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Lymphomatoid papulosis (LyP) is considered as an indolent CD30+ lymphoproliferative cutaneous disorder. 1,2 Nevertheless, 10%–20% up to 50% of patients with LyP have coexistent lymphoma. 1, 3–7 The most frequently associated malignancies are mycosis fungoides, primary cutaneous anaplastic large cell lymphoma, or Hodgkin's disease. There are only seven reported cases of an association between LyP and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). 5, 7-9 Herein, we report and analyze three new cases. This article is protected by copyright. All rights reserved.

Resume L’objectif principal de cette etude est d’evaluer la pertinence de la reglementation recommandant le recours a la transfusion autologue programmee avant un don de moelle osseuse. Les donnees anonymisees des 50 donneurs ayant participe a un programme de transfusion autologue programmee de 2010 a 2012 ont ete analysees retrospectivement. Dans le groupe 1 des 25 patients transfuses avec les produits autologues par rapport au groupe 2 non transfuses, le volume median de moelle osseuse preleve (respectivement 17,7 vs 13,3 mL/k) et la proportion de femmes (respectivement 60 % vs 16 %) ont ete plus importants, mais la perte totale mediane d’hemoglobine moins importante (respectivement −2,9 vs −3,5 g/dL). Par contre, aucune difference entre les deux groupes n’a ete retrouvee pour les taux d’hemoglobine medians apres le don a j+1 (10,9 dans le groupe 1 vs 11,15 g/dL dans le groupe 2). Six evenements indesirables ont ete declares dans chaque groupe. Ils sont survenus chez des donneurs ayant un poids median plus faible (58 vs 75 k) et un volume median de moelle osseuse preleve plus important (20,1 vs 14,3 mL/k). Le don de moelle osseuse s’accompagne d’une perte d’hemoglobine non negligeable, mais non suffisante pour atteindre des taux en postoperatoire necessitant une transfusion sanguine dans cette population en bonne sante. La survenue d’evenements indesirables est frequente (25 % des dons), necessitant une surveillance attentive apres le don. La transfusion autologue programmee ne presente pas un rapport benefice–risque suffisant pour justifier sa prescription systematique au cours d’un don de moelle osseuse.

We have developed a real-time quantitative polymerase chain reaction (PCR) assay using TaqMan technology (Applied Biosystems, Foster City, CA) for monitoring donor cell engraftment in allogenic hematopoietic stem cell transplant recipients. For this purpose, we selected 19 specific sequence polymorphisms belonging to 11 human biallelic loci located on 9 different chromosomes. Using a set of specially designed primers and fluorogenic probes, we evaluated the 19 markers' informativity on a panel of 126 DNA samples from 63 recipient/donor pairs. In more than 90% of these pairs, discrimination between recipient and donor genetic profile was possible. By using serial dilutions of mixed DNAs, we evaluated the linearity and sensitivity of the method. A linear correlation with rhigher than 0.98 and a sensitivity of 0.1% proved reproducible. Fluorescent-based PCR of short tandem repeats (STR-PCR) and real-time PCR chimerism assay were compared with a panel of artificial cell mixtures. The main advantage of the real-time PCR method over STR-PCR chimerism assays is the absence of PCR competition and plateau biases, and results evidenced greater sensitivity and linearity with the real-time PCR method. Furthermore, different samples can be tested in the same PCR run with a final result in fewer than 48 hours. Finally, we prospectively analyzed patients who received allografts and present 4 different clinical situations that illustrate the informativity level of our method. In conclusion, this new assay provides an accurate quantitative assessment of mixed chimerism that can be useful in guiding early implementation of additional treatments in hematopoietic stem cell transplantation.

Resume Introduction Chez les patients atteints d'hemopathies, l'infection a virus respiratoire syncytial est favorisee par la neutropenie et l'immunodepression humorale et cellulaire et elle est responsable d'infections respiratoires severes. Exegese Quatre patients atteints d'hemopathies malignes ont developpe une infection severe a virus respiratoire syncytial. Trois d'entre eux avaient ete traites par autogreffe de cellules souches (AGCS) 2 a 7 ans auparavant. L'evolution a ete favorable chez trois patients. Un patient est decede d'insuffisance respiratoire aigue. Conclusion En periode hivernale, la survenue d'une infection respiratoire severe doit faire rechercher le VRS chez ces patients. Le diagnostic est rapide par immunofluorescence sur le liquide de lavage bronchoalveolaire. Le traitement par aerosols de ribavirine est controverse et doit etre reserve aux patients qui ont un facteur de pronostic defavorable (chimiotherapie intensive, AGCS, pneumonie bilaterale hypoxemiante).

