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1.
Ann Hematol ; 100(3): 645-651, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33515047

RESUMO

Management of adult patients with immune thrombocytopenia (ITP) is often unsatisfactory, due to variable efficacy of treatment, risk of life-threatening bleeding if disease control is poor, and side effects associated with treatment. Lack of data on the platelet count threshold associated with bleeding and infection risk associated with ITP treatment limits risk/benefit clinical decision making. We reviewed medical records of all ITP patients who were admitted to our hospital between 2012 and 2017 to evaluate the platelet count threshold for bleeding, infection burden associated with treatment, and real-world efficacy of second-line treatment. We demonstrated fair discrimination between platelet count and occurrence of bleeding, with 15 × 109/L being the optimal cut-off for predicting any bleeding while 20 × 109/L had the highest negative predictive value for severe bleeding. In multivariable analyses, patients who were treated with corticosteroids for at least 2 months were 5.3 times as likely to have an infection. In addition, rituximab response was strongly associated with response to frontline corticosteroids and infection was associated with older age ≥ 65 years and corticosteroid dependence. If corticosteroids are initiated, physicians should aim for the shortest duration of treatment before switching to effective second-line agents for hemostatic platelet counts.


Assuntos
Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/terapia , Corticosteroides/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Plaquetas/patologia , Estudos de Coortes , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/diagnóstico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/etiologia , Feminino , Seguimentos , Hemorragia/sangue , Hemorragia/diagnóstico , Hemorragia/epidemiologia , Hemorragia/etiologia , Hospitalização/estatística & dados numéricos , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Infecções/epidemiologia , Infecções/etiologia , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Prognóstico , Púrpura Trombocitopênica Idiopática/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Rituximab/uso terapêutico , Singapura/epidemiologia , Esplenectomia/estatística & dados numéricos , Resultado do Tratamento
3.
Ann Hematol ; 99(11): 2513-2520, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32945941

RESUMO

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 Retrospectivos
5.
Int J Infect Dis ; 99: 269-271, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32771636

RESUMO

The novel coronavirus SARS-CoV-2 can cause a severe and even fatal respiratory illness named COVID-19. Apart from respiratory failure, COVID-19 may be associated with various autoimmune complications. We present a case of a critically ill patient with COVID-19 who developed severe immune thrombocytopenia that was successfully treated with a concomitant use of corticosteroids and intravenous immunoglobulins.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Pneumonia Viral/complicações , Púrpura Trombocitopênica Idiopática/etiologia , Estado Terminal , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Transfusão de Plaquetas , Púrpura Trombocitopênica Idiopática/terapia
6.
BMJ Case Rep ; 13(7)2020 Jul 07.
Artigo em Inglês | MEDLINE | ID: mdl-32641442

RESUMO

We report on a patient with coronavirus disease 2019 (COVID-19) and decompensated cirrhosis who experienced a favourable outcome of severe immune thrombocytopaenic purpura (ITP) after administration of intravenous immunoglobulin and high-dose dexamethasone. The present case suggests that it is reasonable to evoke ITP in case of profound thrombocytopaenia in a patient with COVID-19.


Assuntos
Infecções por Coronavirus , Glucocorticoides/administração & dosagem , Imunoglobulinas Intravenosas/administração & dosagem , Cirrose Hepática Alcoólica , Obesidade , Pandemias , Pneumonia Viral , Púrpura Trombocitopênica Idiopática , Adulto , Betacoronavirus/isolamento & purificação , Comorbidade , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/fisiopatologia , Humanos , Hipóxia/diagnóstico , Hipóxia/etiologia , Fatores Imunológicos/administração & dosagem , Cirrose Hepática Alcoólica/diagnóstico , Cirrose Hepática Alcoólica/epidemiologia , Masculino , Obesidade/diagnóstico , Obesidade/epidemiologia , Oxigenoterapia/métodos , Pneumonia Viral/diagnóstico , Pneumonia Viral/epidemiologia , Pneumonia Viral/fisiopatologia , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/etiologia , Púrpura Trombocitopênica Idiopática/fisiopatologia , Púrpura Trombocitopênica Idiopática/terapia , Radiografia Torácica/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
7.
Int J Hematol ; 112(5): 746-750, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32613314

RESUMO

COVID-19 is a new disease with many undescribed clinical manifestations. We report herein a case of severe immune thrombocytopenic purpura (ITP) in a critical COVID-19 patient. A patient presented a severe episode of immune thrombocytopenia (< 10 × 109/L) 20 days after admission for a critical COVID-19. This thrombocytopenia was associated with a life-threatening bleeding. Response to first-line therapies was delayed as it took up to 13 days after initiation of intravenous immunoglobulin and high-dose dexamethasone to observe an increase in platelet count. COVID-19 may be associated with late presenting severe ITP. Such ITP may also be relatively resistant to first-line agents. Hematological manifestations of COVID-19, such as the ones associated with life-threatening bleeding, must be recognized.


