Síndrome de aglutininas frías y púrpura trombocitopénica autoinmune: Un caso inusual de síndrome de Evans / Cold agglutinins syndrome and autoimmune thrombocytopenic purpura: An unusual case of Evans syndrome

El síndrome de Evans es un trastorno poco frecuente en el que se observan trombocitopenia y anemia, ambas de etiología autoinmune; las que pueden ocurrir de manera simultánea o sucesiva. Se presenta un caso poco usual de anemia hemolítica autoinmune por anticuerpos fríos asociada a púrpura trombocitopénica autoinmune. Paciente femenina de 22 años de edad con diagnóstico de púrpura trombocitopénica autoinmune, después de 7 años de evolución y un año en remisión, presentó una anemia hemolítica autoinmune por anticuerpos fríos, refractaria al tratamiento con esteroides y alcaloides de la Vinca, que requirió transfusiones de concentrado de eritrocitos y logró la remisión con la administración de anticuerpo monoclonal anti CD 20. Los restantes estudios de autoinmunidad fueron negativos. Actualmente se mantiene asintomática y sin tratamiento inmunosupresor(AU)

Evans syndrome is a rare disorder in which thrombocytopenia and anemia are observed, both of autoimmune aetiology, which may occur simultaneously or successively. A rare case of cold autoimmune hemolytic anemia associated to autoimmune thrombocytopenic purpura is presented. A 22-year-old female patient with diagnosis of autoimmune thrombocytopenic purpura, after 7 years of evolution and one year in remission, has a cold autoimmune hemolytic anemia, refractory to steroid treatment and vinca alkaloids, which requires transfusions of packed erythrocytes and achieves remission with anti CD 20 monoclonal antibody. The remaining studies of autoimmunity are negative. Currently the patient is asymptomatic and without immunosuppressive therapy(AU)

Lupus eritematoso incompleto con inicio como púrpura trombocitopénica autoinmune, en una lactante de 5 meses de edad / Incomplete lupus erythematosus initially manifesting as autoimmune thrombocytopenic purpura in a five-month old infant

La Púrpura Trombocitopénica Idiopática representa la forma más frecuente de trombocitopenia en la infancia, en ausencia de un trastorno sistémico asociado identificable en la mayoría de los casos. El lupus eritematoso sistémico es una enfermedad de base genética, autoinmune, crónica, multisistémica. El Lupus Eritematoso Incompleto es aquel que se presenta en pacientes que no reúnen criterios de clasificación para lupus eritematoso sistémico de acuerdo al ACR (American College of Rheumatology), junto con Anticuerpos Antinucleares positivos. Se presenta el caso de una lactante menor de 5 meses de edad con diagnóstico de Lupus Eritematoso Incompleto (la menor reportada hasta el momento) que inició su enfermedad con Púrpura Trombocitopénica Autoinmune, además de anticuerpos antinucleares y anticardiolipinas positivos sin afectación en otros sistemas. Por lo que consideramos que la prueba de anticuerpos antinucleares debe solicitarse en casos de púrpura trombocitopénica autoinmune, con el seguimiento adecuado, ya que esta prueba positiva es considerada de riesgo para el desarrollo posterior de lupus eritematoso sistémico, a fin de prevenir daños importantes y mejorar el pronóstico de la enfermedad.

Idiopathic thrombocytopenic purpura is the most common form of thrombocytopenia during childhood, and usually appears without an associated systemic condition being identified. Systemic Lupus Erythematosus is a chronic and multisystemic genetically-based autoimmune disease. Incomplete lupus erythematosus is considered to be present when the patient does not mean the criteria for systemic lupus erythematosus devised by the American College of Rheumatology (ACR) together with positive anti-nuclear antibody titer levels. We present the case of a five-month old female infant diagnosed with incomplete lupus erythematosus (as of current data) whose condition first manifested as autoimmune thrombocytopenic purpura and whose antinuclear and anticardiolipin antibodies titers were positive, but without detected effects on other systems. Since this test is considered to determine the existence of risk of later development of systemic lupus erythematosus, we suggest that antinuclear antibody testing should be requested in cases of autoimmune thrombocytopenic purpura and that adequate follow up be carried out to help avoid negative outcomes and improve patient prognosis.

