Assuntos
Eritema , Humanos , Masculino , Eritema/etiologia , Eritema/diagnóstico , Eritema/patologia , Dermatoses da Mão/diagnóstico , Dermatoses da Mão/patologia , Dermatoses da Mão/etiologia , Mãos/patologia , Doença Aguda , Adulto , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/patologiaAssuntos
Doenças Autoimunes , Rituximab , Humanos , Rituximab/uso terapêutico , Rituximab/administração & dosagem , Doenças Autoimunes/tratamento farmacológico , Dermatopatias/tratamento farmacológico , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/uso terapêuticoRESUMO
ABSTRACT Background: Secondary immune thrombocytopenia (ITP) is a heterogeneous and unpredictable disease associated with various underlying conditions. Objective: The objective of the study was to investigate clinical evolution and chronicity predictors in secondary ITP. Methods: Patients treated at an academic medical center during 2008-2019 were stratified by age as children <16 years and adults >16 years. Responses to steroids, intravenous immunoglobulin G (IVIG), rituximab, and eltrombopag were classified as response (R) and complete response (CR). Risk factors for chronic ITP were determined by multiple regression with uni- and multi-variate analysis. Results: Eighty-three patients were included, 37 children and 46 adults. The most frequent associated conditions were infections 53%, systemic lupus erythematosus (SLE) 24%, thyroid disease 9.6%, and Evans syndrome 3.6%. Response to first-line treatment in the whole cohort was 94%; CR 45.7%; and R 50.6%. Initial response to steroids alone was 91.3% (n = 21/23), rituximab plus high-dose dexamethasone (HDD) 93.3% (n = 14/15); children receiving IVIG alone 100% (n=12/12); and eltrombopag in adults 100% (n = 3/3). Relapse was documented in 19.4% of children and 34% of adults, at a median time of 15 and 2 months, respectively; 30.4% of adults (15.2% from the miscellaneous group, 10.9% SLE-associated, and 4.3% infection-associated) and 18.9% of children followed a chronic course; age ≥10 years and platelets ≥20 × 109/L were risk factors for chronic ITP in children. Conclusion: Evolution was heterogeneous: a better and more sustained response was documented in the infections group compared to SLE or the miscellaneous group. (REV INVEST CLIN. 2021;73(1):31-8)
Assuntos
Humanos , Lactente , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Encaminhamento e Consulta , Doença Crônica , Estudos Retrospectivos , Resultado do Tratamento , HematologiaRESUMO
BACKGROUND: Secondary immune thrombocytopenia (ITP) is a heterogeneous and unpredictable disease associated with various underlying conditions. OBJECTIVE: The objective of the study was to investigate clinical evolution and chronicity predictors in secondary ITP. METHODS: Patients treated at an academic medical center during 2008-2019 were stratified by age as children <16 years and adults <16 years. Responses to steroids, intravenous immunoglobulin G (IVIG), rituximab, and eltrombopag were classified as response (R) and complete response (CR). Risk factors for chronic ITP were determined by multiple regression with uni- and multi-variate analysis. RESULTS: Eighty-three patients were included, 37 children and 46 adults. The most frequent associated conditions were infections 53%, systemic lupus erythematosus (SLE) 24%, thyroid disease 9.6%, and Evans syndrome 3.6%. Response to first-line treatment in the whole cohort was 94%; CR 45.7%; and R 50.6%. Initial response to steroids alone was 91.3% (n = 21/23), rituximab plus high-dose dexamethasone (HDD) 93.3% (n = 14/15); children receiving IVIG alone 100% (n=12/12); and eltrombopag in adults 100% (n = 3/3). Relapse was documented in 19.4% of children and 34% of adults, at a median time of 15 and 2 months, respectively; 30.4% of adults (15.2% from the miscellaneous group, 10.9% SLE-associated, and 4.3% infection-associated) and 18.9% of children followed a chronic course; age ≥10 years and platelets ≥20 × 109/L were risk factors for chronic ITP in children. CONCLUSION: Evolution was heterogeneous: a better and more sustained response was documented in the infections group compared to SLE or the miscellaneous group.
