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Introduction Alors que le taux de lymphomes malins non-Hodgkiniens (LMNH) diagnostiques ne cesse d’augmenter dans le monde, le LMNH a localisation orbitaire est une tumeur relativement rare et de diagnostic difficile. Le but de cette etude est d’analyser les cas de LMNH a localisation conjonctivo-orbitaire examines, dans le service d’Ophtalmologie du CHU d’Amiens, au cours de ces 20 dernieres annees. Materiel et methodes Nous avons mene une etude retrospective des cas de lymphomes a localisation conjonctivo-orbitaire diagnostiques dans le Service d’Ophtalmologie d’Amiens entre 1982 et 2002. Les comptes rendus anatomopathologiques de 22 cas ont ete etudies, notamment le mode de survenue, la description clinique, radiologique, le mode diagnostique, les resultats anatomo-pathologiques et le type de traitement de ces tumeurs. Resultats Tous les LMNH etaient de type B. Ils etaient de bas grade de malignite dans 17 cas, isoles dans 22 cas, primitifs, de localisation orbitaire dans 9 cas et de type indolent. Discussion Le clinicien se heurte souvent a des lesions insidieuses dont l’acces a la biopsie est souvent difficile, retardant le diagnostic. Le premier diagnostic differentiel a evoquer est l’inflammation orbitaire chronique anciennement appelee pseudo-tumeur inflammatoire. Seule une biopsie orbitaire permet d’affirmer le diagnostic de LMNH. Conclusion Les nouveaux moyens diagnostiques tels que l’immunohistochimie et la genetique permettent de depister de plus en plus de LMNH, meme si leur histoire clinique peut encore faire errer le diagnostic De plus, des therapeutiques de plus en plus ciblees sur le type immunohistochimique du LMNH semblent donner des resultats tres prometteurs. Des etudes sont encore en cours.

Published data on transplantation in Waldenstrom's macroglobulinemia (WM) are still limited. We present a retrospective multicentric study of 27 WM patients who underwent 19 autologous (median age, 54 years) and 10 allogeneic (median age, 46 years) transplantations. Median time between diagnosis and transplantation was 36 months; 66% of patients had received three or more treatment lines and 72 % had chemosensitive disease. High-dose therapy (HDT) and autologous transplantation induced a 95% response rate (RR), including 10 major responses. With a median follow-up of 18 months, 12 patients are alive at 10 to 81 months and eight are free of disease progression at 10 to 34 months. The toxic mortality rate (TRM) was 6%. Allogeneic transplantation was preceded by HDT in nine patients and by a nonmyeloablative regimen in one patient. The RR was 80%, including seven major responses. With a median follow-up of 20.5 months, six patients are alive and free of progression at 3 to 76 months. Four patients died, all from toxicity, resulting in a TRM of 40%. HDT followed by autologous transplantation is feasible in WM, even in heavily pretreated patients, with some prolonged responses but a high relapse rate. Conversely, allogeneic transplantation is more toxic, but likely induces a graft-versus-WM effect and may, for some patients, result in long-term disease control.

Background. Primary bone lymphoma (PBL) is a rare entity that has only been reviewed in one prospective and small retrospective studies, from which it is difficult to establish treatment guidelines. We prospectively evaluated high-dose or conventional anthracycline-cyclophosphamide dose and radiotherapy for PBL.Patients and Methods. The GOELAMS prospective multicenter study (1986–1998) enrolled adults with localized high-grade PBL according to age and performance status (PS). Patients Results. Among the 26 patients included (VCAP: 19; VCEP-bleomycin: 7), 39% had poor PS ≥2. With a median follow-up of 8 years, overall survival, event-free survival, and relapse-free survival were 64%, 62%, and 65%, respectively, with no significant difference between treatment groups. Poor PS was significantly associated with shorter OS and EFS.Conclusions. Our results confirm the efficacy of our age-based therapeutic strategy. High-doses anthracycline-cyclophosphamide did not improve the outcome. VCEP-bleomycin is effective and well tolerated for old patients. The intensification must be considered for patients with PS ≥2, a poor prognostic factor.

