Clinical characteristics and risk factors of pericardial effusion after hematopoietic stem cell transplantation in children with thalassemia major / 中华儿科杂志

Objective: To investigate the characteristics, risk factors and outcomes of thalassemia major (TM) children with pericardial effusion (PE) after allo-geneic hematopoietic stem cell transplantation (allo-HSCT). Methods: Clinical data of 446 TM children received allo-HSCT at Shenzhen Children's Hospital between January 2012 and December 2020 were analyzed retrospectively. Patients were divided into PE and non-PE group according to the occurrence of PE. Chi-square tests were used to investigate the risk factors that were associated with the development of PE. Kaplan-Meier method was used for survival analysis of the 2 groups. Results: Twenty-five out of 446 patients (5.6%) developed PE at a time of 75.0 (66.5, 112.5) days after allo-HSCT. Among these patients, 22 cases (88.0%) had PE within 6 months after allo-HSCT and 19 patients (76.0%) had PE within 100 days after allo-HSCT. The diagnoses of PE were confirmed using echocardiography. Pericardial tamponade was observed in only 1 patient, who later undergone emergency pericardiocentesis. The rest of patients received conservative managements alone. PE disappeared in all patients after treatment. Risk factors that were associated with the development of PE after allo-HSCT included the gender of patients, the type of transplantation, the number of mononuclear cells (MNC) infuse, pulmonary infection after HSCT and transplantation associated thrombotic microangiopathy (TA-TMA) (χ²=3.99, 10.20, 14.18, 36.24, 15.03, all P<0.05). In 239 patients that received haploidentical HSCT, the development of PE was associated with the gender of patients, pulmonary infection after HSCT and TA-TMA (χ²=4.48, 20.89, 12.70, all P<0.05). The overall survival rates of PE and non-PE groups were 96.0% (24/25) and 98.6% (415/421). The development of PE was not associated with the overall survival of TM children after allo-HSCT (χ²=1.73, P=0.188). Conclusions: PE mainly develop within 100 days after allo-HSCT in pediatric TM recipients. Haploidentical grafts, female gender, pulmonary infection after HSCT and TA-TMA are the main risk factors associated with PE development after transplant. However, the presence of PE don't have a significant impact on the outcomes of pediatric TM patients after allo-HSCT.

Coronavirus 229E with Rhinovirus co-infection causing severe acute respiratory distress syndrome with thrombotic microangiopathy and death during Covid-19 pandemic: lessons to be learnt

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.

Renal histological findings in a patient with acute renal injury associated with purpura fulminans: a case report / Achados histológicos renais em paciente com injúria renal aguda associada à purpura fulminans: relato de caso

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.

Vasculopatia livedoide tratada com rivaroxabana / Livedoid vasculopathy treated with rivaroxaban

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)

Síndrome nefrótico e injuria renal aguda con microangiopatía trombótica en mujer con enfermedad celíaca: Caso clínico / Nephrotic syndrome and acute kidney injury associated with celiac disease: Report of one case

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.