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1.
Microorganisms ; 9(9)2021 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-34576740

RESUMO

There is mounting evidence for the emerging role of gut microbiota (GM) and its metabolites in profoundly impacting allogenic hematopoietic stem cell transplantation (allo-HSCT) and its subsequent complications, mainly infections and graft versus host-disease (GvHD). The present study was performed in order to investigate changes in GM composition and fecal metabolic signature between transplant patients (n = 15) and healthy controls (n = 18). The intestinal microbiota was characterized by NGS and gas chromatography-mass spectrometry was employed to perform untargeted analysis of fecal metabolites. We found lower relative abundances of Actinobacteria, Firmicutes, and Bacteroidetes and a higher abundance of Proteobacteria phylum after allo-HSCT. Particularly, the GvHD microbiota was characterized by a lower relative abundance of the short-chain fatty acid-producing bacteria, namely, the Feacalibacterium, Akkermansia, and Veillonella genera and the Lachnospiraceae family, and an enrichment in multidrug-resistant bacteria belonging to Escherichia, Shigella, and Bacteroides. Moreover, network analysis showed that GvHD was linked to a higher number of positive interactions of Blautia and a significant mutual-exclusion rate of Citrobacter. The fecal metabolome was dominated by lipids in the transplant group when compared with the healthy individuals (p < 0.05). Overall, 76 metabolites were significantly altered within transplant recipients, of which 24 were selected as potential biomarkers. Furthermore, the most notable altered metabolic pathways included the TCA cycle; butanoate, propanoate, and pyruvate metabolisms; steroid biosynthesis; and glycolysis/gluconeogenesis. Specific biomarkers and altered metabolic pathways were correlated to GvHD onset. Our results showed significant shifts in gut microbiota structure and fecal metabolites characterizing allo-HSCT.

2.
Acta Microbiol Immunol Hung ; 68(2): 73-79, 2021 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-33999858

RESUMO

The purpose of our study was to investigate the epidemiology of coagulase negative staphylococci (CoNS) responsible for bacteremia in hematopoietic stem cell transplant (HSCT) recipients and to determine the prevalence and the genetic background of methicillin resistance. The prevalence of CoNS bacteremia was 7.4% (54/728), higher in allograft (10.7%) than in autograft (4.7%) recipients. A sepsis or a septic shock were observed in 9% of cases. No deaths were attributable to CoNS bacteremia. The methicillin resistance rate was 81%. All MR-CoNS, harbored mecA gene and 90% were typeable with SCCmec typing using PCR amplification. The SCCmec type IV was the most frequent (44%). Clonal dissemination of MR- Staphylococcus epidermidis strains was limited. Our study showed a low prevalence and favorable outcome of CoNS bacteremia in HSCT recipients with limited clonal diffusion. However, they were associated with a significant rate of severe infections and a high rate of methicillin resistance, mediated by SCCmec IV element in most cases.


Assuntos
Bacteriemia/epidemiologia , Coagulase/genética , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Infecções Estafilocócicas/epidemiologia , Staphylococcus/genética , Staphylococcus/patogenicidade , Adolescente , Adulto , Antibacterianos/farmacologia , Bacteriemia/microbiologia , Proteínas de Bactérias/genética , Criança , Coagulase/análise , DNA Bacteriano/genética , Feminino , Humanos , Masculino , Resistência a Meticilina/genética , Testes de Sensibilidade Microbiana , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Infecções Estafilocócicas/complicações , Infecções Estafilocócicas/etiologia , Staphylococcus/efeitos dos fármacos , Staphylococcus/enzimologia , Staphylococcus epidermidis/efeitos dos fármacos , Staphylococcus epidermidis/genética , Tunísia/epidemiologia , Adulto Jovem
3.
Tunis Med ; 99(2): 269-276, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33899198

