Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.594
Filtrar
1.
Paediatr Drugs ; 23(5): 445-455, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34292515

RESUMO

Targeted therapies have emerged as innovative treatments for patients whose disease does not respond to conventional chemotherapy, and their use has widely expanded in the field of pediatric hematologic malignancies in the last decade. While they carry the promise of improved disease control and survival and are currently investigated in first-line treatment protocols for patients with poor prognostic markers, they are associated with a considerable incidence of specific toxicities, including cytokine-release syndrome, neurotoxicity, hepatotoxicity, nephrotoxicity, cardiotoxicity, endocrine adverse events, and infectious complications. Iatrogenic or secondary dysgammaglobulinemia is a main consequence of targeted therapies using monoclonal antibodies and other antibody-derived treatments that target specific antigens on lymphoid cells (blinatumomab, inotuzumab ozogamicin, rituximab), chimeric antigen receptor T cells, tyrosine kinase inhibitors (imatinib, dasatinib, nilotinib) and, to a lesser extent, checkpoint inhibitors (pembrolizumab, nivolumab). This review discusses the diagnosis and incidence of secondary or iatrogenic dysgammaglobulinemia in children treated with targeted therapies for leukemias and lymphomas, and options for monitoring and treatment.


Assuntos
Antineoplásicos Imunológicos , Antineoplásicos , Disgamaglobulinemia , Neoplasias Hematológicas , Leucemia-Linfoma Linfoblástico de Células Precursoras , Anticorpos Monoclonais/uso terapêutico , Antineoplásicos/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Criança , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/tratamento farmacológico , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico
3.
Eur J Haematol ; 106(4): 439-449, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33453130

RESUMO

OBJECTIVES: Secondary antibody deficiency (SAD), associated with severe, recurrent or persistent infections, is common in patients with haematological malignancies (HM), but unifying guidance on immunoglobulin replacement therapy (IgRT) in these patients is lacking. We aimed to develop consensus statements for the use of IgRT in patients with HM. METHODS: A Delphi exercise was employed to test the level of agreement on statements developed by a Task Force based on available data and their clinical experience. In Round 1, an Expert Panel, comprising specialist EU physicians caring for patients with HM, helped to refine the statements. In Round 2, experts rated their agreement with the statements. In Round 3, experts who had scored their agreement as ≤4 were invited to review their agreement based on the overall feedback. RESULTS: Three definitions and 20 statements were formulated and tested for consensus, covering measurement of IgG levels, initiation and discontinuation of IgRT, dosing, and the use of subcutaneous IgG. Consensus (agreement ≥70% on Likert-type scale) was reached for all three definitions and 18 statements. CONCLUSIONS: Recommendations have been developed with the aim of providing guidance for the use of IgRT to prevent severe, recurrent or persistent infections in patients with HM and SAD.


Assuntos
Disgamaglobulinemia/etiologia , Disgamaglobulinemia/terapia , Neoplasias Hematológicas/complicações , Conferências de Consenso como Assunto , Gerenciamento Clínico , Disgamaglobulinemia/diagnóstico , Europa (Continente) , Humanos , Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/uso terapêutico , Guias de Prática Clínica como Assunto , Resultado do Tratamento
5.
Artigo em Inglês | MEDLINE | ID: mdl-32376706

