Características clínicas, de laboratorio y tratamiento en pacientes con púrpura trombocitopénica trombótica.

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Title:	Características clínicas, de laboratorio y tratamiento en pacientes con púrpura trombocitopénica trombótica.
Alternate Title:	Clinical, laboratory and treatment characteristics of patients with thrombotic thrombocytopenic purpura.
Authors:	JESÚS ARIZA-PARRA, EDWIN¹ edwinriza@hotmail.com, JOSÉ ATENCIA-FLÓREZ, CARLOS², CAMILO JARAMILLO-ÁLVAREZ, JUAN², CARDONA-JARAMILLO, MANUELA³, DOMINGO TORRES-HERNÁNDEZ, JOSÉ⁴
Source:	Acta Medica Colombiana. jul-sep2023, Vol. 48 Issue 3, p1-9. 9p.
Abstract (English):	Introduction: thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by non-immune hemolytic anemia, thrombocytopenia and thrombotic microangiopathy. This study describes the clinical, laboratory and treatment characteristics of a series of patients with TTP, comparing them according to the presence or absence of associated illnesses. Materials and methods: a descriptive observational study of patients diagnosed with thrombotic thrombocytopenic purpura at a reference center in Medellín, Colombia, evaluated between 2012 and 2021. Results: a total of 19 patients were collected, with 80% female predominance; the most frequent clinical manifestations were neurological symptoms (73.6%), kidney problems (68.4%), gastrointestinal problems (52.6%) and fever (47.3%). It was associated with systemic lupus erythematosus (SLE) in 47.6% and was idiopathic in 31.5%. The mean hemoglobin was 7.7 gr/dL +/- 1.7, the median platelet count was 12 x 109/L (8-29), and the mean lactate dehydrogenase (LDH) was 1,509 IU/L +/- 862. Altogether, 94.7% were classified as high probability according to the PLASMIC score, ADAMTS13 was measured in 42% and all received plasma exchange therapy. Clinical response was achieved in 78.9%, with refractoriness in 31.5% and 26.3% mortality; the comparison between idiopathic vs. nonidiopathic TTP showed lower kidney involvement (p=0.04) and higher LDH (p=0.02). Conclusion: the clinical presentation of TTP is notable for the predominance of neurological and gastrointestinal symptoms, marked elevation of lactate dehydrogenase and kidney injury, especially in the idiopathic type. We emphasize the need to measure ADAMTS13 activity in all patients prior to beginning plasma exchange or even in the first two sessions and look for SLE-like autoimmune disease. The higher mortality and refractoriness compared with other series presents the potential for improvement in timely diagnosis and availability of all the treatment schemes. [ABSTRACT FROM AUTHOR]
Abstract (Spanish):	Introducción: la púrpura trombocitopénica trombótica (PTT) es una enfermedad infrecuente, que se caracteriza por anemia hemolítica no inmune, trombocitopenia y microangiopatía trombótica. En este estudio se describen las características clínicas, de laboratorio y el tratamiento de una serie de pacientes con PTT comparando según la presencia o ausencia de enfermedad asociada. Material y métodos: estudio observacional descriptivo de pacientes con diagnóstico de púrpura trombocitopénica trombótica en un centro de referencia en Medellín (Colombia), evaluados entre 2012 y 2021. Resultados: se recolectaron 19 pacientes, con predominio de mujeres en 80%; las manifestaciones clínicas más frecuentes fueron síntomas neurológicos (73.6%), afectación renal (68.4%), gastrointestinales (52.6%) y fiebre (47.3%), se asoció a lupus eritematoso sistémico (LES) en 47.6% e idiopático en 31.5%. La media de hemoglobina fue de 7.7 gr/dL ± 1.7, la mediana del recuento de plaquetas de 12 x 109/L (8-29) y una media de lactato deshidrogenasa (LDH) de 1509 UI/L ± 862. Fueron clasificados como alta probabilidad por escala PLASMIC el 94.7%, se midió ADAMTS13 en 42% y todos recibieron terapia con recambio plasmático. La respuesta clínica se logró en 78.9%, con refractariedad en 31.5% y mortalidad 26.3%; en la comparación de PTT idiopática vs no idiopática se documentó una menor frecuencia de afectación renal (p=0.04) y mayor elevación de LDH (p=0.02). Conclusión: en la presentación clínica de PTT se destaca el predominio de los síntomas neurológicos y gastrointestinales, la elevación marcada de lactato deshidrogenasa y la lesión renal en especial en el origen idiopático. Se recalca la necesidad de medir en todos los pacientes la actividad de ADAMTS13, previo al inicio de recambio plasmático o incluso en las primeras dos sesiones y buscar enfermedad autoinmune tipo LES. La mayor mortalidad y refractariedad comparada con otras series plantea la posibilidad de mejoras en el diagnóstico oportuno y la disponibilidad de todos los esquemas terapéuticos. [ABSTRACT FROM AUTHOR]
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Database:	MedicLatina

Description
Abstract:	Introduction: thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by non-immune hemolytic anemia, thrombocytopenia and thrombotic microangiopathy. This study describes the clinical, laboratory and treatment characteristics of a series of patients with TTP, comparing them according to the presence or absence of associated illnesses. Materials and methods: a descriptive observational study of patients diagnosed with thrombotic thrombocytopenic purpura at a reference center in Medellín, Colombia, evaluated between 2012 and 2021. Results: a total of 19 patients were collected, with 80% female predominance; the most frequent clinical manifestations were neurological symptoms (73.6%), kidney problems (68.4%), gastrointestinal problems (52.6%) and fever (47.3%). It was associated with systemic lupus erythematosus (SLE) in 47.6% and was idiopathic in 31.5%. The mean hemoglobin was 7.7 gr/dL +/- 1.7, the median platelet count was 12 x 109/L (8-29), and the mean lactate dehydrogenase (LDH) was 1,509 IU/L +/- 862. Altogether, 94.7% were classified as high probability according to the PLASMIC score, ADAMTS13 was measured in 42% and all received plasma exchange therapy. Clinical response was achieved in 78.9%, with refractoriness in 31.5% and 26.3% mortality; the comparison between idiopathic vs. nonidiopathic TTP showed lower kidney involvement (p=0.04) and higher LDH (p=0.02). Conclusion: the clinical presentation of TTP is notable for the predominance of neurological and gastrointestinal symptoms, marked elevation of lactate dehydrogenase and kidney injury, especially in the idiopathic type. We emphasize the need to measure ADAMTS13 activity in all patients prior to beginning plasma exchange or even in the first two sessions and look for SLE-like autoimmune disease. The higher mortality and refractoriness compared with other series presents the potential for improvement in timely diagnosis and availability of all the treatment schemes. [ABSTRACT FROM AUTHOR]
ISSN:	01202448
DOI:	10.36104/amc.2023.2760