RESUMO

BACKGROUND: Bacteremia become fearsome in hematopoietic stem cell transplant (HSCT) recipients with the emergence of multidrug-resistant (MDR) strains. AIM: Our purpose was to investigate the prevalence of MDR bacteremia in HSCT recipients at the Tunisian National Bone Marrow Transplant Center, associated factors and attributable mortality rate. METHODS: Our retrospective study (January 2010-December 2017) included all MDR bacteremia in the Hematology department. MDR rods were: extended spectrum beta-lactamase producing Enterobacterales (ESBL-E), P. aeruginosa and A. baumannii resistant to at least three families of antibiotics, methicillin-resistant S. aureus (MRSA) and vancomycin resistant E. faecium (VRE). RESULTS: The prevalence of MDR bacteremia among HSCT recipients was 5.9% (48/816) with a stable trend over time (rs=0.18). Neutropenia, prior hospitalization, prior antibiotherapy and prior colonization with MDR pathogens were observed in 59%, 58%, 48% and 31% of cases, respectively. Imipenem was the most prescribed first-line antibiotic (50%). The attributable mortality rate was 13%. MDR bacteria (n=48) belonged to ESBL-E (60%), P. aeruginosa (19%), A. baumannii (13%), MRSA (4%) and VRE (4%). For ESBL-E and P. aeruginosa, the rates of antibiotic resistance were respectively, 17% and 44% to imipenem, 31% and 56% to amikacin and 15% and 0% to colistin. Strains of A. baumannii were susceptible only to colistin. The MRSA (n=2) were resistant to ciprofloxacin and gentamicin and susceptible to glycopeptides. The VRE (n=2) were susceptible to linezolid and tigecycline. CONCLUSION: Low prevalence of MDR bacteremia in HSCT recipients but high attributable mortality rate, requiring reinforcement of hygiene measures.


Assuntos
Bacteriemia , Transplante de Células-Tronco Hematopoéticas , Staphylococcus aureus Resistente à Meticilina , Antibacterianos/uso terapêutico , Bacteriemia/tratamento farmacológico , Bacteriemia/epidemiologia , Farmacorresistência Bacteriana Múltipla , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Testes de Sensibilidade Microbiana , Estudos Retrospectivos
4.
Rep Pract Oncol Radiother ; 25(3): 436-441, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32372884

RESUMO

AIM: The objective of the study was to estimate the cumulative incidence (CI) of relapse, relapse-free survival (RFS) and overall survival (OS) in ALL patients after a once-a-day fractionated TBI (F-TBI) regimen with 9.9 Gy. The secondary objectives were evaluation of short and long-term toxicity and non-relapse mortality (NRM). BACKGROUND: Total body irradiation (TBI), as a part of the conditioning regimen before allogeneic stem cell transplantation (ASCT) for acute lymphoblastic leukemia (ALL), allows disease control by eradicating residual blast cells in the transplant recipient. MATERIALS AND METHODS: Retrospective study conducted in patients with ALL who received between March 2003 and December 2013 a conditioning regimen with F-TBI and chemotherapy. Irradiation was delivered with 3.3 Gy once-a-day for three consecutive days. RESULTS: Eighty-seven patients were included. The median age was 19 years (range: 5-49 years). The 3-year CI of relapse was 30%. The estimated 3-year RFS and OS were 54% and 58%, respectively. Cumulative incidence of acute graft-versus-host disease (aGVHD) grade II-IV and chronic GVHD (cGVHD) was 31% and 40%, respectively. Interstitial pneumonitis was observed in 2 patients. The 3-year CI of NRM was 16%. In multivariate analysis, cGVHD was associated with a lower CI of relapse (RR = 0.26, 95% CI: 0.07-0.95, p = 0.04). High-risk cytogenetics was associated with a lower RFS (RR = 2, 95 CI: 1.04-3.84, p = 0.03). Grade II-IV aGVHD was an independent predictor of higher CI of NRM (RR = 6.7, 95% CI: 1.4-31.7, p = 0.02). CONCLUSIONS: Once-a-day F-TBI regimen is effective, safe and practical in patients who underwent ASCT for ALL.

5.
Biol Blood Marrow Transplant ; 24(12): 2459-2465, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30036571

RESUMO

Human recombinant granulocyte colony stimulating factor reduces the duration of neutropenia following HLA-identical allogeneic bone marrow transplantation. However, its use remains controversial due to the risk of increasing the incidence of acute graft-versus-host disease (GVHD) and slower platelet recovery. To clarify these risks, we conducted a prospective randomized placebo-controlled trial of filgrastim 5 µg/kg/day i.v. from day 7 post-transplant until neutrophil recovery in 145 consecutive adults undergoing HLA-identical allogeneic bone marrow transplantation, with cyclosporine and methotrexate as GVHD prophylaxis. The primary endpoint was the incidence of acute GVHD; hematological recovery, nonrelapse mortality, and post-transplant complications were secondary endpoints. Filgrastim had no significant effect on the incidence of acute GVHD, platelet recovery, platelet transfusion requirements, chronic GVHD, or survival. Filgrastim accelerated granulocyte recovery significantly (with absolute neutrophil counts >.5 × 109/L achieved after a median of 16 days versus 23 days for placebo; P < .0001), and reduced both early nonrelapse mortality (2.9% versus 10.5%; P = .042) and the duration of i.v. antibiotic therapy (18 days versus 26 days; P = .001) and hospitalization (27 versus 34 days; P = .017). In conclusion, in this setting, filgrastim reduced significantly the duration of neutropenia, i.v. antibiotic therapy, hospitalization, and early nonrelapse mortality, without increasing the risk of acute and chronic GVHD or relapse, or delaying platelet recovery.