RESUMO

OBJECTIVE: To investigate the immunologic impact of a single cycle of rituximab (RTX) in children and adolescents with immune-mediated disorders, we evaluated B cells and immunoglobulin levels of 20 patients with neuroimmunologic, nephrologic, dermatologic, and rheumatologic disorders treated under recommended guidelines. METHODS: Retrospective study of immunologic changes in children (aged ≤18 years) diagnosed with immune-mediated disorders in which RTX was prescribed between June 2014 and February 2019. Patients were excluded if they had prior diagnosis of malignant disease or primary immunodeficiency. Patients were clinically and immunologically followed up every 3 months. Only patients having received a single cycle of RTX and with a follow-up greater than 12 months were included in the analysis of persistent dysgammaglobulinemia. RESULTS: Twenty children were included. Median age at RTX treatment was 12.8 years (interquartile range [IQR] 6.6-15.5 years). Median follow-up was 12.6 months (IQR 10.2-24 months). Of the 14 patients eligible for persistent dysgammaglobulinemia analysis (3 had received RTX retreatment, 2 had <12 months post-RTX follow-up, and in 1 data for this time point was missing), 2/14 (14%) remained with complete B-cell depletion, and 5/14 (36%) had dysgammaglobulinemia. Patients with dysgammaglobulinemia were younger (7.8 vs 15.6 years, p = 0.072), had more underlying neuroimmunologic diseases (5/5 vs 0/9, p < 0.001), and had received more frequently concentrated doses of RTX (3/5 vs 1/9, p = 0.05) than patients without dysgammaglobulinemia. Kinetics of immunoglobulins in the 20 patients revealed a decrease as early as 3 months after RTX in patients with neuroimmunologic disorders. CONCLUSION: In our cohort, single-cycle RTX-induced dysgammaglobulinemia was enhanced in patients with neuroimmunologic diseases. Further studies are needed to confirm this observation.


Assuntos
Linfócitos B/efeitos dos fármacos , Disgamaglobulinemia/induzido quimicamente , Doenças do Sistema Imunitário/tratamento farmacológico , Fatores Imunológicos/efeitos adversos , Rituximab/efeitos adversos , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Fatores Imunológicos/administração & dosagem , Masculino , Estudos Retrospectivos , Rituximab/administração & dosagem
6.
J Pediatr ; 223: 207-211.e1, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32423680

RESUMO

The genetic investigation of a family presenting with a dominant form of hyper IgM syndrome published in 1963 and 1975 revealed a R190X nonsense mutation in activation-induced cytidine deaminase. This report illustrates the progress made over 6 decades in the characterization of primary immunodeficiencies, from immunochemistry to whole-exome sequencing.


Assuntos
Citidina Desaminase/genética , Disgamaglobulinemia/genética , Previsões , Síndromes de Imunodeficiência/complicações , Mutação , Citidina Desaminase/metabolismo , Análise Mutacional de DNA , Disgamaglobulinemia/complicações , Disgamaglobulinemia/metabolismo , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade
8.
J Child Adolesc Psychopharmacol ; 29(4): 268-275, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30892924

RESUMO

Background: Inflammation and immune dysregulation have been implicated in the pathogenesis of pediatric-onset obsessive-compulsive disorder (OCD) and tic disorders such as Tourette syndrome (TS). Though few replicated studies have identified markers of immune dysfunction in this population, preliminary studies suggest that serum immunoglobulin A (IgA) concentrations may be abnormal in these children with these disorders. Methods: This observational retrospective cohort study, conducted using electronic health records (EHRs), identified 206 children with pediatric-onset OCD and 1024 adults diagnosed with OCD who also had testing for serum levels of IgA. IgA deficiency and serum IgA levels in pediatric OCD were compared with IgA levels from children diagnosed with autism spectrum disorders (ASD; n = 524), tic disorders (n = 157), attention-deficit/hyperactivity disorder (ADHD; n = 534), anxiety disorders (n = 1206), and celiac disease, a condition associated with IgA deficiency (n = 624). Results: Compared with ASD and anxiety disorder cohorts, the pediatric OCD cohort displayed a significantly higher likelihood of IgA deficiency (OR = 1.93; 95% CI = 1.18-3.16, and OR = 1.98; 95% CI = 1.28-3.06, respectively), though no difference was observed between pediatric OCD and TS cohorts. Furthermore, the pediatric OCD cohort displayed similar rates of IgA deficiency and serum IgA levels when compared with the celiac disease cohort. The pediatric OCD cohort also displayed the highest percentage of IgA deficiency (15%,) when compared with TS (14%), celiac disease (14%), ADHD (13%), ASD (8%), and anxiety disorder (8%) cohorts. When segregated by sex, boys with OCD displayed a significantly higher likelihood of IgA deficiency when compared with all comparison cohorts except for celiac disease and tic disorders; no significant difference in IgA deficiency was observed between female cohorts. Pediatric OCD subjects also displayed significantly lower adjusted serum IgA levels than the ASD and anxiety disorder cohorts. Adults with OCD were also significantly less likely than children with OCD to display IgA deficiency (OR = 2.71; 95% CI = 1.71-4.28). When compared with children with celiac disease, no significant difference in IgA levels or rates of IgA deficiency were observed in the pediatric OCD cohort. Conclusions: We provide further evidence of IgA abnormalities in pediatric-onset OCD. These results require further investigation to determine if these abnormalities impact the clinical course of OCD in children.


