RESUMO
BACKGROUND: Cancer-related thrombotic microangiopathy (CR-TMA) is a rare type of Coombs-negative hemolytic anemia, which is caused by malignancy and has a poor prognosis. CASE: A 76-year-old female was referred to our hospital due to Coombs-negative hemolytic anemia, which was causing fatigue and dyspnea on exertion, accompanied by schistocytosis. A bone marrow examination demonstrated bone marrow carcinomatosis, and the tumor cells were morphologically suspected to be signet-ring cell carcinoma cells. As we failed to find the primary tumor site before the patient died, she was diagnosed with CR-TMA due to bone marrow carcinomatosis of unknown primary origin. Thrombotic thrombocytopenic purpura (TTP) was rapidly ruled out based on her PLASMIC score. In addition, immunohistochemical staining of a clot section of the bone marrow and tumor marker data were useful for narrowing down the likely primary tumor site. CONCLUSION: Although CR-TMA is an extremely rare phenomenon, clinicians who suspect CR-TMA should quickly rule out TTP and decide whether to provide appropriate chemotherapy or plan for palliative care.
Assuntos
Anemia Hemolítica , Carcinoma , Coagulação Intravascular Disseminada , Neoplasias Primárias Desconhecidas , Neoplasias Peritoneais , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas , Feminino , Humanos , Idoso , Coagulação Intravascular Disseminada/etiologia , Coagulação Intravascular Disseminada/complicações , Púrpura Trombocitopênica Trombótica/complicações , Púrpura Trombocitopênica Trombótica/diagnóstico , Medula Óssea , Neoplasias Primárias Desconhecidas/complicações , Neoplasias Primárias Desconhecidas/diagnóstico , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/complicações , Anemia Hemolítica/complicaçõesRESUMO
We report on a 3-month old infant male who had a seven-days history of fever and rhinorrhea associated with wheezing prior to his death, during the Covid-19 pandemic. Viral testing for Covid-19 (SARS-CoV-2) was negative but was positive for Coronavirus 229E and RP Human Rhinovirus. The pulmonary histological examination showed diffuse alveolar damage along with thrombotic microangiopathy affecting alveolar capillaries. Also, thrombotic microangiopathy was evident in the heart, lungs, brain, kidneys and liver. Thrombotic microangiopathy is a major pathologic finding in Acute Respiratory Distress Syndrome and in the multiorgan failure. This is the first report that illustrates thrombotic microangiopathy occurring in lung, heart, liver, kidney and brain in Acute Respiratory Distress Syndrome with Coronavirus 229E with Rhinovirus co-infection. The clinical presentation and pathological findings in our case share common features with Covid-19.
Assuntos
Humanos , Masculino , Lactente , Síndrome do Desconforto Respiratório do Recém-Nascido , Rhinovirus , Infecções por Coronavirus/complicações , Coronavírus Relacionado à Síndrome Respiratória Aguda Grave , Microangiopatias Trombóticas/complicações , Autopsia , Evolução Fatal , Coinfecção , Insuficiência de Múltiplos ÓrgãosRESUMO
A vasculopatia livedoide é uma doença rara caracterizada pela oclusão da microvasculatura da derme, originando lesões maculosas que, posteriormente, podem evoluir para úlceras e cicatrizes atróficas. Como um fenômeno vaso-oclusivo, o tratamento geralmente é realizado com antiplaquetários e fibrinolíticos. O presente relato descreve o caso de uma paciente refratária à terapia convencional, que obteve regressão da doença utilizando a rivaroxabana, um fármaco inibidor seletivo do fator Xa. (AU)
Livedoid vasculopathy is a rare disease characterized by occlusion of the dermis microvasculature, leading to spotted lesions that can later develop into ulcers and atrophic scars. As a vaso- occlusive phenomenon, treatment is usually performed with antiplatelet and fibrinolytic agents. The present report describes the case of a female patient refractory to conventional therapy who presented disease remission using rivaroxaban, a selective factor Xa inhibitor drug. (AU)
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Trombose/tratamento farmacológico , Dermatopatias Vasculares/tratamento farmacológico , Microangiopatias Trombóticas/tratamento farmacológico , Rivaroxabana/uso terapêutico , Vasculopatia Livedoide , Parestesia , Pentoxifilina/uso terapêutico , Polineuropatias/diagnóstico , Trombose/complicações , Vasodilatadores/uso terapêutico , Biópsia , Inibidores da Agregação Plaquetária/uso terapêutico , Nifedipino/uso terapêutico , Fibromialgia , Dermatopatias Vasculares/complicações , Dermatopatias Vasculares/diagnóstico , Doenças do Tecido Conjuntivo/complicações , Extremidade Inferior/lesões , Eletromiografia , Microangiopatias Trombóticas/complicações , Inibidores do Fator Xa/uso terapêutico , Pé/patologia , Doenças Diverticulares , Fumantes , Gabapentina/uso terapêutico , Analgésicos/uso terapêuticoRESUMO