Relapsed or very aggressive high-grade NHL and refractory low-grade NHL have a poor clinical outcome. Autologous BMT may be used but is of limited efficacy in these cases. Allogeneic BMT offers the advantage of tumour-free bone marrow and a possible GVL effect. Between 1987 and 1996, 13 patients (median age 31 years) suffering from lymphoid malignancies underwent allo-BMT. Four patients had low-grade NHL, three intermediate-grade and six high-grade NHL. Three patients were grafted with evolutive disease, four were in partial remission after several courses of chemotherapy, two were in CR2 and four were in CR1 after initial therapy. The mean number of prior treatments was 2.7 (1-6). Median time from diagnosis to BMT was 25 months (4-90). The conditioning regimen consisted of cyclophosphamide (120 mg/kg/day for all, plus VP16 in one case) and total body irradiation. Five out of the seven patients who were not in CR at the time of transplantation entered CR after BMT. Eight patients developed acute GVHD grade > or = II and four had chronic GVHD. Nine patients are alive, eight in CR with a median follow-up of 49.8 months post BMT (2-125). Overall survival is 67.3% and the median time for EFS is 102 months. Two patients with low-grade NHL relapsed 61 and 102 months post BMT and were treated with DLI. One patient with a stage IV SLL had a partial remission and one with multiple cutaneous localisation of FL entered CR after grade IV acute GVHD. Allo-BMT is a highly effective treatment for advanced poor prognosis lymphoid malignancies with acceptable toxicity. Moreover, DLI can be effective in relapsing patients.

Invasive pulmonary aspergillosis (IPA) is an increasing cause of morbidity and mortality in patients with hematologic malignancies. A major program of construction work close to our unit prompted us to evaluate the efficacy of itraconazole prophylaxis in preventing IPA in these patients. During September 1994 to December 1995, 77 patients undergoing 96 neutropenic episodes (mean duration, 19.3 days +/- 9.1) received itraconazole as antifungal prophylaxis. All patients were treated in laminar air flow rooms. Itraconazole was administered at a loading dose of 600mg/d, (day 1 to day 3) and 400mg/d on the following days, in 87 instances. In the remaining episodes, the daily dose was 200 or 400mg. Oral doses were adjusted to reach a plasma itraconazole level (PIL) above 1000ng/l. In cases of inadequate PIL or poor oral intake, IV AmphoB was started at a 20 mg daily dose. Five cases of IPA (proven n = 2, probable n = 3) were observed. This represents an incidence of 5.2% of the total number of episodes. One out of 67 (2%) treatment episodes with adequate PIL were associated with IPA as compared to 4 of 29 (14%) episodes with inadequate PIL, (p0.02). AmphoB was added in 28 cases because of low PIL (n = 25), and/or antibiotic-resistant fever persistent pulmonary infiltrate (n = 8). These results need to be interpreted with caution, because of the absence of randomization or a control group. The efficacy of Itraconazole in neutropenic patients with high risk IPA has to be confirmed on larger and prospective studies.

Resume Dans les anemies hemolytiques auto-immunes, la presence d'anticorps sur la membrane erythrocytaire conduit a une hemolyse selon un processus immunologique, mais ne modifie-t-elle pas les proprietes rheologiques des hematies, participant ainsi a l'hemolyse par un processus mecanique? Le but de ce travail a ete d'etudier les proprietes rheologiques d'hematies sensibilisees par des anticorps de type IgG. L'etude a porte sur 20 patients porteurs d'anemie avec test de Coombs direct positif, dont 12 presentent une hemolyse franche, 10 echantillons sanguins sensibilises in vitro, et 20 sujets temoins. Les parametres hemorheologiques suivants ont ete mesures: filtration erythrocytaire, viscosites sanguine et plasmatique, dosage de l'ATP et du 2,3 DPG, morphologie des hematies en microscopie electronique a balayage (MEB). Les resultats montrent une augmentation des index de rigidite erythrocytaire (p

Four cases of chronic agranulocytosis in patients with systemic diseases different from Felty's syndrome are reported. Two patients had primary Sjogren's syndrome (confirmed in one, suspected in the other), one had systemic lupus erythematosus and the last patient had an unclassifiable connective tissue disease. Only one severe infection was recorded. The pathophysiological mechanisms involved are discussed. There is a strong suspicion that the cytopenia is of immune origin. Corticosteroid therapy was effective in all four patients. One patient who could not tolerate corticosteroids was successfully treated with cyclosporin A.

To evaluate treatment by thalidomide and identify predictive factors of survival, event free survival and response among patients with advanced multiple myeloma treated with thalidomide as single agent therapy.Patients with advanced multiple myeloma (n=83) were treated with an oral dose of thalidomide (median 400 mg/day). At start of treatment, all patients had active disease and 58 (69%) had received at least one autologous transplantation.With a median follow-up of 338 days (range, 247-629 days), 52 patients are alive, whereas 31 died between 8 and 150 days after the first administration of thalidomide. The response to thalidomide was considered as major in 11 patients (13%), partial in 29 patients (35%) and minor in 15 patients (18%), giving a total response rate of 66% (54 out of 83 patients). Thirteen patients had stable disease and 15 patients progressed. In multivariable analysis, age greater than 60 years, short interval between diagnosis and onset of thalidomide, requirement for red blood cell transfusion, IgA isotype, platelets' count80 x 10(9)/l and serum albumin level30 g/l at the start of thalidomide were associated with poor outcome. These three last factors produced a simplified prognostic model for patients with advanced myeloma and treated with thalidomide. Thus, among the 38 patients without any of these unfavorable risk features, one-year overall survival and event free survival were 87% and 78%. By contrast, the 43 patients with at least one unfavorable feature had one-year overall survival and event free survival of 40% and 32%, respectively (Log-Rank, P=0.0002 for both). Patients who receivedor =34.4 g of thalidomide in the first 90 days of treatment had a better outcome than those who received34.4 g. However, the mean received daily dose of thalidomide in the first 90 days has not been found to influence survival, event free survival or response. Short-term side effects of thalidomide were generally moderate.Thalidomide is an effective treatment for patients with advanced myeloma, in particular, who have no poor-risk features. The poor results achieved by the other patients emphasize the need for prospective protocols using thalidomide in combination, especially with dexamethasone. In addition, further studies are needed to determine the optimal thalidomide dose and duration.

