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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 assessed the benefit of predeposite autologous blood donation (PAD) before bone marrow (BM) donation on transfusion requirements, haemoglobin concentrations (Hb) and the occurrence of adverse events (AE). We collected data retrospectively from 50 donors of BM with PAD from 2010 to 2014. An autologous transfusion (AT) was given to 50% of the donors (group 1). In the group 2, the products from PAD were not used. The total volume median of marrow harvested was 17.7 mL/k (range 12.3-21.4) in the group 1 and 13.3 mL/k (8.6-22.6) in the group 2. The female ratio was higher in the group 1 (60%) than in the group 2 (16%). Bone marrow harvest led to a decline in Hb (from PAD to first day after BM donation) by 2.9 g/dL (1.5-5.5) in the group 1 and by 3.5 g/dL (1.2-5) in the group 2. The post-harvest Hb (D+1) median was identical in the two groups: 10.9 g/dL (7.6-13.5) in the group 1 versus 11.5 g/dL (9.3-13.4) in the group 2. Six AE were reported in each group. In the group with AE, the median weight was lower: 58 k (50-71) versus 75 k (52-110); and the median total volume of marrow harvested was higher: 20.1 mL/k (9.9-21.4) versus 14.3 mL/k (8.6-22.6). All post-harvest Hb were ≥ 7.6g/dL. This study shows the high loss of Hb after BM donation but not enough to prove a blood transfusion in BM donors with median age of 36 years (16-62) and without comorbidity. The occurrence of AE (25% of BM donors) justifies a careful surveillance after the BM donation. The PAD should not be routinely offered to bone marrow donors.

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.

Objectif Evaluer la pertinence de la transfusion autologue programmee (TAP) avant un don de moelle osseuse (DMO). Methode Pour chaque DMO realise au CHU entre 2010 et 2012, ont ete recueillies : âge, poids, volume sanguin total, hemoglobinemie (Hb eng.dL-1) avant TAP, delai entre TAP et DMO, volume preleve en DMO, Hb avant, pendant et apres DMO, utilisation des PSL autologues (PSLA) et notion d’incidents. Les donneurs ont ete repartis en 3 groupes : groupe 1 = DMO + TAP avec transfusion des PSLA, groupe 2 = DMO + TAP sans transfusion des PSLA et groupe 3 = DMO sans TAP. Resultats 58 DMO ont ete analyses. L’âge moyen est de 38 ± 12 ans (hommes = 60 %) avec TAP pour 86 % des DMO (1 CGR + 1 PFC preleves). L’Hb moyenne post-DOM a j+1 = 11,19 ± 1,41 et la perte totale moyenne d’Hb au cours d’un DOM = −3,23 ± 0,97. L’Hb post-DMO est plus elevee dans le groupe 2 (11,34 ± 1,10 vs 10,72 ± 1,94 dans le groupe 3). La perte d’Hb est moins importante dans le groupe 1 (−2,90 ± 0,96 vs −3,51 ± 0,88 dans le groupe 2). 13 donneurs (6 dans chaque groupe 1 et 2) ont presente un incident, essentiellement apres le DMO : malaise vagal, asthenie, chutes et hospitalisation > 48 H (1 DOM du groupe 3). Aucun n’a recu de PSL homologues. Discussion L’arrete du 16/12/1998 preconise de proposer une TAP avant un DMO. L’Hb a j+1 est toujours > 7,60. Les incidents surviennent en post-DOM chez des donneurs qui ont, a ce moment, une Hb post-DOM = 9,99 ± 1,75 et la transfusion des PSLA (exclusivement pendant le DOM) ne permet pas de les eviter. Une population a risques peut etre definie : donneurs femmes, non apparentes, jeunes et de poids Conclusion Au total, le rapport benefices/risques de la TAP au cours d’un DMO est faible. Une revision du cadre reglementaire est necessaire. Par contre, la survenue frequente d’incidents doit inciter a realiser une surveillance attentive apres le DOM.

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.

Involvement of the gastrointestinal tract is frequently reported among the extranodal sites of non-Hodgkin's lymphoma, but primary lymphoma of the common bile duct is extremely rare. We report the case of a 29-year-old man who presented with obstructive jaundice, leading to the diagnosis of high-grade primary non Hodgkin's T-cell lymphoma, originating from the extrahepatic biliary tract, and confirmed by endosonography and magnetic resonance cholangiography. This patient was treated by sequential chemotherapy without resection and remained in complete remission after one year.

In hematologic malignancies, respiratory syncytial viral infections can be explained by neutropenia, and cellular and humoral immunodepression, and may cause severe respiratory infections.Four patients with hematologic malignancies developed a severe respiratory syncytial virus infection. Three of them had previously received autologous bone marrow transplantation (ABMT). Progress was favorable for three patients. One patient died of acute respiratory failure.When such patients present with respiratory symptoms, especially during the winter months, they should be screened for RSV. Bronchoalveolar lavage allowed quick and accurate diagnosis by immunofluorescence. Treatment with nebulized ribavirin is controversial. Its use may be interesting in patients with high-risk factors (intensive chemotherapy, ABMT, diffuse pneumonia with hypoxemia).