Assuntos
Infecções por Coronavirus/complicações , Pandemias , Pneumonia Viral/complicações , Púrpura Trombocitopênica Idiopática/etiologia , Betacoronavirus , Terapia Combinada , Infecções por Coronavirus/tratamento farmacológico , Dexametasona/uso terapêutico , Hemorragia/etiologia , Humanos , Imunoglobulinas Intravenosas , Hemorragias Intracranianas/etiologia , Masculino , Pessoa de Meia-Idade , Pneumonia Estafilocócica/etiologia , Pneumonia Associada à Ventilação Mecânica/etiologia , Atelectasia Pulmonar/etiologia , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Púrpura Trombocitopênica Idiopática/terapia , /terapia
8.
Ann Hematol ; 99(9): 2085-2093, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32710167

RESUMO

Clinical research has resulted in an improvement of treatment options for patients with immune thrombocytopenia (ITP) over the last years. However, only few data exist on the real-life management of patients with ITP. To expand the knowledge, a multicenter, national survey was undertaken in 26 hematology practices distributed all over Germany. All patients with a diagnosis of ITP were documented using questionnaires, irrespective of the diagnosis date over a period of 2 years. Overall, data of 1023 patients were evaluated with 56% of patients being older than 60 years. Seventy-nine percent of the patients had chronic (> 12 months), 16% persistent (> 3-12 months), and 5% newly diagnosed (0-3 months) ITP. In 61% of cases, the disease lasted 3 or more years before survey documentation started. Main strategies applied as first-line therapy consisted of steroids in 45% and a "watch and wait" approach in 41% of patients. During second- and third-line strategies, treatment with steroids decreased (36% and 28%, respectively), while treatment modalities such as TPO-RAs increased (19% and 26%, respectively). As expected, patients with a low platelet count and thus a higher risk for bleeding and mortality received treatment (esp. steroids) more frequently during first line than those with a higher platelet count. Up to a third of patients were treated with steroids for more than a year. Overall, our study provides a cross-section overview about the current therapeutic treatment landscape in German ITP patients. The results will help to improve therapeutic management of ITP patients.


Assuntos
Gerenciamento Clínico , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/terapia , Inquéritos e Questionários , Adolescente , Adulto , Criança , Feminino , Alemanha/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/sangue , Estudos Retrospectivos , Esplenectomia/tendências , Esteroides/uso terapêutico , Adulto Jovem
13.
Br J Haematol ; 189(6): 1038-1043, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32374026

RESUMO

This document aims to provide practical guidance for the assessment and management of patients with thrombocytopenia, with a particular focus on immune thrombocytopenia (ITP), during the COVID-19 pandemic. The intention is to support clinicians and, although recommendations have been provided, it is not a formal guideline. Nor is there sufficient evidence base to conclude that alternative approaches to treatment are incorrect. Instead, it is a consensus written by clinicians with an interest in ITP or coagulation disorders and reviewed by members of the UK ITP forum.


Assuntos
Betacoronavirus , Infecções por Coronavirus , Pandemias , Pneumonia Viral , Púrpura Trombocitopênica Idiopática , Adulto , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/etiologia , Infecções por Coronavirus/terapia , Feminino , Humanos , Masculino , Pneumonia Viral/epidemiologia , Pneumonia Viral/etiologia , Pneumonia Viral/terapia , Guias de Prática Clínica como Assunto , Púrpura Trombocitopênica Idiopática/congênito , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/terapia
15.
Platelets ; 31(4): 461-473, 2020 May 18.
Artigo em Inglês | MEDLINE | ID: mdl-32314933

RESUMO

Despite the publication in 2009 of a paper on 'terms and definitions of immune thrombocytopenia' (ITP), some unresolved issues remain and are reflected by the disagreement in the treatment suggested for primary ITP in adults. Considering that these disagreements could be ascribed to non-shared goals, we generated a 'consensus' on some terms, definitions, and assertions useful for classifying the different lines of treatment for primary ITP in adults according to their indications and goals. Agreement on the appropriateness of the single assertions was obtained by consensus for the following indicators: 1. classification of four 'lines of therapy'; 2. acceptance of the expression 'sequences of disease' for the indications of the respective four lines of treatment; 3I . practicability of splenectomy; 3Ib . acceptance, with only some exceptions, of a 'timing for elective splenectomy of 12 months'; and 4a-d . 'goals of the four lines of therapy.' On the basis of the consensus, a classification of four lines of treatment for primary ITP in adults was produced. In our opinion, this classification, whose validity is not influenced by the recently published new guidelines of the American Society of Hematology (ASH) and reviews, could reduce the disagreement that still exists regarding the treatment of the disease.


Assuntos
Púrpura Trombocitopênica Idiopática/terapia , Esplenectomia , Adulto , Consenso , Objetivos , Humanos , Itália , Púrpura Trombocitopênica Idiopática/cirurgia , Fatores de Risco , Esplenectomia/mortalidade , Esplenectomia/estatística & dados numéricos , Inquéritos e Questionários
19.
Platelets ; 31(3): 315-321, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32054377

RESUMO

Diagnosis of immune thrombocytopenia (ITP) and prediction of response to therapy remain significant and constant challenges in hematology. In patients who present with ITP, the platelet count is frequently used as a surrogate marker for disease severity, and so often determines the need for therapy. Although there is a clear link between thrombocytopenia and hemostasis, a direct correlation between the extent of thrombocytopenia and bleeding symptoms, especially at lower platelet counts is lacking. Thus, bleeding in ITP is heterogeneous, unpredictable, and nearly always based on a multitude of risk factors, beyond the platelet count. The development of an evidence-based, validated risk stratification model for ITP treatment is a major goal in the ITP community and this review discusses new laboratory approaches to evaluate the various pathobiologies of ITP that may inform such a model.


Assuntos
Suscetibilidade a Doenças , Púrpura Trombocitopênica Idiopática/etiologia , Pesquisa/tendências , Animais , Biomarcadores , Plaquetas/imunologia , Plaquetas/metabolismo , Plaquetas/patologia , Suscetibilidade a Doenças/imunologia , Humanos , Sistema Imunitário/imunologia , Sistema Imunitário/metabolismo , Megacariócitos/imunologia , Megacariócitos/metabolismo , Megacariócitos/patologia , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/metabolismo , Púrpura Trombocitopênica Idiopática/terapia
20.
Am J Med ; 133(8): e393-e395, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-31987800
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