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Abstract(s)
Introdução: A Trombocitopenia Imune Primária é uma doença auto-imune, que causa destruição
plaquetar periférica. A citopenia é isolada, cursando com discrasias hemorrágicas mucocutâneas,
embora muitos doentes sejam assintomáticos. O diagnóstico é de exclusão e não obriga ao
tratamento. Atualmente, existem guidelines internacionais que definem critérios diagnósticos
e terapêuticos, contribuindo para a qualidade, eficácia, eficiência e segurança dos cuidados.
Materiais e Métodos: Realizou-se um estudo retrospetivo observacional com consulta de processos
e recolha de dados clínicos e laboratoriais de adultos com Trombocitopenia Imune Primária
seguidos de 2008 a 2016 no Centro Hospitalar Universitário Cova da Beira. Baseado nas guidelines
das sociedades americana e espanhola de Hematologia, definiram-se critérios diagnósticos
e terapêuticos. Para todos os doentes, o estudo diagnóstico deve incluir: história clínica e
exame físico, hemograma, esfregaço de sangue periférico, estudo da coagulação, da função renal
e hepática, anticorpos antinucleares, serologias virais e ecografia abdominal. O tratamento
deve ser iniciado na presença de discrasias hemorrágicas ativas ou plaquetas inferiores a 20-30
x 109/L. O tratamento de primeira linha consiste em corticoterapia em altas doses, podendo
ser adicionada Imunglobulina humana. Avaliou-se a concordância entre a prática clínica e os
critérios diagnóstico e terapêuticos definidos.
Resultados: A amostra inclui 52 doentes com um rácio Masculino:Feminino 1:1,9 e idade mediana
ao diagnóstico de 55 anos (18-94). 88% dos doentes tiveram uma evolução crónica da doença.
Os exames mais frequentemente em falta foram a ecografia (27%), o Vírus da hepatite C (23%)
e os anticorpos antinucleares (19%). Foram pesquisados os anticorpos antiplaquetários em 28
doentes (54%) que positivaram em 7 (25%). O mielograma foi realizado em 16 casos (31%). 9
doentes (29%) com infeção a Helicobacter pylori erradicada não corrigiram a trombocitopenia.
67% dos doentes foram tratados, dos quais 2 não cumpriram critérios formais. Todos os doentes
mantidos em vigilância não tinham critério para tratamento. As opções terapêuticas foram a
corticoterapia (n=21), a corticoterapia com IgIV (n=11), IgIV (n=1) e anti-RhD (n=1). 96% dos
doentes tratados com corticoides responderam.
Discussão: As recomendações para o diagnóstico foram cumpridas em apenas 56% da amostra
e exames não recomendados são frequentemente efetuados, como a pesquisa de Helicobacter
pylori, anticorpos antiplaquetares e mielograma. Este estudo permitiu evidenciar as dificuldades
da abordagem diagnóstica da PTI no exercício médico, sendo esta doença um desafio clínico
pelo seu diagnóstico de exclusão. A elaboração de um protocolo diagnóstico tipo check-list poderá
ser de fácil aplicação perante a suspeita da doença. No tratamento, as recomendações
internacionais tiveram uma aplicação mais consensual.