Major histocompatibility complex class I molecule expression is reduced in some malignant tumours permitting escape from immune surveillance and is therefore associated with a poor prognosis. Seven cases of non-Hodgkin lymphomas out of 300 cases of malignant lymphoproliferative disorders totally lacked expression of class I molecules as determined by flow cytometry. Clinical data confirmed a particular aggressiveness of these cases with frequent extra-nodal involvement, a poor international prognostic index, a histological high grade and a poor outcome leading to early death in five of the seven cases. A previous diagnosis of follicular lymphoma characterized by bcl-2 rearrangements was made in four of these cases. HLA-G (class Ib gene), which is reported to bind killer inhibitory receptors on NK cells, was absent from the cell surface. However, it was detected in three out of four cases at the mRNA level with transcripts encoding soluble forms. Additional analysis revealed other abnormalities: class II was negative in four out of the seven NHL cases and decreased expression of beta2 microglobulin was observed in all cases. Peptide transporter proteins (TAP1) were detected in various degrees by immunocytochemistry. These observations showed that total lack of class I or class II molecules is a rare event in NHL and is associated with a poor prognosis. This could support a role for specific autologous T cells in immune surveillance.

Allogeneic BMT for patients with acute myeloid leukemia (AML) is presently a reference therapy. The indications for this therapy mainly rely upon prognostic factors, and their importance is constantly reassessed. To examine the impact of time from diagnosis to transplant on survival and leukemia-free survival (LFS), we analyzed 109 patients from the database of the SFGM comprising patients who had all received an HLA-identical allogeneic BMT for a diagnosis of AML in first complete remission (CR1) between January 1987 and December 1992. All patients were conditioned with cyclophosphamide (CY) and total body irradiation (TBI) (CYTBI), and methotrexate (MTX) + cyclosporin A (CsA) were used as graft-versus-host disease (GVHD) prophylaxis. Patient characteristics were: age = 33 +/- 9, M/F = 64/45, white blood cell count (WBC) at diagnosis = 27 +/- 42 x 10(9)/l, FAB distribution: M1 and M2 = 55; M3 = 15, M4 and M5 = 33, M0, M6 and M7 = 6. Karyotyping was carried out for 64 patients: 32 had a normal karyotype, 16 had good prognosis abnormalities (t(8;21), t(15;17), inv 16) and 16 patients had other abnormalities. Eleven patients needed two courses of induction to achieve CR. Time between diagnosis and BMT was 120 (64-287) days. Forty-nine patients developed gradeor = 2 acute GVHD (actuarial probability = 46%). With a median follow-up of 50 months (27-100), the 5-year probabilities for transplant-related mortality (TRM), relapse, overall survival and LFS are respectively 25%, 26%, 59% and 55%. A multivariate analysis showed that survival is adversely influenced by three independent factors: time to transplant (120 days vsor = 120 days), acute GVHD (grade 2-4 vs grade 0-1) and age (33 vsor = 33). LFS is only influenced by the first two of these factors. The favorable impact of a shorter time from diagnosis to transplant should lead to performing the transplant as early as possible. Practically speaking, this means that when such therapy is chosen for a patient with CR1 AML, the search for an allogeneic donor should begin immediately and transplant be performed as soon as possible.

We prospectively analyzed MDR functional activity by the Rh123 efflux assay in 84 de novo acute leukemias. Thirty of the 60 AML cases (50%) showed a positive dye efflux (in more than 10% of blast cells). In 19 cases, the dye efflux was superior to 30%. Twenty-four of the 30 efflux positive cases were CD34+ and could be studied in double staining. The mean percentage of effluxing CD34+ blast cells was 54%. There was a high correlation between CD34 expression and MDR activity (P10(-4)), MDR activity and PgP expression (P10(-6)). All the efflux negative samples were PgP negative. Nine efflux positive cases were PgP negative. Five of the 24 ALL were efflux positive. MDR activity did not correlate with FAB subtype (with the exception of AML3: 1/6 was efflux positive), age, white blood cell count or LDH level. Forty-seven AML patients were treated with conventional chemotherapy including cytarabine and an anthracycline. Thirty-one (66%) entered complete remission (CR). CR rate was statistically lower for efflux positive as compared to efflux negative patients, 46 vs 87% (P = 0.003), for PgP+ as compared to PgP- patients, 40 vs 78% (P = 0.01), for CD34+ as compared to CD34- patients, 45 vs 84% (P = 0.005). There was no correlation between P110 expression (32 AML cases studied) and FAB subtype, MDR status and clinical outcome. Two years survival was 20% for efflux positive patients as compared to 54% for efflux negative patients (P0.07), 15% for PgP+ vs 54% for PgP- patients (P0.04). The finding of efflux+/PgP- cases suggests the existence of other membrane efflux pumps. Rh123 efflux assay is straightforward in routine and could be included in MDR screening because of its potential interest in clinical outcome in AML.