Introduction Le lymphome malin non hodgkinien (LMNH) conjonctivo-orbitaire est une tumeur relativement rare de diagnostic difficile. L’attitude therapeutique face aux formes indolentes tend a se modifier, et le developpement de l’immunotherapie y contribue. Nous rapportons deux cas de patients traites par rituximab (anticorps anti-CD20). Observation Deux femmes, âgees respectivement de 50 ans et 59 ans, presenterent un LMNH indolent, isole et de bas grade de malignite : l’un, conjonctival de type MALT et l’autre, orbito-palpebral de type A selon la WFC, folliculaire a petites cellules. L’immunohistochimie montrait un marquage diffus pour l’anticorps anti-CD20. Les patientes recurent en 1 re intention une monotherapie de rituximab par voie intraveineuse, debutee en decembre 2000, a raison de 375 mg/m 2 , en 4 injections a 1 semaine d’intervalle. Aucun effet indesirable majeur ne fut constate. Les patientes sont en remission complete depuis la fin du traitement, soit un recul de 4 ans. Elles sont toujours sous suivi hematologique et ophtalmologique regulier. Conclusion Le rituximab (Mabthera ® ) offre une alternative non negligeable dans le traitement des LMNH orbitaires indolents. Son efficacite est surtout documentee dans les LMNH folliculaires ou chez les patients a rechutes. Celles-ci seraient un peu plus frequentes dans le type MALT. Par ailleurs, ce traitement presente une faible toxicite hematologique et est generalement bien tolere.

Rhodotorula species are commensal yeasts of variable pathogenicity. The authors report the case histories of two patients presenting with febrile neutropenia. The first was a 3-year-old girl who had been treated with combination chemotherapy for a tumour of the posterior fossa. The second was a 46-year-old man who had received chemotherapy for lymphoplasmocytic lymphoma, followed by consolidation treatment with autologous bone marrow transplantation. Investigation revealed infection caused by Rhodotorula. The outcome was favourable after removal of the catheter in both patients. Rhodotorula species have been isolated during a variety of infectious complications. Almost all published cases of fungaemia concern patients with central venous catheters that have been in place over long periods, who have also been treated with broad spectrum antibiotics. Neoplasia represents the most frequent underlying disease. The pathogenicity of Rhodotorula species appears to be moderate in most cases; fungal therapy or the removal of infected catheters is generally effective. Nevertheless, Rhodotorula has been reported to provoke fatal endocarditis or meningitis and can probably cause septic shock.

We studied two cases of chronic myelogenous leukemia (CML) with unusual variant Philadelphia (Ph) translocation (22;22)(q11;q13). Southerr, blot analysis showed a chromosomal break in the BCR gene within the 5.8-kilobase (kb) breakpoint cluster region (bcr), between bcr exons 2 and 3 and between bcr exons 3 and 4, respectively. Chimeric bcr-abl mRNA was detected using polymerase chain reaction (PCR) which amplified, according to the respective bcr breakpoints, bcr exon 2- abl exon II and bcr exon 3- abl exon II junction products. These results further support the involvement, even when not cytogenetically detectable, of the 9q34 chromosomal region in all variant Ph translocations and that BCR-ABL gene fusion products are causally involved in the development of Ph positive CML.

From January 1979 to December 1987, 35 cases of primary central nervous system lymphoma (CNS-L) were treated. We recently reviewed these cases focusing on treatment results, treatment modalities, and radiotherapy (RT) or chemotherapy-radiotherapy (CT-RT). Variables such as age, risk factors, presenting symptoms, and histologic condition (all were high-grade or intermediate-grade non-Hodgkin's lymphomas [NHL]) and radiologic data were similar to those of series reported previously. The median survival time was 36 months (+/- 0.2 months) and the disease-free survival (DFS) time was 16 months (+/- 0.12 months). Twelve of 32 patients evaluable for treatment results experienced a recurrence (all but one occurred in the CNS). The DFS rate was 70% for the CT-RT group and 50% for the RT group (median follow-up time, 24 months). Therapeutic results in CNS-L are discussed with special emphasis on a putative role of CT in the management of this rare type of tumor.