Assuntos
Disgamaglobulinemia/imunologia , Imunoglobulina A/imunologia , Transtorno Obsessivo-Compulsivo/fisiopatologia , Adolescente , Fatores Etários , Doença Celíaca/imunologia , Criança , Estudos de Coortes , Disgamaglobulinemia/epidemiologia , Feminino , Humanos , Imunoglobulina A/sangue , Masculino , Transtornos Mentais/imunologia , Transtornos Mentais/fisiopatologia , Transtorno Obsessivo-Compulsivo/imunologia , Estudos Retrospectivos , Fatores Sexuais
9.
Rheumatol Int ; 39(6): 1083-1090, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30923955

RESUMO

Rituximab, an anti-CD20 monoclonal antibody causing selective B-cell depletion, is used for various systemic inflammatory and autoimmune diseases (SIADs). Long-term safety data on rituximab are limited. The objectives of this study were to evaluate the long-term safety and tolerability of rituximab treatment for SIADs. A retrospective, single-center observational study including all patients ≥ 16 years treated with rituximab for SIADs was performed. The electronic medical records were reviewed, and data concerning indication and duration of rituximab treatment, prior and concurrent immunosuppressive therapy, and adverse events such as infections requiring hospitalization, dysgammaglobulinemia and end organ damage, were collected. A total of 70 patients were included, with a median treatment duration of 54 months, ranging 30-138 months. The most common indications for rituximab treatment were granulomatosis with polyangiitis (22.9%), primary Sjögren's syndrome (20.0%) and systemic lupus erythematosus (14.3%). Infections and persistent dysgammaglobulinemia were the most common adverse events, occurring in 34.3% and 25.7%, respectively. A total of 64 infections were observed in 24 (34.3%) patients, including 1 case of fatal infection. Seventeen patients performed B-cell quantitation during the first 2 years following discontinuation, of which only four (19.0%) demonstrated B-cell reconstitution. End organ damage occurred in two patients, presenting as pyoderma gangrenosum and interstitial pneumonitis. No opportunistic infections were observed. Three patients died during the observational period, of which one was due to lethal infection. This study presents observational data with long treatment duration. It demonstrates that long-term rituximab treatment is relatively well tolerated, and that no cumulative side effects were observed.


Assuntos
Antirreumáticos/efeitos adversos , Doenças Autoimunes/tratamento farmacológico , Disgamaglobulinemia/induzido quimicamente , Infecções/etiologia , Inflamação/tratamento farmacológico , Rituximab/administração & dosagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimioterapia Combinada , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Hospitalização , Humanos , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/induzido quimicamente , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Pioderma Gangrenoso/induzido quimicamente , Estudos Retrospectivos , Rituximab/uso terapêutico , Síndrome de Sjogren/tratamento farmacológico , Fatores de Tempo , Adulto Jovem
11.
J Assoc Physicians India ; 66(6): 107-108, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31331154

RESUMO

Immunoglobulin G (IgG) deficiency disorder is a form of dysgammaglobulinemia, where proportional levels of immunoglobulin G isotype is reduced as compared to other immunoglobulin isotypes. Common clinical presentations of IgG deficiency disorder are recurrent sinusitis and/or lower respiratory infections. However, IgG deficiency manifesting as recurrent septic shock in absence of upper and lower respiratory infection has never been reported, in our search for literature from world over. With this rare case, we highlight the importance of investigating IgG levels in clinical scenarios of recurrent sepsis with no known or traceable infective focus.