Introduction: Primary immune thrombocytopenia is an autoimmune illness that causes peripheral platelet destruction. The cytopenia is isolated and causes mucocutaneus hemorrhage, although it can be asymptomatic. The diagnosis is made by exclusion and does not mean that the patient must be treated. Currently, there are international guidelines that define diagnosis and therapeutic criteria, contributing to the quality, efficacy, efficiency and safety of the care. Material and Methods: A retrospective observational study was performed with patient record´s consultation and gathering of clinical and laboratorial data of patients with primary immune thrombocytopenia followed between 2008 and 2016 in the University Hospital Center of Cova da Beira. Based on the guidelines of the American and Spanish societies of Hematology, diagnostic and therapeutic criteria were defined. For all patients, the diagnostic study should include: clinical history and physical examination, blood count, peripheral blood smear, coagulation study, renal and hepatic function, antinuclear antibodies, viral serologies, and abdominal ultrasound. Treatment should be initiated in the presence of active hemorrhage or platelets less than 20-30 x 109/L. First-line treatment consists of high-dose corticosteroids, and human Imunoglobulin may be added. The clinical practice was compared to the diagnostic and therapeutic criteria as defined above. Results: The sample includes 52 patients, with a Male:Female ratio of 1:1,9 and median diagnostic age of 55 years (18-94). 88% of the patients had a chronic evolution of the disease. The exams more frequently not performed were echography (27%), hepatitis C virus (23%) and antinuclear antibodies (19%). Antiplatelet antibodies were screened in 28 patients (54%) and were positive in 7 (25%). The bone marrow examination was performed in 16 cases (31%). 9 patients (29%) with eradicated Helicobacter pylori infection did not correct the thrombocytopenia. 67% of the patients were treated and only 2 did not fulfill formal criteria. All the patients left in surveillance didn´t have criteria for treatment. The therapeutic option were corticosteroids (n=21), corticosteroids with IgIV (n=11), IgIV (n=1) and anti-RhD (n=1). 96% of the patients treated with corticosteroids responded to the therapy. Discussion: The recommendations for the diagnosis were fulfilled only in 56% of the sample and exams not required for the diagnosis are frequently preformed, like the Helicobacter pylori and antiplatelet antibodies screening and the bone marrow aspiration. This study reveals how challenging is the exclusion diagnosis of primary immune thrombocytopenia in clinical practice. The elaboration of a check-list type diagnostic tool may be easy to apply in the suspicion of the illness. In the treatment the international recommendations had a more consensual application.
Introduction: Primary immune thrombocytopenia is an autoimmune illness that causes peripheral platelet destruction. The cytopenia is isolated and causes mucocutaneus hemorrhage, although it can be asymptomatic. The diagnosis is made by exclusion and does not mean that the patient must be treated. Currently, there are international guidelines that define diagnosis and therapeutic criteria, contributing to the quality, efficacy, efficiency and safety of the care. Material and Methods: A retrospective observational study was performed with patient record´s consultation and gathering of clinical and laboratorial data of patients with primary immune thrombocytopenia followed between 2008 and 2016 in the University Hospital Center of Cova da Beira. Based on the guidelines of the American and Spanish societies of Hematology, diagnostic and therapeutic criteria were defined. For all patients, the diagnostic study should include: clinical history and physical examination, blood count, peripheral blood smear, coagulation study, renal and hepatic function, antinuclear antibodies, viral serologies, and abdominal ultrasound. Treatment should be initiated in the presence of active hemorrhage or platelets less than 20-30 x 109/L. First-line treatment consists of high-dose corticosteroids, and human Imunoglobulin may be added. The clinical practice was compared to the diagnostic and therapeutic criteria as defined above. Results: The sample includes 52 patients, with a Male:Female ratio of 1:1,9 and median diagnostic age of 55 years (18-94). 88% of the patients had a chronic evolution of the disease. The exams more frequently not performed were echography (27%), hepatitis C virus (23%) and antinuclear antibodies (19%). Antiplatelet antibodies were screened in 28 patients (54%) and were positive in 7 (25%). The bone marrow examination was performed in 16 cases (31%). 9 patients (29%) with eradicated Helicobacter pylori infection did not correct the thrombocytopenia. 67% of the patients were treated and only 2 did not fulfill formal criteria. All the patients left in surveillance didn´t have criteria for treatment. The therapeutic option were corticosteroids (n=21), corticosteroids with IgIV (n=11), IgIV (n=1) and anti-RhD (n=1). 96% of the patients treated with corticosteroids responded to the therapy. Discussion: The recommendations for the diagnosis were fulfilled only in 56% of the sample and exams not required for the diagnosis are frequently preformed, like the Helicobacter pylori and antiplatelet antibodies screening and the bone marrow aspiration. This study reveals how challenging is the exclusion diagnosis of primary immune thrombocytopenia in clinical practice. The elaboration of a check-list type diagnostic tool may be easy to apply in the suspicion of the illness. In the treatment the international recommendations had a more consensual application.
Description
Keywords
Hematologia Imune Plaquetas Primária Trombocitopenia