to give total lymphnode irradiation (or to give more chemotherapy with economic irradiation). 10 gastro-intestinal cases were treated by moving strip technique with excellent tolerance and 67.5% at 5 years survival. These results show that interesting results may be obtained in non Hodgkin lymphomas by association of chemotherapy, with selective and moderately large fields irradiation, even in disseminated cases, even in diffuse cases.