ABSTRACT
Introducao: A Sindrome de embolia gordurosa e uma complicacao rara da doenca falciforme, descrita principalmente na doenca nao homozigotica. Resulta de extensa necrose da medula ossea (MO), durante crise vasoclusiva (CVO), com liberacao de embolos de gordura na circulacao e disfuncao organica multipla. Os criterios diagnosticos sao envolvimento de multiplos/unico orgao histologicamente comprovado por embolia gordurosa e/ou medular necrotica ou desenvolvimento de insuficiencia respiratoria aguda (IRpA) e manifestacoes neurologicas ou falencia de multiplos orgaos com evidencia de necrose medular (laboratorial ou histologica). Objetivos: Descrever caracteristicas clinicas, laboratoriais e de tratamento de 5 pacientes com sindrome de embolia gordurosa atendidos no HCFMUSP entre 10/2021 e 07/2022. Resultados: Os 5 casos eram HbSS (4 mulheres),1 em uso de hidroxiureia (HU), 2 sem HU por hepatotoxicidade e em programa transfusional, 1 interrompeu o tratamento e 1 nunca havia usado. Mediana de idade no evento: 34 (22-52) anos. Fatores desencadeantes provaveis em 3 pacientes: infeccoes por Influenza, Covid19 e S.aureus Oxacilina resistente. A admissao, todos apresentavam dor generalizada e dessaturacao;4 apresentavam confusao mental e rebaixamento do nivel de consciencia com TC de cranio normal;3 com consolidacao pulmonar sendo iniciado antibiotico. Medianas e ranges de exames a admissao: Hb 5,7 (3,6-7,2)g/dL;leucometria 30900 (8620-51600)/mm3, 2 com desvio ate mielocitos, 2 ate metamielocitos e 1 ate bastoes;eritroblastos 38,5 (2,5-53,8) EOC/100 leucocitos;plaquetas 208 (46-507) mil/mm3;DHL 1296 (502->6000)mg/dL;Cr 1,59 (0,94-3,72)mg/dL;BI/BD 2,32 (2,09-4,84)/3,4 (2,57-12,8)mg/dL;TGO 101 (44-289)mg/dL;TGP 19 (18-29)mg/dL;GGT 185 (118-423)mg/dL;FA 369 (142-1060)mg/dL. Durante a internacao, todos evoluiram com reacao leucoeritroblastica (desvio ate mielocitos/promielocitos), aumento de DHL, TGO, TGP, GGT, FA, BI, BD (predominio de BD) e lesao renal aguda, 3 evoluiram com plaquetopenia e 2 com reticulocitopenia. Todos receberam concentrado de hemacias nas primeiras 24h e durante a internacao (mediana 13;range 2-19), 2 iniciaram hemodialise e 2 foram intubados e receberam drogas vasoativas (DVA). Nenhum desenvolveu CIVD. A biopsia de MO de 1 paciente mostrou tecido hematopoietico difusamente necrotico de padrao isquemico. A mediana de internacao foi 11 dias (range 2-22). 1 paciente faleceu em 48h, 1 foi extubado e teve DVA suspensa apos 17 dias, 4 pacientes receberam alta com Hb proxima ao basal e leucometria, plaquetas e funcao renal normais. Discussao: A sindrome de embolia gordurosa e caracterizada por IRpA e manifestacoes neurologicas, podendo haver comprometimento das funcoes renal e hepatica alem de reacao leucoeritroblastica ou pancitopenia, quadro apresentado por nossos pacientes embora nossos casos destoem da literatura quanto ao genotipo, onde apenas 15% sao HbSS. Suspeitar do diagnostico e fundamental para o desfecho dos casos. Na suspeita, a instituicao rapida de terapia transfusional, para reduzir HbS, e determinante para a sobrevida. Uma revisao sistematica descreveu mortalidade de 29, 61 e 91% para quem recebeu troca, reposicao ou nenhuma transfusao, respectivamente. Conclusao: A falta de suspeita diagnostica dificulta o reconhecimento da sindrome, determinando taxas altas de mortalidade. Familiaridade com o quadro clinico e inicio imediato de terapia transfusional tem se mostrado os unicos indicadores de sobrevida. Copyright © 2022
ABSTRACT
Background: The COVID-19 pandemic had a high burden in Brazil. To date, data on mortality and prognostic factors of COVID-19 infection in Brazilian patients with hematological disorders are scarce. Aims: To describe the characteristics and outcomes of patients with hematological disorders admitted to the hematological COVID care unit of a reference center in Brazil;to analyze the impact of prognostic factors on in-hospital mortality. Methods: This prospective, single-center study,included 118 patients who have been admitted to the hematological COVID care unit of the Hospital das Clínicas da Faculdade de Medicina da USP, S.o Paulo, Brazil, from March to September 2020.All patients had >18 years,an underlying hematological disease and a moderate or severe COVID- 19 infection.For analyses, patients were grouped in:(1)benign or no oncological treatment(n=43),(2) intensive chemotherapy,including induction protocols for acute leukemia and stem cell transplantation conditioning(n=44) or(3) intermediate chemotherapy,including lymphoma regimens,myeloma triple treatment or continuous treatment( n=31).The primary outcome was in-hospital mortality;secondary outcome was overall survival after admission in the COVID-19 unit.Univariate analysis(UVA) used odds ratio(OR) for baseline characteristics and ROC curve analysis for laboratory tests collected at admission.Multivariate analyses(MVA) were adjusted by age and hematological disease status group.The median follow-up and survival time after COVID-19-unit admission were estimated by Kaplan- Meier method.All statistical tests were two-sided;p-values<0.05 were considered significant. Results: Median age was 58(19-90) years and 55% of patients were male. Most patients(83%)had hematological malignancies,- mainly non-Hodgkin lymphoma(29%) and multiple myeloma(19%). The most frequent benign disease was sickle cell disease(5%).12 patients had undergone hematopoietic stem cell transplantation (HSCT),4 allogeneic and 8 autologous.70% had at least one comorbidity, mostly arterial hypertension and diabetes mellitus. Thromboembolic events occurred in 9%. Median hospital stay in the COVID-19 unit was 12(1-63) days;54% needed intensive care and 41% mechanical ventilation.In-hospital mortality rate was 41%[95%CI 32-50];most deaths occurred in patients with malignancies. Median follow-up was 73(95%CI 61-81) and 54(95%CI 39-66) days after admission and discharge from the COVID-19 unit, respectively.UVA showed a risk of death increased by 25% every 10 years old.The risk of in-hospital death was 3-fold and 5-fold higher in groups 2 and 3 compared with group 1.MVA showed higher risk of death in patients from group 2(OR=11.1,95% CI 2.9- 54.8) or group 3(OR=9.7,95%CI 2.4-47.5]),who had lactate dehydrogenase( LDH)>440 U/L(OR=16.8,95%CI 4.9-71.8),C-reactive protein(CRP)>100 mg/L(OR=4.1,95%CI 1.4-13.6) or platelet count<150x10e9/L(OR=3.7,95%CI 1.3-11.7), regardless of age(OR=1.2,95%CI 1.0-1.5).79% of in-hospital deaths were from COVID-19;others were mainly due to hematological disease.The overall median survival time after admission was 92 days(95% CI 34-NA) and the 75-day survival probability was 51%(95%CI 41-60).25% of patients had hospital readmission,mostly due to other infections. Summary/Conclusion: In line with other reports,patients with hematological diseases are at higher risk of mortality from COVID-19 infection, particularly in low and middle income countries.In our cohort, prognostic factors were status of disease,platelets count,LDH and CRP. These findings might help risk stratification and prioritization of vaccines in this setting.