Ciccocioppo R.,University of Pavia |
Comoli P.,Pediatric Hematology Oncology Unit |
Gallia A.,University of Pavia |
Basso S.,Pediatric Hematology Oncology Unit |
And 2 more authors.
Journal of Clinical Immunology | Year: 2014
Purpose: Patients affected by primary immunodeficiency usually undergo a wide range of infections, including reactivation of latent ones. Here we report two cases suffering from late-onset combined immunodeficiency in which ulcerative enteritis due to human Cytomegalovirus caused a life-threatening malabsorption syndrome.Methods: The assessment of the viral load was carried out on both blood and mucosal samples by quantitative real-time polymerase chain reaction assay. The generation of autologous virus-specific cytotoxic T cell lines was performed according to Good Manufacturing Practice protocol after peripheral blood mononuclear cells were collected through a single leukapheresis.Results: In both patients, the viral load resulted negligible in peripheral blood, but very high in mucosal specimens (range 1.064 - 1.031.692 copies/105 cells). After two rounds of antiviral therapy proved unsuccessful, the generation of virus-specific cytotoxic T cell lines was carried out despite severe lymphopenia, and their infusion resulted safe and durably effective in healing intestinal ulcerations and resetting the viral load.Conclusions: Virus-specific cellular therapy was useful in reconstituting specific immunity and treating severe human Cytomegalovirus-related enteritis in patients with primary immunodeficiency. © 2014, Springer Science+Business Media New York.
Datta S.,PGIMER |
Bansal D.,Pediatric Hematology Oncology Unit |
Garg P.,University of Sydney
Indian Pediatrics | Year: 2014
In this single blinded parallel-group randomized controlled trial (RCT), preterm very-low-birth-weight (VLBW) infants received early iron (EI) supplementation (starting at 2 weeks postnatal age), or late iron (LI) supplementation (starting at 6 weeks postnatal age) . The primary outcome was serum ferritin level at 12 weeks, and the secondary outcomes were the incidence of neonatal morbidities, hemoglobin level, anthropometric parameters and blood transfusion requirements. Outcomes were analyzed in 46 and 47 babies in EI and LI groups, respectively. Serum ferritin level was significantly higher (P<0.001) at 12 weeks in the EI group. Hemoglobin and mean corpuscular hemoglobin concentration (MCHC) were also significantly (P<0.001) higher at 12 weeks in the EI group. There were no significant differences in the incidences of neonatal morbidities [necrotizing enterocolitis (NEC), periventricular leukomalacia, retinopathy of prematurity (ROP)], anthropometric parameters and blood transfusion requirements between the two groups. The authors concluded that EI supplementation in preterm VLBW infants improves serum ferritin and hemoglobin levels.
Das A.,Pediatric Hematology oncology Unit |
Bansal D.,Pediatric Hematology oncology Unit |
Bansal D.,Hematology oncology Unit |
Das R.,PGIMER |
And 2 more authors.
Indian Pediatrics | Year: 2014
Objective: To describe profile of 82 children with hereditary spherocytosis diagnosed over a period of 27 years (1985-2011) from a single center. Methods: Retrospective analyses of case records. Results: The mean (SD) age at diagnosis was 6.7 (2.8) years; 7 (8.5%) were diagnosed in infancy. Pallor (100%), icterus (67%), undocumented fever (28%), splenomegaly (96%) and hepatomegaly (73%) were the most frequent findings. Cholelithiasis was observed in 26%. Twenty-six (32%) underwent splenectomy and were followed for a median duration of 4.5 years. Two (7.7%) children developed postsplenectomy sepsis. Conclusion: Anemia, hepato-splenomegaly and jaundice are commonest clinical features of hereditary spherocytosis. Post-splenectomy sepsis is uncommon. © 2014 Indian Academy of Pediatrics.
Das A.,Pediatric Hematology Oncology Unit |
Bansal D.,Pediatric Hematology Oncology Unit |
Ahluwalia J.,Institute of Medical Education and Research |
Das R.,Institute of Medical Education and Research |
And 4 more authors.