Assuntos
Transplante de Medula Óssea/métodos , Filgrastim/uso terapêutico , Fármacos Hematológicos/uso terapêutico , Transplante Homólogo/métodos , Adolescente , Adulto , Feminino , Filgrastim/farmacologia , Fármacos Hematológicos/farmacologia , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Adulto Jovem
6.
Int J Antimicrob Agents ; 52(6): 886-892, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29783023

RESUMO

BACKGROUND: Extended spectrum ß-lactamase-producing Enterobacteriaceae (ESBL-E) create a therapeutic challenge and have high potential for dissemination. The purpose of our study was to investigate the epidemiology of these infections in hematopoietic stem cell transplant (HSCT) recipients and to determine the genes encoding ESBL. MATERIAL/METHODS: This retrospective study comprised adult patients hospitalized at the National Bone Marrow Transplant Center (NBMTC) and infected with ESBL-E post-HSCT between January 2006 and December 2016. The search for the ESBL and carbapenemase genes was performed by polymerase chain reaction (PCR) amplification. Molecular typing was performed by pulsed field gel electrophoresis (PFGE) after digestion with XbaI. RESULTS: Forty ESBL-E were responsible for infections in 34 HSCT recipients (3.3% of total HSCT recipients). Prior hospital stay, prior antibiotic therapy and prior colonization with ESBL-E were reported in 62.5%, 70% and 50% of the infectious episodes, respectively. The initial antibiotic treatment was appropriate in 67.7% of cases. Imipenem was the most prescribed antibiotic (64.5%). The mortality rate due to ESBL-E infection was 8.8%. The ESBL-E, isolated mainly from blood cultures (40%), belonged mostly to K. pneumoniae (n=19) and E. coli (n=17). Associated antibiotic resistance rates were 17.5% for ertapenem, 85% for ciprofloxacin and 30% for amikacin. The predominant gene encoding ESBL was blaCTX-M (55%). Among the seven carbapenem-resistant strains, four had the blaOXA-48 gene and two the blaKPC gene. There was no clonal relationship between the strains. CONCLUSION: There was low prevalence of ESBL-E infections in HSCT recipients in our center, with no epidemic distribution but non-negligible mortality rate.


Assuntos
Infecções por Enterobacteriaceae/epidemiologia , Infecções por Enterobacteriaceae/microbiologia , Enterobacteriaceae/enzimologia , Enterobacteriaceae/isolamento & purificação , Transplante de Células-Tronco Hematopoéticas , beta-Lactamases/genética , Adolescente , Adulto , Eletroforese em Gel de Campo Pulsado , Enterobacteriaceae/classificação , Enterobacteriaceae/genética , Infecções por Enterobacteriaceae/mortalidade , Feminino , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Epidemiologia Molecular , Tipagem Molecular , Reação em Cadeia da Polimerase , Prevalência , Estudos Retrospectivos , Análise de Sobrevida , Adulto Jovem
7.
Transpl Infect Dis ; 20(3): e12887, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29573075

RESUMO

A 27-year-old man with severe aplastic anemia underwent bone marrow transplantation from his HLA identical brother in July 2016. Conditioning included ATGAM 30 mg/kg for 3 days and Cyclophosphamide 50 mg/kg for 4 days. The patient received several platelet and red blood cell transfusions before and after the conditioning. The patient received broad spectrum antibiotics and caspofungin because persistant febrile neutropenia without bacteriological or mycological documentation. Hemophagocytic syndrome was diagnosed on day +12. Steroids at 1 mg/kg were started on day +12. Fever resolved the same day but resumed 3 days later associated to intravascular hemolysis with no schizocytes on blood smears and negative DAT. Thick blood film smears performed on day +26 revealed Plasmodium falciparum parasites (parasitemia = 20%). Except the level of parasitemia, there were no signs of gravity. Quinine was started on day 26 at a loading dose of 15 mg/kg followed by 8 mg/kg three times a day for 20 doses. Fever vanished after 2 days. Parasitemia cleared in 3 days and remained negative thereafter. Investigations revealed that the patient was transfused by a red cell unit harvested in a voluntary donor native of a malaria endemic country. PCR for P. falciparum performed in this donor in the frame of investigations was positive. The patient is alive with a normal blood count 1 year after BMT.