To report on an uncommon association of agranulocytosis in primary Sjögren's syndrome (SS).The clinical, haematological, and immunological features of seven patients with primary SS associated with a chronic (6 months) agranulocytosis, and the outcome of the patients, were analysed.Patients were white women with an unexplained agranulocytosis. They all had non-erosive arthritis and three had a thrombocytopenia or Evan's syndrome. In three patients, transient or durable expansion of T lymphocytes was present in the peripheral blood or in the bone marrow, but evolved independently from neutrophil counts. There was no paroxysmal nocturnal haemoglobinuria clone or antibodies to neutrophil surface antigens. In vitro bone marrow culture was normal (four patients) or showed a decrease in colony forming unit-granulocyte monocyte (CFU-GM) and colony forming unit-erythroblast (CFU-E) (one patient). Serum levels of soluble Fas ligand (sFasL) were normal, and granulocyte-colony stimulating factor (G-CSF) concentrations were either normal or raised. One patient was treated with steroids associated with intravenous immunoglobulins and achieved a lasting response. Two other patients were treated with steroids and methotrexate, with poor efficacy. Short courses of subcutaneous G-CSF produced a transient and mild response in all three patients. Complete recovery of the neutrophils occurred temporarily during pregnancy in two patients. After a mean follow up of 34.8 months (range 6-139) all patients were alive and none developed serious infections.A subset of patients with primary SS and non-destructive arthritis may develop a chronic but well tolerated agranulocytosis that is usually poorly responsive to steroids and oral methotrexate.

We designed a randomized trial of IC with or without quinine, an agent capable of reverting the multidrug resistance (mdr) phenotype, in patients aged < 65 years with high risk MDS. Patients were randomized to receive Mitoxantrone 12mg/m2/d d2–5 + AraC 1g/m2/12h d1–5, with (Q+) or without (Q) quinine (30mg/kg/day). 131 patients were included. PGP expression analysis was successfully made in 91 patients and 42 patients (46%) had positive PGP expression. In PGP positive cases, 13 of the 25 (52%) patients who received quinine achieved CR, as compared to 3 of the 17 (18%) patients treated with chemotherapy alone (p=0.02). In PGP negative cases, the CR rate was 35% and 49%, respectively in patients who received quinine or chemotherapy alone (difference not significant). In the 42 PGP positive patients, median Kaplan-Meier (KM) survival was 13 months in patients allocated to the quinine group, and 8 months in patients treated with chemotherapy alone (p=0.01). In PGP negative patients, median KM survival was 14 months in patients allocated to the quinine group, and 14 months in patients treated with chemotherapy alone. Side effects of quinine mainly included vertigo and tinnitus that generally disappeared with dose reduction. Mucositis was significantly more frequently observed in the quinine group. No life threatening cardiac toxicity was observed. In conclusion, results of this randomized study show that quinine increases the CR rate and survival in PGP positive MDS cases treated with IC. The fact that quinine had no effect on the response rate and survival of PGP negative MDS suggests a specific effect on PGP mediated drug resistance rather than, for instance, a simple effect on the metabolism of Mitoxantrone and/or AraC.

