Catastrophic antiphospholipid syndrome after pregnancy complicated by hemolysis, elevated liver enzymes and low platelets syndrome.

Background: Antiphospholipid syndrome (APLS) is rarely complicated by catastrophic antiphospholipid syndrome (CAPS). Peripartum CAPS is rarer still and can masquerade as other obstetric disorders. A high degree of suspicion is critical for early diagnosis and specific management given the significant morbidity and mortality associated with this disorder. Case: We report a case of a 27-year-old at 22 week's gestation with a history of APLS found to have severe hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome, resulting in termination of pregnancy. Further workup revealed the diagnosis of CAPS followed by prompt treatment with triple therapy leading to clinical improvement. Conclusion: CAPS should be considered within the differential in an obstetric patient with a history of APLS who has evidence of multiorgan involvement with macro- or microvascular thrombosis. Although this may mimic alternative disorders, prompt diagnosis is imperative for appropriate therapy and reduction in maternal morbidity and mortality.

[Up-to-date on the HELLP syndrome (Hemolysis, Elevated Liver enzymes and Low Platelets)]. / Actualité sur le syndrome HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets).

HELLP syndrome is an acronym for Hemolysis, Elevated Liver enzymes and Low Platelets. It is generally considered in the literature as a particular clinical form of pre-eclampsia, a severe complication of the second half of pregnancy. However, this syndrome can occur in isolation in the absence of pre-eclampsia symptoms. Its pathophysiology remains still unclear. The clinical picture is often incomplete and fruste at first. To date, its diagnosis and management is still the subject of much controversy. Associated or not with a vascular and renal manifestations, the HELLP syndrome is a high-risk maternal disorder. The objective of this article is to review the pathophysiological and clinical data and current treatment.

Síndrome de Hellp: Revisión / Hellp Syndrome: Review

Se presenta una revisión sobre el síndrome HELLP con mayor énfasis en las nuevas teorías que pretenden explicar la fisiopatología y unas recomendaciones para su manejo. Este síndrome se caracteriza por daño endotelial microvascular; sin embargo, su fisiopatología aún no está bien definida. Existe un creciente interés en las citoquinas inflamatorias, factores genéticos y ambientales en la patogénesis de la misma. Al considerarla una microangiopatía difusa del embarazo se presta atención a los factores angiogénicos, y podría ser explicada por el desbalance de los factores angiogénicos circulantes, caracterizado por altos niveles circulantes de tirosincinasa tipo fms 1 soluble, endoglina y otros factores antiangiogénicos, bajos niveles circulantes de factor de crecimiento placentario y factor de crecimiento endotelial vascular. El impacto sistémico es de tal magnitud que los niveles séricos de proteínas de choque térmico son significativamente superiores en los pacientes con el síndrome HELLP. Para el diagnóstico es preciso la presentación clínica y estudios que demuestren la alteración de los parámetros que lo caracterizan. Se presentan alternativas para los exámenes cotidianos, como la baja concentración de haptoglobina, que puede ser usada para el diagnóstico de hemólisis y es el marcador de preferencia para la misma. La glutatión S-transferasa-a1 plasmático podría ser un indicador más sensible que las transaminasas para diagnóstico temprano de daño hepático. Finalmente se presentan recomendaciones de manejo clínico de la enfermedad, que incluye la necesidad de manejo interdisciplinario en una unidad de cuidados intensivos.

A HELLP syndrome review is presented, with greater emphasis on new theories that seek to explain the pathophysiology and management recommendations. The syndrome is characterized by microvascular endothelial damage. However, the pathophysiology of the syndrome is not yet well defined. There is a growing interest in inflammatory cytokines, genetic and environmental factors in the pathogenesis of the same. To consider it a diffuse Microangiopathy pregnancy pays attention to the angiogenic factors and could be explained by the imbalance of the circulating angiogénics, characterized by high circulating levels of tirosincinasa factors type 1 soluble fins and endoglin and other angiogenic factors and lower circulating levels of placental growth factor and vascular endothelial growth factor. The systemic impact is of such magnitude that serum levels of heat shock proteins are significantly higher in patients with the HELLP syndrome. The diagnosis necessitates the clinical presentation and studies showing the alteration of the parameters that characterize it. Alternatives for everyday exams as the low concentration of which can be used for the diagnosis of hemolysis and is the preference for the same marker haptoglobin are presented. Glutathione S-transferase-al plasma could be a more sensitive transaminases to diagnosis early liver damage. Finally, recommendations are presented for clinical management of the disease, including the need for interdisciplinary management in an intensive care unit.