Assuntos
Transplante de Medula Óssea/efeitos adversos , Linfo-Histiocitose Hemofagocítica/parasitologia , Malária Falciparum/transmissão , Plasmodium falciparum/efeitos dos fármacos , Reação Transfusional/parasitologia , Adulto , Anemia Aplástica/complicações , Antimaláricos/uso terapêutico , Artemisininas/uso terapêutico , Transfusão de Sangue , Humanos , Linfo-Histiocitose Hemofagocítica/etiologia , Malária Falciparum/tratamento farmacológico , Malária Falciparum/parasitologia , Masculino , Parasitemia/tratamento farmacológico , Plasmodium falciparum/isolamento & purificação , Resultado do Tratamento
8.
Ann Hematol ; 97(4): 597-604, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29305630

RESUMO

Data are limited in developing countries regarding the clinicopathologic features and response to therapy of chronic myeloid leukemia (CML) in the era of imatinib (IM). The objective of this study is to report on the clinicoepidemiologic features of CML in Tunisia, to evaluate the long-term outcome of patients in chronic (CP) or accelerated phase (AP) treated with IM 400 mg daily as frontline therapy, and to determine imatinib's efficacy and safety. From October 2002 to December 2014, 410 CML patients were treated with IM in six Tunisian departments of hematology. Response (hematologic, cytogenetic, and molecular responses) and outcome-overall survival (OS), event-free survival (EFS), and progression-free survival (PFS)-were evaluated. The following prognostic factors were analyzed for their impact on the European leukemia net (ELN) response, OS, EFS, and PFS at 5 years: age, sex, leukocyte count, Sokal score, European Treatment and Outcome Study (EUTOS) score, CML phase, time to starting IM, and impact of adverse events. The median age was 45 years (3-85 years). Two hundred ten (51.2%) patients were male. Splenomegaly was present in 322 of the 410 (79%). Additional cytogenetic abnormalities were encountered in 25 (6.3%) patients. At diagnosis, 379 (92.4%) patients were in CP, 31 (7.6%) were in AP. The Sokal risk was low in 87 (22.5%), intermediate in 138 (35.7%), and high in 164 patients (41.9%). The EUTOS risk was low in 217 (74%), and high in 77 (26%) patients. The rates of cumulative complete cytogenetic response (CCyR), major molecular response (MMR), and molecular response 4/5 log (MR4.5) in CP/AP-CML patients were 72, 68.4, and 46.4%, respectively. The median time to reach CCyR, MMR, and MR4.5 was 6 months (3-51), 18 months (3-72), and 24 months (3-100), respectively. According to the ELN criteria, optimal, suboptimal response, and failure were noted in 206 (51.8%), 61 (15.3%), and 125 (31.4%) patients, respectively. Five-year event-free survival (EFS), progression-free survival (PFS), and overall survival (OS) were 81, 90, and 90%, respectively. By multivariate analysis, AP, high EUTOS risk, and baseline WBC ≥ 150G/l remained independent predictive factors of non-optimal response to IM. The adverse events (AE) of IM were moderate and tolerable. With the caveats that the monitoring of the disease was not optimal, response rates were similar to those reported in previous studies. It is clear to us that improvements should be made in treatment of AP-CML and high Sokal risk group of CP-CML. The frontline use of second-generation tyrosine kinase inhibitor (TKI) is expected to improve the results of the first-line treatment of these high-risk Tunisian patients, but cost and accessibility of this therapy remain the problems in developing countries.


Assuntos
Antineoplásicos/uso terapêutico , Mesilato de Imatinib/uso terapêutico , Leucemia Mieloide de Fase Acelerada/tratamento farmacológico , Leucemia Mieloide de Fase Crônica/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/efeitos adversos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Mesilato de Imatinib/efeitos adversos , Leucemia Mieloide de Fase Acelerada/diagnóstico , Leucemia Mieloide de Fase Acelerada/epidemiologia , Leucemia Mieloide de Fase Acelerada/patologia , Leucemia Mieloide de Fase Crônica/diagnóstico , Leucemia Mieloide de Fase Crônica/epidemiologia , Leucemia Mieloide de Fase Crônica/patologia , Masculino , Pessoa de Meia-Idade , Padrões de Prática Médica , Prognóstico , Inibidores de Proteínas Quinases/efeitos adversos , Estudos Retrospectivos , Esplenomegalia/etiologia , Esplenomegalia/patologia , Esplenomegalia/prevenção & controle , Análise de Sobrevida , Carga Tumoral/efeitos dos fármacos , Tunísia/epidemiologia , Adulto Jovem
9.
Saudi J Kidney Dis Transpl ; 25(5): 1062-4, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25193909