Assuntos
Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Doença Crônica , Hematologia , Humanos , Lactente , Pessoa de Meia-Idade , Encaminhamento e Consulta , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
Primary immune thrombocytopenia (ITP) is an intriguing autoimmune disease characterized by autoantibodies against platelets and megakaryocytes. Clinical outcomes, response to treatment, and chronicity predictors were investigated. Patients with newly diagnosed primary ITP treated at a hematology referral center from 2008 to 2018 with complete medical and recent medication history were stratified by age as children < 16 years and adults > 16 years. Responses to treatment including steroids, splenectomy, rituximab, and eltrombopag were classified as response (R) and complete (CR). Factors for developing chronic ITP were determined by multiple regression with uni- and multivariate analysis. p < 0.05 was considered significant. A total of 175 patients were included, 52 children and 123 adults; women predominated with 57.7%. Response to first-line treatment in the whole cohort was 86.18%, CR 43.42% and R 42.76%. The initial response to steroids alone was 83.9% (n = 52/62), rituximab plus high-dose dexamethasone (HDD) 87.2% (n = 34/39), eltrombopag plus HDD 90.9% (n = 10/11), and children receiving IVIG alone 100% (n = 8/8); 9 children were under clinical observation and achieved spontaneous response; loss of response was documented in 15.21% children and 28.3% adults with a median time of 15.95 and 4.07 months respectively; 37.39% of adults and 30.76% of children progressed to a chronic course. Platelets ≥ 20 × 109/L and age ≥ 6 years were risk factors for chronic ITP in the univariate analysis in the adult and children groups, respectively. Clinical course and treatment outcomes for ITP are considerably heterogeneous. Higher platelet counts at diagnosis in adults and age ≥ 6 years in children were associated with an increased risk of chronicity.
Assuntos
Benzoatos/administração & dosagem , Dexametasona/administração & dosagem , Hidrazinas/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Púrpura Trombocitopênica Idiopática/terapia , Pirazóis/administração & dosagem , Rituximab , Esplenectomia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/sangue , Estudos RetrospectivosRESUMO
BACKGROUND: The impact of HLA-DPB1 compatibility and its role as a transplantation antigen in haploidentical-related hematopoietic stem cell transplant (haplo-R-HSCT) have not been established, and a negative effect on survival has been suggested. OBJECTIVE: The objective of the determine was to study the frequency and clinical effects of incompatibility at the HLA-DPB1 locus in the haplo-R-HSCT setting. METHODS: Clinical records and electronic files of 91 patients with a hematological disease who underwent haplo-HSCT from January 2009 to October 2017 in a university medical center were scrutinized. Overall survival (OS) was estimated by the Kaplan-Meier method; the cumulative incidence of transplant-related mortality (TRM) and relapse rates was determined. Acute graft-versus-host disease was assessed by binary logistic regression. Cox regression model with a 95% confidence interval was used to examine the association between the different variables and their effect on OS. RESULTS: Of the 91 donor-recipient pairs, 24 (26.37%) shared complete DPB1 identity, 60 (65.93%) had a mismatch at one allele, and 7 (7.70%) were mismatched at two alleles. Twenty-four different HLA-DPB1 alleles were found; the most frequent were DPB1*04:01 (34.1%) and DPB1*04:02 (27.5%). Two-year OS, the cumulative incidence of TRM and relapse was 51.3 ± 6.8%, 28 ± 6% and 60 ± 7.8% for all haplo-related transplants, respectively, with no statistical difference between HLA-DPB1 matched and partially matched patients. In Cox regression analysis, no risk factors associated with OS, TRM, or relapses were identified. CONCLUSION: HLA-DPB1 mismatching in the haplo-R-HSCT setting did not influence transplant outcomes and was clinically tolerable. A high degree of homozygosity was found.
Assuntos
Cadeias beta de HLA-DP , Doenças Hematológicas/cirurgia , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante Haploidêntico , Adolescente , Adulto , Criança , Pré-Escolar , Seleção do Doador , Feminino , Doenças Hematológicas/mortalidade , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
ABSTRACT Background: The impact of HLA-DPB1 compatibility and its role as a transplantation antigen in haploidentical-related hematopoietic stem cell transplant (haplo-R-HSCT) have not been established, and a negative effect on survival has been suggested. Objective: The objective of the determine was to study the frequency and clinical effects of incompatibility at the HLA-DPB1 locus in the haplo-R-HSCT setting. Methods: Clinical records and electronic files of 91 patients with a hematological disease who underwent haplo-HSCT from January 2009 to October 2017 in a university medical center were scrutinized. Overall survival (OS) was estimated by the Kaplan-Meier method; the cumulative incidence of transplant-related mortality (TRM) and relapse rates was determined. Acute graft-versus-host disease was assessed by binary logistic regression. Cox regression model with a 95% confidence interval was used to examine the association between the different variables and their effect on OS. Results: Of the 91 donor-recipient pairs, 24 (26.37%) shared complete DPB1 identity, 60 (65.93%) had a mismatch at one allele, and 7 (7.70%) were mismatched at two alleles. Twenty-four different HLA-DPB1 alleles were found; the most frequent were DPB1*04:01 (34.1%) and DPB1*04:02 (27.5%). Two-year OS, the cumulative incidence of TRM and relapse was 51.3 ± 6.8%, 28 ± 6% and 60 ± 7.8% for all haplo-related transplants, respectively, with no statistical difference between HLA-DPB1 matched and partially matched patients. In Cox regression analysis, no risk factors associated with OS, TRM, or relapses were identified. Conclusion: HLA-DPB1 mismatching in the haplo-R-HSCT setting did not influence transplant outcomes and was clinically tolerable. A high degree of homozygosity was found.
Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Transplante de Células-Tronco Hematopoéticas/métodos , Cadeias beta de HLA-DP , Transplante Haploidêntico , Doenças Hematológicas/cirurgia , Taxa de Sobrevida , Estudos Retrospectivos , Resultado do Tratamento , Seleção de Pacientes , Seleção do Doador , Doenças Hematológicas/mortalidadeRESUMO
Background: Lumbar puncture (LP) is a hematology procedure that can require repeated attempts leading to traumatic LP (TLP), which has been related to the central nervous system (CNS) relapse. LP success can depend on the size and anatomy of the patient and the skill of the hematologist. The main objective was to determine the influence of body mass index (BMI) on LP outcomes. Materials and Methods: Adults with lymphoid malignancies requiring LP were included prospectively over one year; hematology residents performed most procedures. A 22-gauge Quincke needle was employed. Comparison between non-traumatic vs. traumatic LPs according to BMI, CNS relapse, and residents' year was performed. Results: Fifty-four patients with a mean age of 31.5±15.57 years were included. Diagnosis was Acute Lymphoblastic Leukemia-B (74%), Acute Lymphoblastic Leukemia-T (13%) and Non-Hodgkin Lymphoma (13%). 227 LPs were performed, 121 (53.3%) successful, 98 (43.2%) traumatic, 11 (11.2%) TLPs were macroscopically detectable and 87 (88%) microscopic; 8 (3.5%) were dry-taps. Median time between punctures was 11 days (1-202). Median BMI was 25 (22.8-39.6). Main indication for LP was prophylactic (74.5%); 39.2% were performed by first-year, 35.2% by second-year, 19.6% by third-year hematology residents. No difference (p = 0.145) for a TLP was found among residents. A BMI ≥30 (p = 0.040), non-palpable intervertebral space (p = 0.001) and more than one attempt (p = 0.001) were significant for TLP. TLP was not associated with CNS relapse (p = 0.962). Conclusion: Obesity predicted a TLP. A traumatic puncture did not increase the risk of CNS relapse at one-year follow-up.
RESUMO
BACKGROUND: Warm autoimmune hemolytic anemia (w-AIHA) is an uncommon disease with heterogeneous response to treatment. Steroids are the standard treatment at diagnosis, whereas rituximab has recently been recommended as the second-line therapy of choice. Our main objective was to document the response to treatment in patients with newly diagnosed w-AIHA, including the effectiveness of low-dose rituximab as frontline treatment and for refractory disease. METHODS: Patients with w-AIHA from 2002 to 2017 were included. Relapse-free survival (RFS), probability of maintained response (MR), and time-to-response were analyzed using the Kaplan-Meier method. Response was classified as complete, partial, and no response. RESULTS: We included 64 adults with w-AIHA (39 women and 25 men). The median age was 37 (16-77) years. Response rates to steroids alone were 76.7%, rituximab plus steroids, 100%; and cyclophosphamide, 80%. RFS with steroids at 6, 36, and 72 months was 86.3%, 65.1%, and 59.7%, respectively. Eighteen patients received rituximab at 100 mg/wk for 4 weeks plus high-dose dexamethasone as first-line therapy, with RFS at 6, 36, and 72 months of 92.3%, 58.7% and 44.1%, respectively. Eight patients refractory to several lines of therapy were treated with low-dose rituximab, and all achieved a response (three complete response and five partial response) at a median 16 days (95% confidence interval, 14.1-17.8), with a 75% probability of MR at 103 months; the mean MR was 81.93±18 months. CONCLUSION: Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.