Automatic depletion with Spectra Optia allows a safe 16% reduction of red blood cell pack consumption in exchanged sickle cell anemia patients.

BACKGROUND: Chronic red blood cell exchanges (RBCXs) are frequently used to prevent complications in patients with sickle cell anemia, but the scarcity of matched red blood cell packs (RBCPs) is a serious concern. The main goal of this study was to compare the number of RBCPs used during RBCXs between the Spectra Optia (SO) device (with the automatic depletion step) and the former Cobe Spectra (CSP) device. STUDY DESIGN AND METHODS: The performances and safety of 300 SO sessions using the automatic depletion step (SO/DE) in 50 patients with sickle cell anemia under a chronic transfusion program over a 1-year period were prospectively analyzed. The numbers of RBCPs saved using this protocol compared to the SO device without depletion and to the CSP device were determined. RESULTS: The SO/DE protocol appeared to be safe, as only 5% and 17% of the sessions were characterized by a significant decrease in blood pressure and increase in heart rate (grade 2 adverse events), respectively. Postapheresis hematocrit and fraction of cells remaining reached expected values. The SO/DE protocol required 16% fewer RBCPs compared to SO without depletion, allowing a mean saving of 12 RBCPs per patient and per year and 13% fewer compared to CSP device. Interestingly, the saving was more important for patients with high total blood volume and/or high preapheresis hematocrit. CONCLUSION: The SO/DE protocol is an efficient, safe and cost-effective procedure for patients with sickle cell anemia under a chronic transfusion program.

[A positioning pillow to improve lumbar puncture in paediatric haematology-oncology patients: a randomized controlled trial.]. / Coussin de positionnement pour ponctions lombaires en oncohématologie pédiatrique : résultats d'un essai randomisé

ObjectivesLumbar punctures (LPs) are common in children with cancer. Although pain management during the LP has been well standardised, dealing with stress and anxiety is not well addressed yet. Our objective was to evaluate the potential improvement of the LPs success rate using a positioning pillow, to ensure maximum lumbar flexion, and allow paravertebral muscles to relax, in children who are awake, with either conscious sedation or no sedation.Patients and methodsChildren aged 2-18 years undergoing LPs were randomly assigned to a positioning pillow or no intervention. The primary outcome was the rate of success, i.e. achieving the LPs (sampling or injection) at the first attempt, without bleeding (RBC < 50/mm3). The secondary outcomes included: the child's pain, assessed by a self-administered visual analogical scales (VAS) for children over 6 years of age; the parents' and caregivers' perception of the child's pain; the satisfaction of the children, the parents, the caregivers and the physician. The child's cooperation and the occurrence of post-LP syndrome were also evaluated.ResultsOne hundred twenty-four children (62 in each group) were included. The LP pillow tended to increase the success rate of LPs (67 vs 57%; P = 0.23), and decreased the post-LP syndromes (15 vs 24%; P = 0.17) but the differences were not statistically significant. In children over 6-year of age (N = 72), the rate of success was significantly higher in the pillow group (58.5 vs 41.5%; P = 0.031), with a tendency to feel less pain (for less pain and better satisfaction) [median VAS: 25 vs 15 mm; P = 0.39] and being more satisfied (84.4 vs 75.0%; P = 0.34).ConclusionEven if the results presented here are not as optimistic as we might have wished, we can confirm that there is a benefit in using this pillow for LPs. It is especially promising in children over 6-year of age.

[Thrombocytosis and essential thrombocythemia in childhood]. / Thrombocytoses et thrombocytémies essentielles de l'enfant.

Thrombocytosis is frequently observed in pediatric patients. Among them the secondary thrombocytosis are the most frequent and result from several causes. The rarely primary thrombocytosis can be either constitutive (and often familial) or acquired (essential thrombocythemia). The purpose of this article is to give diagnostic orientation and to suggest which biological tests should be performed.

Acute childhood leukaemia and environmental exposure to potential sources of benzene and other hydrocarbons; a case-control study.

AIM: To analyse the association between potential environmental exposure to hydrocarbons and the risk of acute childhood leukaemia. METHODS: A hospital based multicentre case control study, stratified on centre, age, and sex, with 280 leukaemia cases and 285 controls was carried out. Data were collected by a standardised interview of the mothers. RESULTS: No clear association was seen between maternal occupational exposure to hydrocarbons during pregnancy and leukaemia, or between residential traffic density and leukaemia. There was an association between dwellings neighbouring a petrol station or a repair garage during childhood and the risk of childhood leukaemia (OR 4.0, 95% CI 1.5 to 10.3), with a duration trend. The association, which appeared particularly strong for acute non-lymphocytic leukaemia (OR 7.7, 95% CI 1.7 to 34.3), was not altered by adjustment for potential confounding factors. CONCLUSIONS: Results showed an association between acute childhood leukaemia and dwellings neighbouring auto repair garages and petrol stations, which are benzene emitting sources. These findings could be due to chance, although the strength of the association and the duration trend are arguments for a causal association.

Outcome of 27 patients with Hurler's syndrome transplanted from either related or unrelated haematopoietic stem cell sources.