RESUMO

Disturbed kidney function is a common complication after bone marrow transplantation. Recently, attention has been given to immune-mediated glomerular damage related to graft versus host disease (GVHD). We describe a 19-year-old woman who developed membranous glomerulonephritis after bone marrow transplantation (BMT). Six months later, she developed soft palate, skin and liver lesions considered to be chronic GVHD. Fifteen months after undergoing BMT, this patient presented with nephrotic syndrome. A renal biopsy showed membranous glomerulonephritis associated with a focal segmental glomerulosclerosis. She was started on corticosteroid treatment with good outcome.


Assuntos
Transplante de Medula Óssea/efeitos adversos , Glomerulonefrite Membranosa/etiologia , Glomerulosclerose Segmentar e Focal/etiologia , Doença Enxerto-Hospedeiro/etiologia , Leucemia Mielogênica Crônica BCR-ABL Positiva/cirurgia , Síndrome Nefrótica/etiologia , Corticosteroides/uso terapêutico , Biópsia , Feminino , Glomerulonefrite Membranosa/diagnóstico , Glomerulonefrite Membranosa/tratamento farmacológico , Glomerulosclerose Segmentar e Focal/diagnóstico , Glomerulosclerose Segmentar e Focal/tratamento farmacológico , Doença Enxerto-Hospedeiro/diagnóstico , Doença Enxerto-Hospedeiro/tratamento farmacológico , Humanos , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
10.
Tunis Med ; 91(5): 337-41, 2013 May.
Artigo em Francês | MEDLINE | ID: mdl-23716328

RESUMO

BACKGROUND: Interleukine 6 (IL-6) is the most important cytokine involved in malignant plasma cells growth and survival. AIM: To analyse bone marrow plasma cells IL6 receptor gene expression in both multiple myeloma patients at diagnosis and healthy bone marrow donors. METHODS: Clinical and biological patients' features and responses to Dexamethasone-Thalidomide induction therapy were gathered. 47 patients and 16 case controls were analyzed: Bone marrow plasma cells were isolated; and IL6 receptor gene expression was quantified using Taqman quantitative PCr technology and 2-ΔCT formula. RESULTS: Quantitative and qualitative IL6 receptor gene expression were negatively correlated with the degree of response to therapy (p= 0.02). In this study, plasma cells IL6 receptor gene expression seems to be decisive in predicting the response to treatment. CONCLUSION: Understanding the mechanisms involved in plasma cells IL6 receptor gene expression may offer a better appreciation of the physiopathologic and anti-oncogenic ways of drug resistance in multiple myeloma and consequently the discovery of new specific drugs.


Assuntos
Quimioterapia de Indução , Mieloma Múltiplo/terapia , Plasmócitos/metabolismo , Receptores de Interleucina-6/genética , Adulto , Estudos de Casos e Controles , Feminino , Expressão Gênica , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Receptores de Interleucina-6/metabolismo
11.
J Pediatr Hematol Oncol ; 35(7): 547-50, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23337544

RESUMO

BACKGROUND: Chromosome breakage hypersensitivity to alkylating agents is the gold standard test for Fanconi anemia (FA) diagnosis. The aim of the present study was to assess the proportion of FA cases among aplastic anemia (AA) in Tunisian pediatric patients. OBSERVATION: Investigation of mitomycin C-induced chromosomal breakage was carried out in 163 pediatric patients with AA and siblings of the cases where diagnosis of FA was confirmed. We identified 31 patients with FA whose percentage of unstable mitoses ranges from 65% to 100%. Among 18 siblings who were investigated for chromosomal instability, 3 were incidentally found to be affected. CONCLUSIONS: FA is an important cause of AA in Tunisia. Our report is the first study in North Africa that explored cytogenetic and phenotypic findings in FA children. It also showed the importance of mitomycin C sensitivity screening in all FA siblings.