We have tested the efficiency of GM-CSF to mobilize peripheral blood progenitor cells (PBPC) and evaluated the hematological reconstitution after GM-CSF primed-PBPC infusion following myeloablative therapy. Twenty three patients suffering from hematological malignancies were included in this study. Starting 24 hours after completion of a standard dose chemotherapy including vindesine, cyclophosphamide, adriblastine, prednisone, (VCAP), 5 micrograms/kg sub-cutaneous daily dose GM-CSF was given for a median time of 14 days followed by three consecutives cycles of leukapheresis. Fifteen of these 23 patients underwent GM-CSF primed-PBPC autotransplantation following high dosed intensification regimen. PBPC collection and hematopoietic recovery were compared with a 15 patients control group who did not receive GM-CSF. No marrow or growth factors were administered after PBPC reinfusion in the two groups. VCAP/GM-CSF mobilization induced significantly higher yields of CFU-GM (3.8 fold) than did VCAP mobilization alone, 19 x 10(4)/kg (2-73) vs 5 x 10(4)/kg (2-27), (p0.005). The median number of days to achieve 1.10(9)/l neutrophils, platelet count20.10(9)/l and50.10(9)/l was significantly lower in the GM-CSF group than in the control group, respectively 13 vs 19 days (p = 0.04), 15.5 vs 27 days (p0.02), 19 vs 51 days (p0.01). When compared with the control group, transfusion requirements and median of hospital stay were both significantly decreased for the patients receiving GM-CSF primed-PBPC. Our study confirms that infusion of GM-CSF primed-PBPC as a sole source of hematopoietic support improves hematopoietic reconstitution following myeloablative therapy.

14 adult patients between 16 and 50 years old with small non-cleaved cell lymphoma (Burkitt's lymphoma) were prospectively treated from 1982 to 1990 with the LMB protocols of the Societe Francaise d'Oncologie Pediatrique (SFOP). No HIV-positive patients were included. All patients had extensive disease with bad prognosis factors, i.e. 10 patients had Murphy stage III and 4 had stage IV with bone marrow involvement. The LMB protocols were characterised by high-dose fractionated cyclophosphamide, high-dose methotrexate (HD-MTX), and cytosine arabinoside. No local or central nervous system irradiation was used. Treatment duration ranged from 5 (LMB 84) to 12 (LMB 81) months. There were no therapy-related deaths. All patients achieved complete remission (CR). 6 patients relapsed between 2 and 30 months following CR. 8 of the 14 patients (57%) are still alive and disease-free after treatment by LMB protocol alone. 2 patients were salvaged with bone marrow transplantation after relapse and a total of 10 out of 14 patients (71%) are disease-free at the time of this report. Our results showed the high curability of advanced Burkitt's lymphoma using a paediatric protocol, even in adult patients. The LMB protocol may be applied to adult patients but requires intensive care during the induction period.

In autoimmune haemolytic anaemia, the presence of antibodies on the erythrocyte membrane results in haemolysis through an immune process, but does it not alter the rheological properties of red blood cells (RBC), thus adding a mechanical factor to haemolysis? This study was designed to examine the rheological properties of erythrocytes sensitized with IgG-type antibodies. The study involved 20 patients with anaemia and positive direct antiglobulin test, including 12 with straightforward haemolysis, 10 samples sensitized in vitro, and 20 controls. The following haemorheological parameters were studied: erythrocyte filtration, blood and plasma viscosities, titration of adenosine triphosphate (ATP) and 2-3-DPG, erythrocyte morphology under scanning electron microscopy. The results showed increased erythrocyte rigidity (P less than 0.025) as well as higher blood viscosity compared to controls with similar haematocrit values, and unaltered ATP and 2-3-DPG (consistently with scanning electron microscope observations). These haemorheological disorders were more noticeable in patients with clear-cut haemolysis, and there was a correlation between the increase in erythrocyte rigidity indices and the haemolytic parameters, especially haptoglobin (P less than 0.001). The in vitro study confirmed the results obtained ex vivo. To conclude, the mechanical properties of antibody-coated erythrocytes are impaired, which may promote the immunological mechanism favouring haemolysis in the spleen.

One frequent complication of allogeneic bone marrow transplantation (BMT) is graft-versus-host-disease (GVHD). GVHD occurs in 25 % to 75 % of patients with HLA identical, MLC-unreactive donors and T lymphocytes in the marrow graft are likely to mediate this reaction.