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A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2.09 mg/dl with preserved diuresis. A female infant, 1045 grams, was delivered by emergency caesarean section. Following the surgery, she experienced diplopia, dysarthria, bradypsychia, and sensory alterations in the lower extremities, necessitating emergency hemodialysis due to pulmonary congestion. Blood smear revealed schistocytes, LDH elevated at 1148 IU/L, while transaminases and liver function remained normal, suggesting thrombotic thrombocytopenic purpura. ADAMTS13 revealed 6% activity with the presence of inhibitor. Mycophenolate and daily plasmapheresis with fresh frozen plasma replacement yielded unsatisfactory response, unaffected by the addition of methylprednisolone pulses and rituximab. Eventually, intravenous cyclophosphamide was introduced, resulting in complete hematological remission and normalization of ADAMTS13, however dialysis-dependence persisted and four years later, right renal cancer prompted bilateral nephrectomy. After a total follow-up of six years, she remained free of neoplastic recurrence and lupus activity, receiving prednisone and hydroxychloroquine. The differential diagnosis of microangiopathic syndrome in a pregnant lupus patient is discussed.

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Introducción: Las manifestaciones clínicas de la infección por SARS-CoV-2 son menos frecuentes y graves en el niño que en el adulto, sin embargo, recientes publicaciones sugieren la posibilidad de un cuadro clínico severo secundario a la infección por este coronavirus, denominado síndrome inflamatorio multisistémico en el niño. Este síndrome tiene un carácter posinfeccioso y su fisiopatología probablemente resulte de una activación anormalmente organizada del sistema inmune, en un contexto genético de predisposición y activada por la peculiar biología del SARS-CoV-2. Objetivo: Describir el primer caso cubano con criterios de síndrome multisistémico asociado a COVID 19. Presentación del caso: Paciente masculino de 2 años de edad, previamente sano, con evidencias clínicas y de laboratorio de anemia microangiopática, hiperinflamación sistémica y disfunción múltiple de órganos y sistemas, asociado con evidencias serológicas de infección previa por SARS-CoV-2. Conclusiones: El correcto abordaje de casos como el notificado en este trabajo, requiere mantener un alto nivel de alerta clínica, con una definición clara de los casos sospechosos, la participación multidisciplinaria y la instauración temprana de una estrategia terapéutica adecuada que resultaría trascendental en la reducción de la extensión del daño de órganos y sistemas, así como incrementar la posibilidad de revertir la disfunción establecida(AU)

Introduction: Clinical manifestations of the infection by SARS-CoV-2 are less frequent and severe in children than in adults; however, recent publications suggest the possibility of a severe clinical scenario secondary to the infection by this coronavirus called multisystem inflammatory syndrome in children (MIS-C). This syndrome has a post-infection nature and its physiopathology is probably the result of an abnormally organized activation of the immune system in a genetic context of predispostion, and actived by the particular biology of SARS-CoV-2. Objective: To describe the first Cuban case with criterion of multisystemic syndrome associated to COVID-19. Case presentation: 2 years old male patient, previously healthy, with clinical and laboratory evidences of microangiopathic anemia, systemic hyperinflammation and organs and systems´ multiple dysfunction, associated with serologic evidences of previous infection by SARS-CoV-2. Conclusions: The proper approach to cases as the above mentioned in this work requires to keep a high level of clinical alert, with a clear definition of suspicious cases, multidisciplinary participation and the early establishment of and adequate therapeutic strategy that will be significant in the reduction of systems and organs damage´s extension; as well as increasing the chance to improve the dysfunction(AU)

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BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease that can be fatal if not treated early. We aimed to describe the clinical characteristics of Mexican patients with idiopathic TTP. STUDY DESIGN AND METHODS: We conducted a retrospective study, including all adult patients diagnosed with idiopathic TTP from 2011 to 2017 in two Mexican centers. We further compared our results with the published literature. RESULTS: Twenty patients were included; 70% were female, with a median age of 38.5 years at diagnosis (range 16-63). The median time from onset of symptoms to hospital admission was 1.5 days (range 0-16). Most patients (85%) presented with at least one systemic manifestation at admission (including fever) and 90% had neurological symptoms, most of them major (70%) including loss of consciousness, transient focal abnormalities, headache, and confusion. Only one patient (5%) had the classical pentad at the time of admission. Kidney failure was present in 25% of patients and hemorrhagic symptoms in 60%. Digestive and cardiorespiratory symptoms were less common (45% and 15%, respectively). Median platelet count and lactate dehydrogenase were 10 500/µL and 1319 IU/L, respectively. Eighty percent of patients achieved remission following treatment. Patients admitted within the first 48 hours (after the onset of symptoms) tended to have better overall survival. CONCLUSION: Clinical presentation in Mexican TTP patients is similar to that in other countries. Early admission and a high suspicion for the disease will avoid delays in the initial work-up and initiation of therapy, further improving prognosis.

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RESUMEN Se describen tres casos de pacientes con lupus y microangiopatía. Los casos descritos representan las principales condiciones asociadas a lupus que pueden generar anemia microangiopática, como son: anemia hemolítica microangiopática, síndrome antifosfolípido con microangiopatía localizada y síndrome antifosfolípido catastrófico. La alta mortalidad que conlleva la anemia microangiopática, sin el tratamiento oportuno, hace necesario motivar a los clínicos a conocer ampliamente la enfermedad para poder reconocerla prontamente.

SUMMARY In this article, we describe three cases of lupic patients that developed microangiopathy. Those cases represent the principal etilogies of microangiopathy in Lupus such as, microangiopathic haemolytic anaemia and antiphospholipid syndrome with and without castastrofic manifestations. The clinicians must know deeply about this condition to recognize it in a timely manner.

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