ABSTRACT
Púrpura trombocitopênica trombótica (PTT) é uma alteração hematológica rara e com risco de morte, caracterizada por trombocitopenia, anemia hemolítica microangiopática e alterações neurológicas e/ou renais. A PTT foi descrita em raros pacientes com lúpus eritematoso sistêmico juvenil (LESJ) e, até onde se sabe, a prevalência dessa manifestação em uma população de lúpus pediátrico ainda não foi estudada. Assim, entre janeiro de 1983 e dezembro de 2010, revisamos os prontuários de 5.508 pacientes acompanhados na Unidade de Reumatologia Pediátrica do nosso hospital universitário. Foram identificados 279 (5,1%) casos de LESJ que preencheram os critérios de classificação do American College of Rheumatology. Dois destes (0,7%) apresentavam PTT, ambos no início do LESJ, e foram aqui descritos. Os dois pacientes tinham febre, anemia hemolítica microangiopática (com esquizócitos no sangue periférico) e trombocitopenia. O paciente do gênero masculino apresentava hemiparesia e proteinúria, e a paciente do gênero feminino tinha cefaleia persistente e hematúria. Ambos foram tratados com metilprednisolona endovenosa e plasmaferese quando do diagnóstico de PPT. Após tratamento, não houve recidiva da PTT, e hematócritos, contagens de plaquetas e níveis de desidrogenase lática permaneceram normais. Em conclusão, a PTT é uma rara e grave manifestação no início do LESJ. Os casos relatados reforçam a importância de um diagnóstico precoce e de uma terapia agressiva em pacientes com PTT, devido à sua alta morbidade.
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening hematological abnormality characterized by thrombocytopenia and microangiopathic hemolytic anemia, with neurological abnormalities and/or renal disease. TTP has been rarely reported in juvenile systemic lupus erythematosus (JSLE) patients and, to our knowledge, its prevalence in a paediatric lupus population has not been studied. Therefore, from January 1983 to December 2010, we reviewed the charts of 5,508 patients followed-up at the Paediatric Rheumatology Unit of our university hospital. We identified 279 (5.1%) JSLE cases that met the American College of Rheumatology classification criteria. Two (0.7%) of them had TTP, both at JSLE onset, and were described herein. Both patients had fever, microangiopathic hemolytic anemia (with schistocytes in blood smears), and thrombocytopenia. The male patient had hemiparesis and proteinuria and the female patient had persistent headache and hematuria. Both were treated with intravenous methylprednisolone and courses of plasma exchange therapy at TTP diagnosis. After treatment, TTP did not recur and their hematocrit, platelet count, and lactic dehydrogenase remained normal. In conclusion, TTP is a rare and severe manifestation at JSLE onset. The case reports reinforce the importance of early diagnosis and early aggressive treatment for patients with TTP, due to its high morbidity.
Subject(s)
Child , Female , Humans , Male , Lupus Erythematosus, Systemic/complications , Purpura, Thrombotic Thrombocytopenic/etiology , Lupus Erythematosus, Systemic/diagnosisABSTRACT
Thrombotic microangiopathies encompass a group of disorders characterized by microangiopathic he-molytic anemia, thrombocytopenia associated with hyaline thrombi [comprised primarily of platelet aggregates in the microcirculation], and varying degrees of end-organ failure. Many primary [genetic] and secondary etiological predisposing factors have been describednamely pregnancy, autoimmune disorders, cancer, drugs and antineoplastic therapy, bone marrow transplantation/solid organ transplantation, and infections. In the setting of infectious diseases, the association with Shiga or Shiga-like exotoxin of Escherichia coli 0157:H7 or Shigella dysenteriae type 1-induced typical hemolytic uremic syndrome is well known. Recently however, an increasing body of evidence suggests that viruses may also play an important role as trigger factors in the pathogenesis of thrombotic microangiopathies. This is a comprehensive review focusing on the current understanding of viral associated/induced endothelial stimulation and damage that ultimately leads to the development of this life-threatening multisystemic disorder
Subject(s)
Humans , Thrombotic Microangiopathies/pathology , Purpura, Thrombotic Thrombocytopenic/etiology , Hemolytic-Uremic Syndrome/etiology , Hemolytic-Uremic Syndrome/virology , Purpura, Thrombotic Thrombocytopenic/virologyABSTRACT
Se revisa la presentación de 6 pacientes adultos con púrpura trombótica trombocitopénica (PTT), que fueron atendidos en el Servicio de Clínica Médica del Hospital Escuela de Corrientes durante un período de 11 años. La púrpura trombótica trombocitopénica es una anemia hemolítica microangiopática caracterizada por una pentada clínica típica: trombocitopenia, anemia hemolítica microangiopática, fallo renal, fiebre y manifestaciones neurológicas. Todos los pacientes presentaron trombocitopenia y anemia hemolítica microangiopática. El compromiso neurológico fue la segunda característica más frecuente. Todos los pacientes fueron tratados con plasmaféresis. Tres pacientes se recuperaron completamente y 3 fallecieron. El pilar del tratamiento es la transfusión de plasma y la plasmaféresis. Se hace hincapié en su implementación inmediata dada la alta mortalidad de esta patología.