From May 1992 to November 1994, 240 patients with de novo acute myeloid leukemia (AML) and aged 55–75 years (median 68 years) were enrolled in a multicenter, double-blind randomized study comparing placebo and granulocyte-macrophage colony-stimulating factor (GM-CSF) (Escherichia coli derived) (5/µg/kg per day 6-h infusion) during induction chemotherapy (idarubicin 8 mg/m2 per day i.v. on days 1–5 plus cytarabine, ara-C, 100 mg/m2 per day continuous infusion on days 1–7) and until recovery of a neutrophil count > 0.5 x 109/1. Post-remission therapy was identical in the two arms. The two groups were comparable regarding the following parameters: age, sex, performance status, fever, organomegaly, initial blood cell counts, French-American-British (FAB) classification, presence of myelodysplastic features, and incidence of karyotypic abnormalities. Of the 209 patients currently evaluable, 130 (62%) achieved complete remission (CR) with no significant difference between patients aged 55–64 (57/90 = 63%) and patients aged 65 to 75 years (73/119 = 61%). There were 17 (8%) early deaths, 18 (9%) deaths in aplasia, and 45 (21%) failures. No significant difference was observed between the GM-CSF group (n = 103) and the placebo group (n = 106) regarding the CR rate (63% vs. 61%), the incidence of early deaths (10% vs. 6%), of deaths in aplasia (8% vs. 9%), and of failures (19% vs. 24%). The duration of neutropenia was shorter in the GM-CSF group (median 22 days vs. 26 days, p = 0.002). However, the incidence of febrile episodes and of bacteriemias and the duration of hospitalization was similar in the two groups. With a median follow up of 19 months, the overall survival of all eligible patients was similar in both groups (39% actuarial survival at 2 years in the GM-CSF group vs. 33% in the placebo group, p = 0.45 log-rank test). Nonetheless, the disease-free survival was longer for patients who achieved CR in the GM-CSF group (44% continuous CR rate at 2 years vs. 19%, p = 0.024). The study medication was prematurely stopped due to toxicity in 17 patients (14 GM-CSF, three placebo; p = 0.003). We conclude that (a) the induction treatment with idarubicin and conventional doses of ara-C obtains a high CR rate in elderly patients (b) the administration of GM-CSF during and after induction chemotherapy results in a faster recovery of neutrophils but does not reduce infectious toxicity and increases neither the efficacy of this chemotherapy nor the overall survival. However, the disease-free survival was significantly longer for patients who received GM-CSF before achieving CR.

Low-dose chemotherapy in myelodysplastic syndromes (MDS) gives overall limited results in high-risk MDS [1–3]. Intensive chemotherapy in MDS and secondary leukemias (i.e., acute myeloid leukemia, AML, following de novo or therapy-related MDS) gives lower complete remission (CR) rates and shorter CR duration than in de novo AML [4–11]. This may be due in part to a higher incidence of mdr gene expression in MDS than in de novo AML as mdr gene expression is associated in our experience, with very low CR rates with intensive chemotherapy [12]. These recent results suggested that the use of agents capable of reverting mdr gene expression could improve the CR rate in MDS and secondary leukemias.

The frequency of chronic lymphocytic leukemia (CLL) varies a great deal from one population to another. We have undertaken to compare the aspects of CLL in Maghreb and Europe through two series of com

Le diagnostic de granulomatose de Wegener est assez facilement evoque dans une forme complete associant des manisfestations pulmonaires, renales et des voies aeriennes superieures. L'association a une autre pathologie est peu frequente. Nous rapportons un cas de maladie de Wegener de forme incomplete associee a une leucemie myelo-monocytaire

Resume Les auteurs rapportent un cas de bacteriemie a Branhamella catarrhalis productrice de penicillinase et Staphylococcus aureus chez une femme de 48 ans porteuse d'un lymphome malin non Hodgkinien en aplasie post-chimiotherapie. Aucune porte d'entree n'a ete mise en evidence. L'evolution a ete favorable a court terme. Branhamella catarrhalis est un commensal du rhinopharynx qui agit en opportuniste. L'incidence croissante de souches productrices de β lactamase depuis 1977 est a connaitre pour la therapeutique.

The coexistence of autoimmune haemolytic anaemia (direct IgG antiglobulin test) and pure bone marrow sarcoidosis is described in a 69-year-old woman. This case report seems to be the 5th in the available literature.

In 1981 the unexpected appeareance of opportunistic infections and Kaposi’s sarcoma in homosexual men in the United States marked the beginning of the AIDS epidemic [8]. Shortly thereafter the observation of an increasing number of male homosexual subjects suffering from high-grade non-Hodgkin’s lymphomas [6, 15, 16] resulted in the inclusion of this hematological malignancy in the Centers for Disease Control (CDC) definition of AIDS [4], As early as 1983 the retrovirus responsible for this major immunodeficiency state, now called human immunodeficiency virus (HIV), was discovered [3, 11]. Since then the HIV infection epidemic has been spreading rapidly by means of sexual and intravenous transmission routes in America as well as in Africa and Europe. The discovery of different types of hematological and non hematological malignancies in AIDS patients and HIV-positive subjects led us to create a French register of HIV-related neoplasia. This paper reports the first 68 cases of hematological malignancies identified in this register between 1983 and June 1987.