Assuntos
Disgamaglobulinemia , Deficiência de IgG , Infecções Respiratórias , Choque Séptico , Humanos , Imunoglobulina G , Síncope
12.
Lab Med ; 48(4): 314-325, 2017 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-29126302

RESUMO

Measurement of IgG subclass concentrations is a standard laboratory test run as part of a panel to investigate the suspicion of antibody deficiency. The assessment is clinically important when total IgG is within the normal age-specific reference range. The measurement is useful for diagnosis of IgG subclass deficiency, to aid the diagnosis of specific antibody deficiency, as a supporting test for the diagnosis of common variable immunodeficiency, as well as for risk stratification of patients with low IgA. The measurement of IgG subclasses may also help determine a revaccination strategy for patients and support patient management. In certain circumstances, the measurement of IgG subclasses may be used to monitor a patient's humoral immune system. In this review, we discuss the utility of measuring IgG subclass concentrations.


Assuntos
Imunoglobulina G , Síndromes de Imunodeficiência , Imunodeficiência de Variável Comum , Disgamaglobulinemia , Humanos , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Imunoglobulina G/classificação , Síndromes de Imunodeficiência/sangue , Síndromes de Imunodeficiência/classificação , Síndromes de Imunodeficiência/diagnóstico
14.
Int Forum Allergy Rhinol ; 7(1): 30-36, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27552393

RESUMO

BACKGROUND: Patients with primary antibody deficiencies have an increased frequency of sinonasal and pulmonary infections. Immunoglobulin (Ig) replacement is a standard therapy for common variable immunodeficiency (CVID) and other antibody deficiency diseases. Although there is convincing evidence that Ig replacement reduces pulmonary infections, there is little evidence that it reduces sinus infections or abates chronic rhinosinusitis (CRS). This study aims to identify the impact of Ig replacement on CRS in antibody deficiencies. METHODS: A single-center, retrospective chart review of adult patients from 1995 to 2015 was performed. Inclusion criteria were diagnosis of CVID or specific antibody deficiency (SAD), history of CRS requiring medical and/or surgical management within the year prior to presentation, treatment with Ig replacement therapy, and follow-up interval of at least 1 year after initiating Ig replacement. Patients with secondary immune deficiencies were excluded. Thirty-one patients met criteria. Data collected included pretreatment and posttreatment Lund-Mackay scores, and frequency of sinusitis and pulmonary infections requiring rescue antibiotics. Statistical analysis was performed using Wilcoxon signed-rank tests. RESULTS: A significant decline in the Lund-Mackay score was evidenced from pretreatment to posttreatment (p < 0.01). Treatment also resulted in significantly lower rates of sinusitis (p < 0.01) and pulmonary infections (p < 0.01). Additionally, 56% of patients who were on prophylactic antibiotics prior to Ig replacement were able to discontinue their use. CONCLUSION: We present objective evidence showing that Ig replacement therapy has a positive impact on the frequency of sinusitis and confirm its positive impact on pulmonary infections in adult patients with CVID and SAD.


Assuntos
Disgamaglobulinemia/tratamento farmacológico , Imunoglobulinas/uso terapêutico , Rinite/prevenção & controle , Sinusite/prevenção & controle , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Doença Crônica , Disgamaglobulinemia/complicações , Disgamaglobulinemia/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Seios Paranasais/diagnóstico por imagem , Infecções Respiratórias/diagnóstico por imagem , Infecções Respiratórias/tratamento farmacológico , Infecções Respiratórias/etiologia , Infecções Respiratórias/prevenção & controle , Rinite/diagnóstico por imagem , Rinite/tratamento farmacológico , Rinite/etiologia , Sinusite/diagnóstico por imagem , Sinusite/tratamento farmacológico , Sinusite/etiologia , Tomografia Computadorizada por Raios X , Adulto Jovem
15.
J Assoc Physicians India ; 64(7): 91-93, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-27759358

RESUMO

Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. sIgMD may occur as a primary or secondary condition. sIgMD is much more common than primary .Hemophagocytic lymphohistiocytosis (HLH) is also a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes and can be primary or secondary, characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations. We report an adult case of primary sIgMD with absent B lymphoid cells and secondary HLH syndrome who presented with recurrent infections, fever and pancytopenia.