Assuntos
Anemia Aplástica/diagnóstico , Anemia Aplástica/genética , Análise Citogenética , Anemia de Fanconi/diagnóstico , Anemia de Fanconi/genética , Adolescente , Anemia Aplástica/complicações , Criança , Pré-Escolar , Instabilidade Cromossômica , Quebra Cromossômica/efeitos dos fármacos , Consanguinidade , Diagnóstico Diferencial , Anemia de Fanconi/complicações , Feminino , Humanos , Lactente , Masculino , Mitomicina/farmacologia , Tunísia
12.
Exp Clin Transplant ; 10(1): 76-80, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22309426

RESUMO

We report a case of autoimmune polyglandular syndrome type II that developed in an 11-year-old boy with homozygous sickle cell disease after allogeneic bone marrow transplant; the donor was his father, who was human leukocyte antigen identical and had vitiligo. On day 24 after transplant, the patient developed grade 1 acute graft-versus-host disease, which was controlled over a period of 3 months with corticosteroid-induced immunosuppression. Full donor engraftment was documented on day 31 after transplant, and this was further confirmed on days 59, 231, 321, 472, 549, and 720. Three months after transplant, the recipient developed adrenal insufficiency, and at 13 months, he developed vitiligo. Seventeen months after transplant, autoimmune thyroid disease, positive for thyroid peroxidase and thyroglobulin autoantibodies, was diagnosed. At the same time, we identified adrenal insufficiency in the donor. We analyzed a serum sample from the recipient for autoantibody markers for type 1 autoimmune diabetes mellitus. The sample was positive for antiglutamic acid decarboxylase. Antibody against 21-hydroxylase enzyme was also found (261 U/mL; normal value, < 1 U/mL). We conclude that the recipient developed autoimmune polyglandular syndrome type II after bone marrow transplant from his father, who was probably affected by the same syndrome.


Assuntos
Anemia Falciforme/cirurgia , Transplante de Medula Óssea/efeitos adversos , Poliendocrinopatias Autoimunes/etiologia , Poliendocrinopatias Autoimunes/genética , Doença de Addison/genética , Insuficiência Adrenal/genética , Criança , Predisposição Genética para Doença/genética , Humanos , Masculino , Poliendocrinopatias Autoimunes/diagnóstico , Doadores de Tecidos , Vitiligo/genética
13.
Exp Clin Transplant ; 8(3): 269-71, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20716049

RESUMO

In this article, we report a switch of beta-thalassemia major to intermedia beta-thalassemia after allogeneic bone marrow transplant of a 6-year-old girl from her HLA-matched brother. After stable mixed chimerism, the patient had a secondary graft rejection and returned to total recipient chimerism as assessed by real-time polymerase chain reaction assay. Nonetheless, with a medium hemoglobin rate of 89 g/L, she did not need further transfusions for 60 months after rejection. We conclude that complete loss of donor cells after bone marrow transplant for beta-thalassemia major is compatible with a stable clinical state, probably due to a gamma-globin gene demethylation that enhances gamma-globin chain production and further allows constitution of a fetal hemoglobin rate compatible with free transfusion survival.


Assuntos
Transplante de Medula Óssea/efeitos adversos , Rejeição de Enxerto/etiologia , Quimeras de Transplante , Talassemia beta/cirurgia , Criança , Família , Feminino , Hemoglobina Fetal/metabolismo , Rejeição de Enxerto/sangue , Rejeição de Enxerto/genética , Rejeição de Enxerto/imunologia , Antígenos HLA/imunologia , Hemoglobina A/metabolismo , Hemoglobina A2/metabolismo , Teste de Histocompatibilidade , Humanos , Reação em Cadeia da Polimerase , Fatores de Tempo , Quimeras de Transplante/genética , Condicionamento Pré-Transplante , Transplante Homólogo , Resultado do Tratamento , Talassemia beta/sangue , Talassemia beta/genética , Talassemia beta/imunologia
15.
Int J Hematol ; 89(3): 368-373, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19252966

RESUMO

The aim of this prospective observational study was to evaluate the incidence of hemophagocytic syndrome (HPS) after hematopoietic stem cell transplantation (HSCT). Between July 2006 and December 2007, all patients who received a HSCT in our institution were included in this study. All the following criteria were needed for the diagnosis of HPS: sustained fever over 7 days; cytopenia (neutropenia and/or thrombocytopenia); presence of more than 3% mature macrophages in bone marrow; hyperferritinaemia (>1,000 ng/mL). During this study, 171 patients received a HSCT (68 allogeneic and 103 autologous). The median age was 32 years (3-62). We observed six cases of HPS (6/68; 8.8%) after allogeneic stem cell transplantation (ASCT): one case of EBV-related HPS, two cases of CMV-related HPS, and three cases with no evidence of bacterial, fungal or viral infections. We observed only one case of CMV-related HPS (1/103; 0.9%) after autologous stem cell transplantation. Four patients died despite aggressive supportive care. To our knowledge, this is the first prospective observational study conducted with the aim to evaluate the incidence of HPS after HSCT. This study provides a relatively high incidence of HPS after ASCT. When sustained fever with progressive cytopenia and hyperferritinaemia are observed, HPS should be suspected, and bone marrow aspirate considered. The rapid diagnosis of HPS and the early initiation of an appropriate treatment are essential for patient management.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Linfo-Histiocitose Hemofagocítica/cirurgia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Ferritinas/sangue , Seguimentos , Doença Enxerto-Hospedeiro/imunologia , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/imunologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Transplante Homólogo/imunologia
16.
Blood ; 111(4): 1805-10, 2008 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-17875806