Subject(s)
Humans , Male , Adolescent , Adult , Female , Middle Aged , Purpura, Thrombotic Thrombocytopenic/therapy , Disintegrins , Metalloendopeptidases , Plasmapheresis/adverse effects , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/prevention & control , Purpura, Thrombotic Thrombocytopenic/drug therapyABSTRACT
La disfunción ADAMTS13 ha sido implicada en la patogénesis de la púrpura trombocitopénica trombótica (PTT). Este desorden ocurre con mayor frecuencia en mujeres y, en el 13 por ciento de ellas, se asocia al embarazo. Sin embargo, hay poca información sobre el comportamiento de la proteasa en embarazo normal. Estudiamos el factor de de von Willebrand y la actividad ADAMTS13 en mujeres sanas no embarazadas, embarazadas y puerperio. Se incluyeron a cincuenta y cinco mujeres no embarazadas, donantes normales del banco de sangre, que no tomaban píldoras anticonceptivas, como controles. Se incluyeron 270 embarazadas y puérperas normales. La actividad ADAMTS13 disminuyó progresivamente a partir del período de 12-16 semanas hasta el final del puerperio temprano (media el 52 por ciento, rango 22-89, p < 0,0001), con un aumento leve posterior. Los niveles fueron levemente menores en nulíparas que en mujeres con paridad previa (65 por ciento vs 83 por ciento, p = 0,0003), y primíparas que en multiparas entre 6-11 semanas hasta 17-23 semanas del embarazo (69 por ciento vs. 80 por ciento, p = 0,005). Aunque en todas las mujeres los níveles de proteasa fueron iguales en los diferentes grupos sanguíneos, las mujeres no embarazadas del grupo sanguíneo O demostraron una media mayor de actividad de ADAMTS13 que el no-O (78 por ciento vs 69 por ciento, p = 0,064). Nuestros resultados sugieren que los cambios de actividad de ADAMTS13, durante el embarazo y puerperio, podrían hacer que el final del embarazo fuera un período más vulnerable para el desarrollo de microangiopatía trombótica.
Subject(s)
Humans , Adult , Female , Pregnancy , ADAM Proteins , Metalloendopeptidases/blood , Cohort Studies , Cross-Sectional Studies , von Willebrand Factor/biosynthesis , Immunoelectrophoresis , Platelet Count , Postpartum Period , Pregnancy Trimester, First , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/pathology , Reference Values , Time FactorsABSTRACT
A 17 years old female diagnosed with acute myeloid leukemia (AML)-M2 received an allogeneic haematopoietic stem cell transplant (HSCT) and was given graft versus host disease (GVHD) prophylaxis with methotrexate, cyclosporin-A (CsA) and methyl prednisolone. On day +42 post-transplant, she was diagnosed to have thrombotic thrombocytopenic purpura (TTP). Therapeutic plasma exchange (TPE) (40 ml/kg body mass) using fresh frozen plasma was performed on 8 consecutive days. The renal function, LDH levels, platelet count and peripheral smear findings improved but the neurological symptoms persisted even after TPE. Few reports are available in literature on the effectiveness of therapeutic plasma exchange (TPE) in post-bone marrow transplant (BMT) TTP. The good hematologic response achieved in this patient suggests that TPE could be life-saving and should be tried in every patient with post-BMT TTP.
Subject(s)
Acute Disease , Adolescent , Female , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Leukemia, Myeloid/surgery , Plasma Exchange , Purpura, Thrombotic Thrombocytopenic/etiologyABSTRACT
Os autores relatam um caso infreqüente de púrpura trombocitopênica trombótica associada à infecção urinária por Escherichia coli e apresentam uma revisão bibliográfica sobre o assunto. A púrpura trombocitopênica trombótica é uma complicação incomum da infecção urinária por Escherichia coli. Caracteriza-se por anemia hemolítica microangiopática, trombocitopenia, sintomas neurológicos, insuficiência renal e febre. Entretanto, somente 40% dos casos apresentam todos esses sinais/sintomas. Sua evolução pode ser fatal se não for diagnosticada e tratada precocemente. No caso descrito, somente o tratamento agressivo do quadro séptico, com antibióticos, medidas de suporte e remoção do foco infeccioso (nefrectomia), e o manejo da púrpura trombocitopênica, com imunoglobulina, corticoterapia e plasmaférese associada à reposição de plasma fresco possibilitaram a recuperação da paciente (AU)
The authors report a rare case of a patient with thrombotic thrombocytopenic purpura associated with Escherichia coli urinary tract infection and present a review about this subject. Thrombotic thrombocytopenic purpura is an uncommon complication of Escherichia coli urinary tract infection. It is characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, renal failure and fever. However, only 40% of cases do present all of these signs and symptoms. The disease can be fatal if not diagnosed and treated early. In our report, only the aggressive management of the urinary sepsis, with general measures, antibiotics and the removal of the septic focus (with nephrectomy), and the treatment of the thrombotic thrombocytopenic purpura with immunoglobulins, corticosteroids and plasmapheresis with administration of fresh plasma have allowed the patient to recover (AU)
Subject(s)
Humans , Female , Middle Aged , Purpura, Thrombotic Thrombocytopenic/etiology , Urinary Tract Infections/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , Escherichia coli Infections/drug therapy , Kidney/pathology , Anti-Bacterial Agents/therapeutic useABSTRACT
La púrpura trombocitopénica trombótica y el síndrome hemolítico urémico en el adulto son expresiones distintas de una misma enfermedad. Su incidencia es muy baja, aproximadamente uno en un millón de habitantes, pero su mortalidad sin tratamiento adecuado es alta. Las manifestaciones clínicas son heterogéneas y se pueden confundir con las producidas por otras entidades. En este informe se presentan y discuten las características clínicas de nueve pacientes vistos en seis años en un hospital universitario de referencia y se enfatizan los hallazgos que deben hacer sospechar la existencia de la enfermedad.