Pediatric Blood and Cancer | Year: 2014
Background: The aim was to study risk-factors for vascular thrombosis and incidence of pulmonary artery hypertension (PAH) in splenectomized children with hereditary spherocytosis (HS) at a single center. Procedure: Pre- and post-splenectomy hemoglobin and platelet counts were recorded. Post-splenectomy lipid-profile, fibrinogen, D-dimer, CRP and anti-coagulant-protein levels were compared to established controls. Echo-Doppler was performed for PAH. Results: Twenty-six children with HS had undergone splenectomy; the mean age at surgery was 7.9±3.7 years. Nineteen of the 26 were prospectively investigated at a median duration of 4.5 years (range: 4 months to 19 years) following splenectomy. Thrombocytosis was observed in 19 (73%), whereas no patient had erythrocytosis at the last follow-up visit. Total cholesterol, LDLC, HDL-C, and triglyceride levels were not deranged (P≥0.3). Mean CRP levels (males: 2.8±0.5; females: 2.1±0.5 mg/L) were significantly higher than described for normal children (P<0.001). Six (23%) patients had a positive D-dimer assay. Protein S, antithrombin- III and fibrinogen were in range. A single patient had a borderline low protein C activity. Lupus anticoagulant and anticardiolipin antibody assays were negative. The mean tricuspid regurgitant jet velocity (TRJV) was 1.8±0.55 meter per second (range: 0-2.4). None had a TRJV ≤2.5 meter per second to suggest PAH. Conclusions: There was no evidence of PAH, dyslipidemia, elevation of fibrinogen or a reduction in anti-coagulant proteins, at a median follow-up duration of 4.5 years following splenectomy in children with HS. However, elevated CRP level (42%), persistent thrombocytosis (73%) and elevated D-dimer levels (23%) were observed. These have been recognized as risk factors for cerebrovascular and coronary heart disease. © 2013 Wiley Periodicals, Inc.
Montagna M.,Foundation IRCCS Policlinico San Matteo |
Montillo M.,Niguarda Hospital |
Avanzini M.A.,Pediatric Hematology Oncology Unit |
Tinelli C.,Clinical Epidemiology and Biometric Unit |
And 8 more authors.
Haematologica | Year: 2011
Alemtuzumab serum levels and clinical response after subcutaneous administration (10 mg 3 times/week for six weeks) have been explored in 29 chronic lymphocytic leukemia patients receiving the monoclonal antibody as consolidation. Serum concentrations after each administration gradually increased during the first week and more markedly during weeks 2 and 3, approaching the steady-state at week 6. Absorption continued slowly through the tissues for about 2- 3 weeks after the last administration, starting to decrease thereafter. Difference between Responders and Non-responders was statistically significant: maximal concentration (Cmax) was 1.69 μg/mL vs. 0.44 μg/mL; concentration before subcutaneous administration (Cpre-dose) on day 15 was 0.7 vs. 0.21 μg/mL, area under curve (AUC0-12h) was 11.09 vs. 2.26 μg x h/mL for Responders and Non-responders, respectively. Higher systemic exposure to alemtuzumab correlated with a better clinical response and minimal residual disease. Results suggest that an adjusted schedule according to serum level could improve clinical outcome of patients receiving subcutaneous alemtuzumab. © 2011 Ferrata Storti Foundation.
Hagag A.,Pediatric Hematology Oncology Unit |
Shebl S.,Pediatric Hematology Oncology Unit |
El-Fadaly N.,Tanta University
South Asian Journal of Cancer | Year: 2014
Background: Molecular cytogenetic abnormalities involving 11q23 are among the most common cytogenetic abnormalities in acute myeloid leukemia (AML) patients.Aim of the work: we aimed to evaluate the frequency of MLL/AF9 fusion gene in de novo AML patients, its impact on clinical features, and its prognostic significance.Patients and methods: Twenty-eight children patients with AML and 20 healthy controls were subjected to complete clinical examination and laboratory investigations including, complete hemogram and bone marrow (BM) examination. Diagnosis was based on FAB morphologic and immunophenotypic criteria. Detection of (MLL/AF9) fusion gene was assessed by dual color fluorescent in situ hybridization (FISH). Follow-up were carried out clinically and by blast count in BM, and response to therapy to detect the outcome of the disease.Results: The incidence of MLL-fusion gene MLL/AF9 in AML cases was about (6/28) (21%). Four patients with MLL/AF9 fusion gene were newly diagnosed, two cases were at relapse and no patient at remission showed positivity. As regard the clinical outcome, five out of six MLL positive cases died, three of them during induction and two during relapse. The FAB AML subtypes with MLL/AF9 fusion were one M2, three M4, and two M5.Conclusion: MLL-fusion gene MLL/AF9 was found in about 21% of studied AML patients when assessed by FISH technique and this is of high clinical relevance as most of these abnormalities have been associated with poor prognosis.
Todeschini G.,University of Verona |
Bonifacio M.,University of Verona |
Tecchio C.,University of Verona |
Balter R.,Pediatric Hematology Oncology Unit |
And 10 more authors.
American Journal of Hematology | Year: 2012
The optimal treatment of advanced sporadic Burkitt lymphoma in adults is still a matter of debate. The salutary results of pediatric therapies did open the road for improving the adult outcome. Between May 1988 and March 2009, 71 consecutive patients-46 adults, 25 children-affected by Burkitt lymphoma/leukemia were treated with the same intensive pediatric protocol alternating vincristine, adriamycine and fractionated ciclophosphamide (phase A) with high dose methotrexate and high dose cytarabine (phase B) in four Italian institutions. Eighty-nine per cent of patients were in Stage III-IV or had L3 leukemia. Complete remissions were 67/71 (94.4%), 24/25 (96%) in children, and 43/46 (93.5%) in adults. Toxic deaths were 3/71 (4.2%), all in adults. There were nine relapses (one in children, eight in adults), all but one observed early. After a median observation of 94 months (range 23-275), the Event-Free Survival rate is 92% in children and 71.7% in adults (P = 0.067). The 23 more recent adults received also rituximab, without differences in outcome as compared to patients who did not. Our experience confirms that such an intensive pediatric-derived chemotherapy is feasible and improves the long-term outcome of adults with advanced Burkitt lymphoma. © 2011 Wiley Periodicals, Inc.