Assuntos
Disgamaglobulinemia/complicações , Imunoglobulina M/deficiência , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico , Adulto , Linfócitos B , Disgamaglobulinemia/sangue , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Masculino
16.
Clin Chest Med ; 37(3): 463-74, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27514593

RESUMO

Lymphocytic interstitial pneumonia (LIP) is a rare lung disease on the spectrum of benign pulmonary lymphoproliferative disorders. LIP is frequently associated with connective tissue diseases or infections. Idiopathic LIP is rare; every attempt must be made to diagnose underlying conditions when LIP is diagnosed. Computed tomography of the chest in patients with LIP may reveal ground-glass opacities, centrilobular and subpleural nodules, and randomly distributed thin-walled cysts. Demonstrating polyclonality with immunohistochemistry is the key to differentiating LIP from lymphoma. The 5-year mortality remains between 33% and 50% and is likely to vary based on the underlying disease process.


Assuntos
Disgamaglobulinemia/imunologia , Infecções por Vírus Epstein-Barr/imunologia , Infecções por HIV/imunologia , Doenças Pulmonares Intersticiais/imunologia , Síndrome de Sjogren/imunologia , Lavagem Broncoalveolar , Comorbidade , Doenças do Tecido Conjuntivo/imunologia , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pneumopatias/diagnóstico , Pneumopatias/imunologia , Pneumopatias/patologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/patologia , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/imunologia , Transtornos Linfoproliferativos/patologia , Pseudolinfoma/diagnóstico , Pseudolinfoma/imunologia , Pseudolinfoma/patologia , Tomografia Computadorizada por Raios X
17.
Allergol Immunopathol (Madr) ; 44(4): 286-91, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27083494

RESUMO

BACKGROUND: Chronic urticaria can be the initial clinical presentation of a number of different diseases. The objective of the present study was to report the associated diseases during a ten-year clinical-laboratory follow-up in patients with an initial diagnosis of chronic spontaneous urticaria (CSU) of unknown cause. METHODS: A prospective, longitudinal cohort study with a ten-year clinical-laboratory follow-up was conducted. Patients with a history of urticarial plaques of over six weeks presenting as the only clinical symptom were selected. Individuals with other clinical conditions, urticaria of known causes or chronic physical urticaria were excluded. The following tests were initially performed: haemogram, urine type I, stool parasite exam and sedimentation rate. The following exams were ordered during follow-up: PPD; urine culture; serology tests; antithyroid and antinuclear antibodies, rheumatoid factor, lupus anticoagulant; thyroid hormones; serum immunoglobulin; paranasal sinus and thorax radiographs; testing for BK and Helicobacter pylori; and prick tests. RESULTS: Infections were diagnosed in 29% of patients (syphilis, parasitosis, H. pylori, urinary infection, tuberculosis, hepatitis B and C); autoimmune diseases in 21% (thyroiditis, rheumatoid arthritis and antiphospholipid antibody syndrome); primary immunodeficiencies in 4% (IgA and IgG2 deficiencies); and chronic myeloid leukaemia in 1%. At ten-years of follow-up, the urticaria diagnosis was CSU of unknown cause in 45% of the cases. CONCLUSION: This ten-year clinical-laboratory follow-up of 100 individuals with chronic urticaria as the initial diagnosis revealed the presence of associated diseases in over half of the cases. The most prevalent diseases were infections and autoimmune diseases besides primary immunodeficiencies and blood diseases.