Subject(s)
Humans , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/physiopathology , Purpura, Thrombotic Thrombocytopenic/drug therapy , Purpura, Thrombotic Thrombocytopenic/rehabilitation , Hemolytic-Uremic Syndrome/diagnosis , Hemolytic-Uremic Syndrome/etiology , Hemolytic-Uremic Syndrome/physiopathology , Hemolytic-Uremic Syndrome/drug therapy , Hemolytic-Uremic Syndrome/rehabilitationABSTRACT
Las manifestaciones hematológicas en la infección por el virus de la inmunodeficiencia humana son floridas, ya que esta patología ha adquirido particular relevancia en los últimos años por el aumento en la frecuencia de su presentación. Se analizan las distintas formas de compromiso hematológico, así como las indicaciones para la realización de estudios invasivos : biopsia de médula ósea y biopsia ganglionar
Subject(s)
Humans , Anemia/etiology , Bone Marrow Diseases/etiology , HIV Infections/complications , Leukopenia/etiology , Thrombocytopenia/etiology , Anemia/physiopathology , Hematologic Diseases/therapy , Purpura, Thrombotic Thrombocytopenic/etiologySubject(s)
Humans , Male , Female , Pregnancy , Adolescent , Adult , Middle Aged , Blood Transfusion , Hemolytic-Uremic Syndrome , Plasma Exchange , Plasmapheresis , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/etiology , Purpura, Thrombotic Thrombocytopenic/physiopathology , Purpura, Thrombotic Thrombocytopenic/history , Purpura, Thrombotic Thrombocytopenic/mortality , Purpura, Thrombotic Thrombocytopenic/therapy , Aspirin/therapeutic use , Phenytoin/therapeutic use , Follow-Up Studies , Glucocorticoids/therapeutic use , Prednisone/therapeutic use , Recurrence/prevention & control , Splenectomy , Survival RateSubject(s)
Humans , Male , Adult , HIV Infections/complications , Purpura, Thrombotic Thrombocytopenic/etiology , Acquired Immunodeficiency Syndrome/complications , HIV Infections/drug therapy , Plasmapheresis/standards , Purpura, Thrombotic Thrombocytopenic/drug therapy , Purpura, Thrombotic Thrombocytopenic/bloodABSTRACT
We report on a patient with systemic lupus erythematosus, who, during the course of the illness, developed thrombotic thrombocytopenic purpura. In this case, the coexistence of these two conditions was confirmed by laboratory and pathologic findings. The infusion of fresh frozen plasma with plasmapheresis reversed the course of thrombotic thrombocytopenic purpura.
Subject(s)
Adult , Humans , Male , Blood Transfusion , Combined Modality Therapy , Lupus Erythematosus, Systemic/complications , Plasma , Plasmapheresis , Purpura, Thrombotic Thrombocytopenic/etiology , SyndromeSubject(s)
Humans , Female , Adult , Pregnancy , Purpura, Thrombotic Thrombocytopenic/diagnosis , Hemolytic-Uremic Syndrome/diagnosis , Acute Kidney Injury , Eclampsia , Pregnancy Complications , Puerperal Disorders , Purpura, Thrombotic Thrombocytopenic/etiology , Hemolytic-Uremic Syndrome/etiologyABSTRACT
O paciente aqui relatado apresentou um quadro de trombocitopenia, disfunçäo renal, febre, após ser ferida por larvas de borboleta; näo apresentando anormalidades neurológicas, o que caracterizaria a síndrome de Moschcowitz. O quadro de anemia hemolítica com células vermelhas fragmentadas foi positivo, o que nos levou a pensar no diagnóstico de Púrpura Trombocitopênica Trombótica (TTP), mas estudo histológico näo foi realizado. Neste presente caso, relata-se a remissäo sustentada do quadro clínico após transfusöes de sangue fresco e sessöes de hemodiálises, precocemente instituidas