Over the last 15 years, we have performed a total of 30 haematopoietic stem cell transplants on 27 children suffering from Hurler's syndrome. These children were of median age 11 months at the time of diagnosis and 25 months at the time of transplantation. The phenotype was severe in 21 cases (78%). The donor was familial in 13 cases: nine genotypically identical, one phenotypically identical father and three HLA-mismatched donors. Unrelated donors were selected in 17 cases: four phenotypically identical and 13 with 1-4 HLA mismatches. The conditioning regimen generally consisted of busulphan 600 mg/m(2) plus cyclophosphamide (Endoxan) 260 mg/kg and cyclosporin with methotrexate for GvHD prophylaxis. Rabbit anti-thymocyte globulin (Thymoglobuline) was given for all unrelated or familial mismatched transplantations. The median nucleated cell dose infused was 6.00 x 10(8) TNC/kg. No bone marrow (apart from one) was T cell depleted. For first transplants, engraftment was observed in 23/27 patients (pts) (85%). Primary graft failure was observed in 4/27 patients (16%), two were retransplanted from an unrelated donor, one with success. Four patients have died. The primary cause of death was infection in three cases (TRM : 11%) and disease progression in one case, after primary graft failure. Of the 23 living patients, two have disease progression after graft failure and 21 (78%) have functional grafts with a favourable long-term outcome after a median follow-up of 4.7 years, having either full or mixed chimaerism. Among surviving patients with functional grafts, 13 (62%) were transplanted from unrelated donors of whom 10 (77 %) had HLA disparities. There was a remarkably low incidence of GvHD. In our experience, haematopoietic stem cell transplantation using an HLA-matched familial donor or an HLA-matched or -mismatched unrelated donor without T cell depletion or irradiation can achieve a favourable outcome in Hurler's syndrome, with improved cognitive function, but with a limited effect on the corneas and skeleton.

Epstein-Barr virus-related lymphoproliferative disease complicating childhood acute lymphoblastic leukemia: no recurrence after unrelated donor bone marrow transplantation.

A 16-year-old boy in complete remission of ALL, undergoing oral maintenance therapy, developed intestinal perforation related to EBV-associated lymphoproliferative disease (LPD). He was successfully managed with surgical resection, acyclovir, immunoglobulins and discontinuation of maintenance therapy. Leukemic marrow relapse occurred 3 months later, treated by polychemotherapy followed by unmanipulated BMT from a matched unrelated EBV seropositive donor. Donor lymphocytes were infused twice after transplant because of delayed immunologic recovery and severe CMV colitis. This was followed by acute GVHD requiring prolonged immunosuppressive treatment. Despite intensive and prolonged immunosuppression, recurrence of LPD was not observed. Following EBV-related LPD, allogeneic BMT can be performed if indicated. Selection of an EBV seropositive donor is of major importance for the prevention of LPD recurrence as the recipient may be protected by passive transfer of EBV-specific cytotoxic T cells.

Renal enlargement as presentation of isolated renal relapse in childhood leukemia.

Extramedullary relapses in children with acute lymphoblastic leukemia occur most frequently in the central nervous system and in the testis. In this report, the authors describe a 16-year-old girl with an isolated renal relapse of acute lymphoblastic leukemia after a disease-free interval of 2 years and 8 months. This clinically inconspicuous renal relapse was suggested by a routine follow-up renal sonography. No evidence of disease was found in bone marrow or peripheral blood. Renal biopsy was required to establish the diagnosis. Treatment consisted of intensive chemotherapy and autologous bone marrow transplantation. The patient has been in second complete continuous remission for 7 years. The authors recommend the use of an intensive multidrug salvage regimen.

[A rare cause of hyponatremia during introductory treatment of acute lymphoblastic leukemia in an infant: inappropriate secretion of atrial natriuretic factor?]. / Une cause rare d'hyponatrémie lors du traitement d'induction d'une leucémie aiguë lymphoblastique chez un nourrisson: la sécrétion inappropriée de facteur atrial natriurétique?

BACKGROUND: --Hyponatremia is frequently seen during the treatment of acute lymphoblastic leukemia: its causes are numerous. This work aims to present a case in whom hyponatremia was possibly due to an increased secretion of atrial natriuretic factor. CASE REPORT: --A 3 week-old baby was admitted because of malignant hemopathy. A diagnosis of acute lymphoblastic leukemia was rapidly made and the patient was firstly given alkaline diuresis, urate-oxidase and corticosteroids. Vincristine and daunorubicin were associated one week later. Insertion of a central intravenous line in the right subclavicular artery failed so that this catheter was finally inserted into the left jugular vein. Natremia was 126 mmol/l at that time and dramatically decreased within 24 hours to 109 mmol/l without net changes in water and electrolytic input. At that time, sodium urinary excretion was 6 mmol/kg/day (diuresis: 420 mlF/day). There was no hemodynamic changes, nor digestive or cardiac manifestations. Ultrasonography showed that the left superior cava vein drained into the right cardiac atrium. The catheter was withdrawn and the patient was given sodium supplementation permitting complete and definitive cure of hyponatremia within 2 days. CONCLUSIONS: --All usual causes of hyponatremia having been ruled out in this patient, we postulate that hyponatremia was due to direct stimulation of atrial natriuretic peptide through an increase in atrial pressure secondary to the catheter insertion near the cardiac atrium.