Púrpura trombocitopénica autoinmune. Informe de 108 casos

Introducción. La púrpura trombocitopénica autoinmune (PTA) es un padecimiento hemorragíparo de presentación cada vez más frecuente en la infancia. Se puede manifestar con sangrados a todos los niveles. Se divide en 2 grandes grupos: púrpura trombocitopénica aguda (PTA-A) y la crónica (PTC-A). Objetivos: identificar las formas de presentación, evolución clínica y modalidades terapéuticas empleadas en pacientes con PTA-A. Material y métodos. Se realizó un estudio retrospectivo en el Servicio de Medicina Interna del Hospital Infantil del Estado de Sonora, reuniendo 108 egresos con diagnóstico de PTA en los últimos 10 años. Las variables analizadas fueron: edad, sexo, época del año, cuadro clínico, complicaciones, tratamiento y evolución. Resultados. El padecimiento fue más frecuente en niños menores de 6 años de edad, no hubo diferencias significativas en cuanto al género; los síntomas más frecuentes fueron: hemorragias en los tejidos cutáneos y en mucosas; la mayoría tuvo tratamiento con prednisona (89%); una minoría recibió gammaglobulina y danazol (4.5%); se les realizó esplenectomía a 11 pacientes (14.5%), sin encontrar en este grupo mortalidad por hemorragia del sistema nervioso central; 85% remitieron y 15% evolucionaron a la cronicidad. No se realizó esplenectomía de urgencia a ningún paciente y de aquellos con PTC-A esplenectomizados la remisión ocurrió en 91%. Conclusiones. La PTA-A puede presentar remisión, aún sin tratamiento; sin embargo, se observaron mejores resultados con incremento plaquetario y de forma más rápida con el uso de esteroides, reduciéndose el tiempo de hospitalización. En esta serie no se presentaron complicaciones graves.

Introduction. Autoimmmune thrombocytopenic purpura (ATP) is a hemorrhagic disease with a more frequent presentation in infancy. Could appear with an all level of bleeding and sometimes involves the central nervous system (CNS). It is divided in 2 groups: the acute thrombocytopenic purpura (A-ATP) and the chronic form (C-ATP). Objectives. To identify the presentation, evaluation and therapeutic modalities applied to patients with A-ATP. Material and methods. We realized a retrospective study in the Internal Medicine. Unit at the Children's Hospital in the State of Sonora, detecting 108 patients with the diagnosis of ATP in the last 10 years. The variables studied were: age gender, clinical features, complications, treatment and evolution. Results. We observed that the disease was more frequent in children under 6 years of age; there were no significant differences regarding gender, and the most common symptoms were bleeding of mucocutaneous tissues: 89% of the patients received treatment with prednisone; gammaglobulin and danazol in 4.5% and splenectomy 11 cases (14.5%); no evidence of mortality due to CNS hemorrhage; 85% presented remission and 15% went to chronicity. Urgent splenectomy was not required in our group, but these who underwent this type of surgery were cured in 91%. Conclusions. Being ATP an autoimmune disease the majority of patients may go under remission even without treatment; however, we have observed better results in the use of steroids, shortening their hospital stay; in this series we did not had any of the most severe complications of ATP.

Postsplenectomy Recurrence of Thrombocytopenia with an Accessory Spleen

Autoimmune thrombocytopenic purpura (AITP) is an autoimmune disorder that results from antiplatelet autoantibodies; these autoantibodies cause platelet destruction in the reticluoendothelial system. Oral corticosteroid therapy is the first line treatment. Splenectomy is the major treatment modality after the failure of more conservative medical therapy. Approximately 15% of the patients will relapse either soon after splenectomy or, as is less common, many years later. The presence of an accessory spleen should be sought. We experienced a patient with a known diagnosis of autoimmune thrombocytopenic purpura who had a worsening thrombocytopenia 11 years after splenectomy. This patient was diagnosed with an accessory spleen. Accessory splenectomy was performed with only a transient elevation of the platelets. We report here on this case with a review of the literature.

A Case of Membranous Glomerulonephropathy Associated with Idiopathic Autoimmune Thrombocytopenic Purpura / 대한신장학회잡지

Membranous glomerulonephropathy is the most common cause of nephrotic syndrome in adults and idiopathic autoimmune thrombocytopenic purpura is autoimmune disease caused by autoantibody to platelet membrane glycoprotein. Although there are some pathologic similarity between two diseases that 'membrane attack complex' play a role in pathologic process, but only 3 cases worldwide are reported about membranous glomerulonephropathy associated with idiopathic autoimmune thrombocytopenic purpura. So we report a case of sixty eight years of woman who had symptoms of generalized edema, foamy urine, anorexia and thrombocytopenia on admission and developed more severe symptomatic thrombocytopenia there after. She was diagnosed membranous glomeulonephropathy on renal biopsy and also diagnosed idiopathic autoimmune thrombocytopenic purpra on bone marrow biopsy and on the basis of exclusion.