PubMed | Pediatric Hematology Oncology Unit
Type: Journal Article | Journal: Pediatric blood & cancer | Year: 2013
The aim was to study risk-factors for vascular thrombosis and incidence of pulmonary artery hypertension (PAH) in splenectomized children with hereditary spherocytosis (HS) at a single center.Pre- and post-splenectomy hemoglobin and platelet counts were recorded. Post-splenectomy lipid-profile, fibrinogen, D-dimer, CRP and anti-coagulant-protein levels were compared to established controls. Echo-Doppler was performed for PAH.Twenty-six children with HS had undergone splenectomy; the mean age at surgery was 7.9 3.7 years. Nineteen of the 26 were prospectively investigated at a median duration of 4.5 years (range: 4 months to 19 years) following splenectomy. Thrombocytosis was observed in 19 (73%), whereas no patient had erythrocytosis at the last follow-up visit. Total cholesterol, LDL-C, HDL-C, and triglyceride levels were not deranged (P 0.3). Mean CRP levels (males: 2.8 0.5; females: 2.1 0.5 mg/L) were significantly higher than described for normal children (P < 0.001). Six (23%) patients had a positive D-dimer assay. Protein S, anti-thrombin-III and fibrinogen were in range. A single patient had a borderline low protein C activity. Lupus anticoagulant and anti-cardiolipin antibody assays were negative. The mean tricuspid regurgitant jet velocity (TRJV) was 1.8 0.55 meter per second (range: 0-2.4). None had a TRJV 2.5 meter per second to suggest PAH.There was no evidence of PAH, dyslipidemia, elevation of fibrinogen or a reduction in anti-coagulant proteins, at a median follow-up duration of 4.5 years following splenectomy in children with HS. However, elevated CRP level (42%), persistent thrombocytosis (73%) and elevated D-dimer levels (23%) were observed. These have been recognized as risk factors for cerebrovascular and coronary heart disease.
PubMed | Jawaharlal Institute of Postgraduate Medical Education & Research and Pediatric Hematology Oncology Unit
Type: | Journal: Pediatric blood & cancer | Year: 2016
The study aims to validate a score predicting risk of complications in pediatric patients with chemotherapy-related febrile neutropenia (FN) and evaluate the performance of previously published models for risk stratification.Children diagnosed with cancer and presenting with FN were evaluated in a prospective single-center study. A score predicting the risk of complications, previously derived in the unit, was validated on a prospective cohort. Performance of six predictive models published from geographically distinct settings was assessed on the same cohort.Complications were observed in 109 (26.3%) of 414 episodes of FN over 15 months. A risk score based on undernutrition (two points), time from last chemotherapy (<7 days = two points), presence of a nonupper respiratory focus of infection (two points), C-reactive protein (>60mg/l= five points), and absolute neutrophil count (<100 per l = two points) was used to stratify patients into low risk (score <7, n = 208) and assessed using the following parameters: overall performance (Nagelkerke RAn indigenous decision rule using five simple predefined variables was successful in identifying children at risk for complications. Prediction models derived in developed nations may not be appropriate for low-middle-income settings and need to be validated before use.
Recommendations for the use of long-term central venous catheter (CVC) in children with hemato-oncological disorders: management of CVC-related occlusion and CVC-related thrombosis On behalf of the coagulation defects working group and the supportive therapy working group of the Italian Association of Pediatric Hematology and Oncology (AIEOP)
PubMed | University of Bari, Giannina Gaslini Childrens Hospital, Pediatric Hematology Oncology and Bone Marrow Unit, University of Turin and 3 more.
Type: Journal Article | Journal: Annals of hematology | Year: 2015
Central venous catheters (CVC), used for the management of children with hemato-oncological disorders, are burdened by a significant incidence of mechanical, infective, or thrombotic complications. These complications favor an increasing risk in prolongation of hospitalization, extra costs of care, and sometimes severe life-threatening events. No guidelines for the management of CVC-related occlusion and CVC-related thrombosis are available for children. To this aim, members of the coagulation defects working group and the supportive therapy working group of the Italian Association of Pediatric Hematology and Oncology (AIEOP) reviewed the pediatric and adult literature to propose the first recommendations for the management of CVC-related occlusion and CVC-related thrombosis in children with hemato-oncological disorders.