RESUMO

From April 2003 to December 2006, 195 patients with de novo symptomatic myeloma and younger than 60 years of age were randomly assigned to receive either tandem transplantation up front (arm A, n = 97) or one autologous stem-cell transplantation followed by a maintenance therapy with thalidomide (day + 90, 100 mg per day during 6 months) (arm B, n = 98). Patients included in arm B received a second transplant at disease progression. In both arms, autologous stem-cell transplantation was preceded by first-line therapy with thalidomide-dexamethasone and subsequent collection of peripheral blood stem cells with high-dose cyclophosphamide (4 g/m(2)) and granulocyte colony stimulating factor. Data were analyzed on an intent-to-treat basis. With a median follow-up of 33 months (range, 6-46 months), the 3-year overall survival was 65% in arm A and 85% in arm B (P = .04). The 3-year progression-free survival was 57% in arm A and 85% in arm B (P = .02). Up-front single autologous transplantation followed by 6 months of maintenance therapy with thalidomide (with second transplant in reserve for relapse or progression) is an effective therapeutic strategy to treat multiple myeloma patients and appears superior to tandem transplant in this setting. This study was registered at www.ClinicalTrials.gov as (NCT 00207805).


Assuntos
Mieloma Múltiplo/terapia , Transplante de Células-Tronco/métodos , Talidomida/uso terapêutico , Adulto , Inibidores da Angiogênese/uso terapêutico , Proteínas Sanguíneas/metabolismo , Terapia Combinada , Intervalo Livre de Doença , Feminino , Hemoglobinas/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/sangue , Mieloma Múltiplo/mortalidade , Mieloma Múltiplo/patologia , Estadiamento de Neoplasias , Seleção de Pacientes , Análise de Sobrevida , Fatores de Tempo , Coleta de Tecidos e Órgãos/métodos , Transplante Autólogo , Resultado do Tratamento
17.
Radiat Med ; 25(8): 407-10, 2007 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-17952545

RESUMO

PURPOSE: The aim of this study was to assess the impact of fractionated total body irradiation (F-TBI) on treatment-related mortality (TRM) and relapse in patients who received a non-T-cell-depleted allogeneic stem cell transplantation (ASCT) for hematological malignancies. MATERIALS AND METHODS: Between March 2003 and December 2004, a total of 24 patients with HLA-identical sibling donors entered this study and received three doses of 3.33 Gy F-TBI separated by 24 h and cyclophosphamide or etoposide. RESULTS: At a median follow-up of 37 months (range 29-47 months), 4 of the 24 patients (16.6%) died of TRM. Relapse occurred in 10 patients at a median of 9 months (range 2-18 months). Overall, 13 of 24 patients (54%) died. Relapse was the most common cause of death (9/13). The 2-year actuarial survival rate was 46% (+/-11%). CONCLUSION: In our experience, ASCT conditioned with F-TBI was associated with low TRM but a high early relapse rate in patients with hematological malignancies.


Assuntos
Transplante de Medula Óssea/efeitos adversos , Antígenos HLA/imunologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia/radioterapia , Linfoma não Hodgkin/radioterapia , Irradiação Corporal Total/efeitos adversos , Adolescente , Adulto , Criança , Feminino , Humanos , Leucemia/mortalidade , Leucemia/cirurgia , Linfoma não Hodgkin/mortalidade , Linfoma não Hodgkin/cirurgia , Masculino , Recidiva , Irmãos , Taxa de Sobrevida , Linfócitos T/imunologia , Transplante Homólogo , Irradiação Corporal Total/métodos
18.
J Support Oncol ; 5(6): 273-8, 2007 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17624052