ABSTRACT Introduction: Purpura fulminans (PF) is a rapid progressive thrombotic disease in which hemorrhagic infarction of the skin and disseminated intravascular coagulation (DIC) occurs. It can potentially cause acute kidney injury (AKI). However, there is no description in the medical literature of renal histological findings of PF. Case report: A 20-year-old female patient, previously healthy, was admitted to the emergency department (ED) with odynophagia, fever, generalized myalgia and anuria, which evolved with the appearance of purpuric plaques on the face and limbs. She required dialysis on admission. Laboratorial tests showed anemia, leukocytosis, thrombocytopenia, and elevation of lactic dehydrogenase (LDH). The purpuric lesions became bullous with ruptures and then necrotic and erosive, reaching the dermis, subcutaneous tissue and musculature, until bone exposure. There was no improvement with initial antibiotic therapy aimed at the treatment of meningococcemia. Thrombotic microangiopathy (TMA) and PF were then suspected. The patient remained in daily dialysis, requiring plasmapheresis. After sustained improvement of the thrombocytopenia, she underwent renal biopsy, which was not compatible with TMA, characterizing possible PF. A complete recovery of the renal function was achieved and cutaneous sequels were treated with grafts. Conclusion: When thrombotic and hemorrhagic phenomena overlap, obtaining a renal biopsy can be difficult. However, in the presented case, the biopsy allowed the exclusion of AKI caused by TMA, presenting for the first time, histological findings compatible with PF.
RESUMO Introdução: Purpura Fulminans (PF) é uma doença trombótica de rápida progressão, com infarto hemorrágico da pele e coagulação intravascular disseminada (CIVD). É potencialmente causadora de injúria renal aguda (IRA). Porém, não há descrição na literatura médica dos achados histológicos renais causados por PF. Relato de caso: Mulher, 20 anos, previamente hígida, hospitalizada por odinofagia, febre, mialgia generalizada e anúria, evoluiu com aparecimento de placas purpúricas em face e membros. Necessitou de hemodiálise (HD) já na admissão. Exames laboratoriais mostravam anemia, leucocitose, plaquetopenia e elevação de desidrogenase lática. As lesões purpúricas tornaram-se bolhosas com rompimento e progressão para necrose, se aprofundaram, atingindo derme, subcutâneo e musculatura, até a exposição óssea. Não houve melhora com antibioticoterapia inicial voltada para tratamento de meningococemia. Suspeitou-se, então, de microangiopatia trombótica (MAT) e PF. A paciente permaneceu em HD diária e necessitou também de plasmaférese, após melhora sustentada da plaquetopenia, foi submetida à biópsia renal, que não foi compatível com MAT, possivelmente caracterizando PF. Houve recuperação completa da função renal e as sequelas cutâneas foram tratadas com enxerto. Conclusão: Em casos nos quais os fenômenos trombóticos e hemorrágicos se sobrepõem, a obtenção da biópsia renal se torna difícil. Neste caso, a biópsia permitiu excluir IRA causada por MAT e mostrar, pela primeira vez, achados compatíveis com PF.
Assuntos
Humanos , Feminino , Adulto Jovem , Púrpura Fulminante/complicações , Púrpura Fulminante/diagnóstico , Microangiopatias Trombóticas/complicações , Microangiopatias Trombóticas/diagnóstico , Injúria Renal Aguda/complicações , Injúria Renal Aguda/patologia , Rim/patologia , Biópsia , Diálise Renal , Plasmaferese , Transplante de Pele , Resultado do Tratamento , Injúria Renal Aguda/terapia , Tempo de InternaçãoRESUMO
Celiac disease may be associated with other autoimmune diseases and exceptionally with glomerulopathies and nephrotic syndrome. Associations have been reported with IgA nephropathy, membranoproliferative glomerulonephritis, membranous glomerulopathy and minimal change disease. We report a 63-year-old woman who simultaneously presented with massive nephrotic syndrome (proteinuria 46 g/day) and cachexia due to a malabsorption syndrome secondary to celiac disease. The course of her diseases was complicated with cardiomyopathy due to severe malnutrition, septic shock, acute kidney injury that required dialysis for seven weeks and severe hypertension. A renal biopsy showed a membranoproliferative pattern of injury secondary to a thrombotic microangiopathy and diffusepodocyte damage. Fouryears later, the patient was in good general health, the glomerular filtration rate was 30 ml/min/1.73m² and there was non-nephrotic proteinuria.