Assuntos
Doenças Autoimunes/complicações , Doenças Transmissíveis/complicações , Disgamaglobulinemia/complicações , Urticária , Adulto , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/epidemiologia , Doença Crônica , Doenças Transmissíveis/diagnóstico , Doenças Transmissíveis/epidemiologia , Disgamaglobulinemia/diagnóstico , Disgamaglobulinemia/epidemiologia , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Prevalência , Estudos Prospectivos , Testes Cutâneos , Urticária/diagnóstico , Urticária/epidemiologia , Urticária/imunologia
18.
Clin Exp Immunol ; 186(1): 57-63, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27125474

RESUMO

Immunoglobulin (Ig)G4 disease can have apparently 'normal' levels of IgG4 due to antigen excess conditions. IgG4 measurement therefore appears falsely low. UK National External Quality Assurance Scheme (UK NEQAS) data and other reports have suggested that this problem occurred despite pre-existing antigen excess detection steps. To determine the clinical relevance of the problem, we examined the prevalence and characteristics of prozoning in our laboratory and patient cohorts. We establish that the prevalence of raised IgG4 in routine IgG4 analysis is low (< 1%) using one of the two routine methods in use in the United Kingdom. We show that subsequent assay modification appears to have reduced the likelihood of misleading readings. However, the original version of the assay prozoned to low levels (below 0·64 g/l) in 41% of high IgG4 samples in our patients. This may explain the previous reports of low sensitivity of raised IgG4 for IgG4RD, and predictive values should be re-evaluated in this disease using modified prozone-resistant protocols. All laboratories providing IgG4 measurements should verify that their assays are fit for the clinical quality requirement of detection raised IgG4 levels and must verify the upper limit of their reference ranges and freedom from prozoning.


Assuntos
Disgamaglobulinemia/sangue , Imunoglobulina G/sangue , Antígenos/imunologia , Testes Diagnósticos de Rotina/métodos , Testes Diagnósticos de Rotina/normas , Disgamaglobulinemia/diagnóstico , Disgamaglobulinemia/imunologia , Humanos , Imunoglobulina G/imunologia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Reino Unido
19.
Int J Hematol ; 104(1): 85-91, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27040278

RESUMO

Splenectomy remains the preferred treatment for chronic immune thrombocytopenia (ITP) after corticosteroid failure, despite the risks of despite surgical complications and infection. The aim of this study was to assess the efficacy of and tolerance to rituximab through a retrospective analysis of 35 refractory/relapsing ITP patients treated from 2004 to 2013. The median age of subjects was 46 years (14-80). Rituximab was given at a weekly dose of 375 mg/m(2) for 4 weeks. Median time from diagnosis to first infusion was 17 months (1-362) and follow-up was 47 months (2-133). The overall response rates at 1 and 2 years after the first infusion were 47 and 38 %, with complete response rates of 24 and 25 %, respectively. Median duration of response was 38 months (1-123), with 37 % of patients maintaining a durable response (>1 year). Twenty-nine percent of patients had undergone splenectomy. A durable response after rituximab was more frequently observed in patients undergoing second-line therapy than those in third or later (83 versus 35 %, P = 0.01). Forty-four percent of patients experienced mild hypogammaglobulinaemia after rituximab, and no clinical infection occurred. To conclude, rituximab should be considered as an alternative treatment to splenectomy. Its efficacy and safety profile should lead us to choose this medical option therapy before surgery for ITP patients.


Assuntos
Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Rituximab/uso terapêutico , Terapia de Salvação/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Disgamaglobulinemia/induzido quimicamente , Humanos , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/cirurgia , Estudos Retrospectivos , Rituximab/efeitos adversos , Terapia de Salvação/normas , Esplenectomia , Resultado do Tratamento , Adulto Jovem
20.
Int J Mycobacteriol ; 5(1): 106-10, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26928000

RESUMO

Selective immunoglobulin M (SIgM) deficiency is a rare form of dysgammaglobulinemia. Here we are reporting a 31year old man with multiple cervical and testicular abscesses who was investigated and found to have miliary tuberculosis (MTB) with primary SIgM deficiency (Serum IgM: 17.4mg/dL) and was treated aggressively with anti-tuberculous treatment.


Assuntos
Imunoglobulina M/deficiência , Tuberculose Miliar/complicações , Tuberculose Miliar/tratamento farmacológico , Abscesso/microbiologia , Adolescente , Adulto , Idoso , Antituberculosos/uso terapêutico , Criança , Disgamaglobulinemia/classificação , Disgamaglobulinemia/complicações , Disgamaglobulinemia/microbiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testículo/microbiologia , Adulto Jovem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...