RESUMO

Bloodstream infections related to the use of central venous catheters are an important cause of patient morbidity, mortality, and increased health care costs. Catheter-related infection may be due to fibrin deposition associated with catheters. Interventions designed to decrease fibrin deposition have the potential to reduce catheter-related infections. This study was a randomized, controlled trial in which 246 patients with nontunneled central venous catheters were randomly assigned to receive a heparin-coated catheter with 50 mL/d of normal saline solution as a continuous infusion (heparin-coated group) or a noncoated catheter with a continuous infusion of low-dose unfractionated heparin (control group: continuous infusion of 100 U/kg/d). Catheter-related bloodstream infection occurred in 2.5% (3/120 catheters) in the heparin-coated group (0.9 events per 1,000 days) and in 9.1% (11/120 catheters) in the control group (3.5 events per 1,000 days; P = 0.027). No other risk factors were found for the development of catheter-related bloodstream infection. Six and seven patients experienced severe bleeding in the heparin-coated and control groups, respectively (P = 1.00). We did not observe heparin-induced thrombocytopenia. The use of heparin-coated catheters can be a safe and effective approach to the prevention of catheter-related bloodstream infection in patients with hematooncologic disease.


Assuntos
Cateterismo Venoso Central/efeitos adversos , Cateterismo Venoso Central/instrumentação , Cateteres de Demora/efeitos adversos , Materiais Revestidos Biocompatíveis/uso terapêutico , Heparina de Baixo Peso Molecular/administração & dosagem , Sepse/prevenção & controle , Adolescente , Adulto , Anti-Infecciosos Locais/uso terapêutico , Anticoagulantes/uso terapêutico , Cateteres de Demora/microbiologia , Criança , Pré-Escolar , Infecção Hospitalar/prevenção & controle , Método Duplo-Cego , Feminino , Humanos , Infusões Intravenosas/métodos , Masculino , Pessoa de Meia-Idade , Sepse/etiologia
19.
Tunis Med ; 85(1): 35-8, 2007 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-17424707

RESUMO

AIM: In the present study we report the clinical outcome of 27 patients with refractory or relapsed Hodgkin's lymphoma (HL) undergoing autologous peripheral stem-cell transplantation (ASCT). METHODS: On transplant, 18 patients had sensitive disease (SD) and 9 resistant disease (RD). The median time between diagnosis and ASCT was 18 months (range, 7 to 96 months). The conditioning consisted of BEAM regimen. RESULTS: The 100-day mortality rate was 3%. Three months after transplant, 12 patients transplanted with SD were in complete remission (CR) and only one of the 9 patients transplanted with RD achieved CR. Overall survival and disease-free survival after 3 years were 68% and 60%, respectively. CONCLUSION: the present results confirm the efficacy and safety of the ASCT in refractory or relapsed HL patients with SD. Other strategies should be investigated for patients with RD.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/tratamento farmacológico , Doença de Hodgkin/terapia , Transplante de Células-Tronco de Sangue Periférico , Condicionamento Pré-Transplante , Adolescente , Adulto , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Carmustina/administração & dosagem , Carmustina/uso terapêutico , Criança , Citarabina/administração & dosagem , Citarabina/uso terapêutico , Interpretação Estatística de Dados , Intervalo Livre de Doença , Resistencia a Medicamentos Antineoplásicos , Etoposídeo/administração & dosagem , Etoposídeo/uso terapêutico , Seguimentos , Doença de Hodgkin/mortalidade , Doença de Hodgkin/patologia , Humanos , Melfalan/administração & dosagem , Melfalan/uso terapêutico , Estadiamento de Neoplasias , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Recidiva , Indução de Remissão , Fatores de Tempo , Transplante Autólogo , Resultado do Tratamento
20.
Blood Coagul Fibrinolysis ; 18(3): 279-81, 2007 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-17413766

RESUMO

A 55-year-old man with multiple myeloma developed sustained bleeding after bone marrow aspiration and cutaneous bleeding. Routine coagulation studies revealed a prolonged activated partial thromboplastin time and thrombin time (> 60 s) with a normal reptilase time. Further evaluation showed failure of the activated partial thromboplastin time to correct completely in a 1: 1 mixture with normal plasma. Treatment of the patient's plasma in vitro with protamine sulfate normalized the thrombin time. The presence of a heparin-like anticoagulant was suspected. The plasma heparin level was 0.73 IU/ml. Intravenous infusion of protamine sulfate appeared to neutralize the anticoagulant activity and stop the bleeding. The cancer cells themselves or the invasive nature of this type of cancer might result in a massive release of a heparinoid. Such coagulopathy appears to be a rare mechanism of bleeding and it is an important entity to consider since it is potentially reversible with protamine sulfate.


Assuntos
Hemorragia/etiologia , Mieloma Múltiplo/complicações , Protaminas/uso terapêutico , Testes de Coagulação Sanguínea , Hemorragia/induzido quimicamente , Hemorragia/tratamento farmacológico , Heparina/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade
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