Unrelated donor searches for 100 Caucasian patients were referred to France Greffe de Moëlle Registry (FGM) from September 1987 (24,600 donors) to December 1993 (71,500 donors, 61% DR typed). After DR typing of HLA-A,B matched donors, unsuccessful searches were extended to other European Registries for 36 patients. Twenty two patients had a donor (FGM: 19, other Registries: 3) selected on: (1) HLA-A,B and DRB,DQB1 split identity; and (2) unidirectional relative response5% in MLR performed twice. Estimated probability of finding a compatible donor at 9 months in FGM was 12% (s.e. +/- 4%) and 25% at 2 years (s.e. +/- 6%). This probability was stringently dependent on a phenoidentity to one very common HLA-A,B,DR or B,DR haplotype (25% at 9 months when present, representing 19 of 19 patients with a compatible donor). Without this phenoidentity, the probability was zero per cent (P = 0.0001) in FGM searches and4% (n = 1) in extended searches. The MLR test was shown to be insensitive for screening for DPB1 mismatches. Clinical status influenced the probability of finding a compatible donor at one year ranging from 9% +/- 9% for ALL to 23% +/- 8% for CML (NS). Disregarding DPB1 mismatches is the most efficient way of increasing search efficiency.

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.

We investigated the prognosis value of CD34 and P-170 expression in blast cells of adult patients affected by de novo acute myeloid leukemia (AML). CD34 antigen was analyzed by indirect immunofluorescence (IFI) and alkaline phosphatase-labeled streptavidin biotine (AP-LSAB) in 62 patients (median age: 51 years). P-170 expression was determined by AP-LSAB in 51 cases using JSB1 and C219 monoclonal antibodies. All patients were treated with conventional chemotherapy induction regimen. Follow-up was from 6 to 79 months. Complete remission (CR) rate was not statistically different between CD34+ and CD34- patients (67 vs. 84%, p = 0.2). The duration of CR and survival were not influenced by CD34 expression. Karyotype abnormalities were more frequent among MDR+ patients (65 vs. 21%, p0.01). CR rate was statistically lower in MDR+ patients as compared to MDR- patients (63 vs. 96%, p = 0.01). Median disease-free survival (DFS) was shorter for MDR+ patients but the difference was not significant (5 vs. 10 months, p = 0.09). Patients who were positive for both parameters CD34 and P-170, had a poor prognosis with a 50 vs. 100% CR rate for CD34/P-170 negative patients, (p = 0.002), a lower median DFS (3 vs. 12 months, p = 0.01) and overall survival (OS) (3 vs. 14.5 months, p = 0.01). Results of cytogenetic analysis did not influence CR rate but the relapse rate was higher, although not significant, for the patients with unfavorable karyotype (63 vs. 33%). The seven CD34+/MDR+ patients with poor prognosis karyotype had a statistically lower CR rate, median DFS and OS than the 7 CD34-/MDR- patients with normal or favorable karyotype (CR: 29% vs. 100%, p = 0.02), (DFS: 3 vs.12 months, p = 0.01), (OS: 4 vs.12 months, p = 0.02). Our data indicate that P-170 but not CD34 expression is predictive for a lower CR rate. The identification of a bad prognosis subgroup of CD34+/MDR+ AML patients (and especially those with poor prognosis karyotype) has to be confirmed on larger series using uniform methodology.

We present a retrospective analysis of 111In-platelet sequestration studies in 111 patients with the clinical diagnosis of idiopathic thrombocytopenic purpura (ITP). Fifty-one of these patients underwent splenectomy, independent of the results of the 111In-platelet studies to determine if these isotopic results could accurately predict a beneficial response to splenectomy. Between January 1984 and June 1990, 111 patients who presented with ITP were subjected to a study of autologous 111In-labeled platelets through autotransfusion. The platelet sequestration site was splenic (81%), mixed (12%), or hepatic (7%). Fifty-one patients with persistent drug-resistant thrombocytopenia underwent splenectomy regardless of the isotopic results: 33 patients beyond 6 mo after diagnosis and 18 with high hemorrhagic risks before this delay. The follow-up median duration was 2.9 yr. Thirty-three of the 38 patients with splenic sequestration showed a normalized platelet count, as opposed to 2 of the 13 with mixed or hepatic sequestration (p0.001). In addition, platelet survival extended beyond 8 days in six patients, with no apparent sequestration site. The platelet isotopic study performed with this technique appears to be indicated in ITP: it guides clinicians in their decision to perform splenectomy and relates to a more central mechanism certain thrombocytopenias that are inappropriately categorized as ITP.

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.

A 26-year-old woman delivered a normal child 5 years after bone marrow transplantation for severe aplastic anemia. The conditioning regimen comprised high dose cyclophosphamide and thoraco-abdominal irradiation (6 Gy). This and two previous cases demonstrate that normal pregnancy can follow total body or thoracoabdominal irradiation.

Differences in erythrocyte isoenzyme patterns may be used as biologic markers to monitor allogenic bone marrow transplants. In 19 patients, these markers proved useful for confirming engraftment, although they failed to predict any of the recurrences observed.

Four cases of chronic agranulocytosis in patients with systemic diseases different from Felty's syndrome are reported. Two patients had primary Sjögren'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.

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.