RESUMO
OBJECTIVES: Extrapulmonary tuberculosis (EPTB) can be difficult to diagnose, especially in severe forms. The Xpert MTB/RIF Ultra test introduced an additional category called trace to reference very small amounts of Mycobacterium tuberculosis complex (MTBC) DNA. The objective of our multicenter study was to evaluate whether the trace result on an extrapulmonary (EP) sample is a sufficient argument to consider diagnosing tuberculosis and starting treatment, even in severe cases. METHODS: A retrospective, multicenter cohort study was conducted from 2018 to 2022. Patients strongly suspected of EPTB with a trace result on an EP specimen were included. Hospital records were reviewed for clinical, treatment, and paraclinical data. RESULTS: A total of 52 patients were included, with a severe form in 22/52 (42.3%) cases. Culture was positive for MTBC in 33/46 (71.7%) cases. Histological analysis showed granulomas in 36/45 (80.0%) cases. An Ultra trace result with a presumptive diagnosis of TB led to the decision to treat 41/52 (78.8%) patients. All patients were started on first-line anti-TB therapy (median duration of 6.1 months), with a favorable outcome in 31/35 (88.6%) patients. The presence of a small amount of MTBC genome in EPTB is a sufficient argument to treat patients across a large region of France.
Assuntos
Doenças do Colágeno , Exantema , Doenças Reumáticas , Neoplasias Cutâneas , Tuberculose Cutânea , Uveíte , Humanos , Pele , Uveíte/diagnósticoRESUMO
BACKGROUND: Immunoglobulin replacement therapy is recommended in case of severe hypogammaglobulinemia after allogeneic hematopoietic stem cell transplantation (allo-HSCT). However, the supposed increased risk of infection in case of hypogammaglobulinemia has not been confirmed in allo-HSCT. In this study, we assessed the relationship between the gamma globulin level and the risk of infection during the 100 days following the allo-HSCT. METHODS: We gathered the weekly laboratory tests from day 7 to day 100 of 76 allograft patients, giving a total of 1 044 tests. 130 infections were documented clinically, by imaging, or microbiologically. RESULTS: Average gamma globulin levels between D-7 and D100 did not differ between patients with or without infection (642 ± 232 and 671 ± 246 mg/dL, respectively, P = .65). Gamma globulin level <400 mg/dl was not associated with the occurrence of infection between the test studied and the next one (aOR 1.33 [0.84-2.15], P = .24). The gamma globulin level was not predictive of bacterial or fungal infections (AUC 0.54 [95%CI: 0.47-0.61]) nor of viral reactivations (AUC 0.51 [95%CI: 0.43-0.60]). CONCLUSIONS: This confirmed that the humoral deficiency is a minor part of the immune deficiency in the 100 days post-transplant. This questions the relevance of the indications of immunoglobulin substitution during this period.
Assuntos
Transplante de Células-Tronco Hematopoéticas/métodos , Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/terapia , Leucemia/terapia , Linfoma/terapia , Síndromes Mielodisplásicas/terapia , Infecções Oportunistas/diagnóstico , Idoso , Infecções Bacterianas/diagnóstico , Infecções Bacterianas/imunologia , Infecções Bacterianas/microbiologia , Ciclosporina/administração & dosagem , Ciclosporina/efeitos adversos , Feminino , Doença Enxerto-Hospedeiro/imunologia , Doença Enxerto-Hospedeiro/patologia , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Síndromes de Imunodeficiência/etiologia , Síndromes de Imunodeficiência/imunologia , Síndromes de Imunodeficiência/patologia , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Leucemia/imunologia , Leucemia/patologia , Linfoma/imunologia , Linfoma/patologia , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/administração & dosagem , Ácido Micofenólico/efeitos adversos , Micoses/diagnóstico , Micoses/imunologia , Micoses/microbiologia , Agonistas Mieloablativos/uso terapêutico , Síndromes Mielodisplásicas/imunologia , Síndromes Mielodisplásicas/patologia , Infecções Oportunistas/imunologia , Infecções Oportunistas/microbiologia , Infecções Oportunistas/virologia , Prognóstico , Curva ROC , Condicionamento Pré-Transplante/métodos , Transplante Homólogo , Ativação Viral/efeitos dos fármacos , gama-Globulinas/metabolismoAssuntos
Leucemia Mieloide Aguda , Pneumopatias , Mucormicose , Doenças Nasais , Pancitopenia , Triazóis/administração & dosagem , Idoso , Anfotericina B/administração & dosagem , Antifúngicos/administração & dosagem , Humanos , Hospedeiro Imunocomprometido , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/patologia , Leucemia Mieloide Aguda/terapia , Pneumopatias/diagnóstico , Pneumopatias/tratamento farmacológico , Pneumopatias/microbiologia , Pneumopatias/fisiopatologia , Masculino , Mucormicose/tratamento farmacológico , Mucormicose/etiologia , Mucormicose/imunologia , Mucormicose/fisiopatologia , Doenças Nasais/diagnóstico , Doenças Nasais/tratamento farmacológico , Doenças Nasais/imunologia , Doenças Nasais/microbiologia , Pancitopenia/diagnóstico , Pancitopenia/etiologia , Resultado do TratamentoRESUMO
BACKGROUND: Monoclonal gammopathy-associated systemic capillary-leak syndrome, also known as Clarkson disease, is a rare condition characterized by recurrent life-threatening episodes of capillary hyperpermeability in the context of a monoclonal gammopathy. This study was conducted to better describe the clinical characteristics, natural history, and long-term outcome of monoclonal gammopathy-associated systemic capillary-leak syndrome. METHODS: We conducted a cohort analysis of all patients included in the European Clarkson disease (EurêClark) registry between January 1997 and March 2016. From diagnosis to last follow-up, studied outcomes (eg, the frequency and severity of attacks, death, and evolution toward multiple myeloma) and the type of preventive treatments administered were monitored every 6 months. RESULTS: Sixty-nine patients (M/F sex ratio 1:1; mean ± SD age at disease onset 52 ± 12 years) were included in the study. All patients had monoclonal gammopathy of immunoglobulin G type, with kappa light chains in 47 (68%). Median (interquartile range) follow-up duration was 5.1 (2.5-9.7) years. Twenty-four patients (35%) died after 3.3 (0.9-8) years. Fifty-seven (86%) patients received at least one preventive treatment, including intravenous immunoglobulins (IVIg) n = 48 (73.8%), theophylline n = 22 (33.8%), terbutaline n = 22 (33.8%), and thalidomide n = 5 (7.7%). In the 65 patients with follow-up, 5- and 10-year survival rates were 78% (n = 35) and 69% (n = 17), respectively. Multivariate analysis found preventive treatment with IVIg (hazard ratio 0.27; 95% confidence interval, 0.10-0.70; P = .007) and terbutaline (hazard ratio 0.35; 95% confidence interval, 0.13-0.96; P = .041) to be independent predictors of mortality. CONCLUSIONS: We describe the largest cohort to date of patients with well-defined monoclonal gammopathy-associated systemic capillary-leak syndrome. Preventive treatment with IVIg was the strongest factor associated with survival, suggesting the use of IVIg as the first line in prevention therapy.
Assuntos
Síndrome de Vazamento Capilar/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Paraproteinemias/diagnóstico por imagem , Síndrome de Vazamento Capilar/etiologia , Síndrome de Vazamento Capilar/mortalidade , Síndrome de Vazamento Capilar/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paraproteinemias/complicações , Paraproteinemias/mortalidade , Paraproteinemias/patologia , Análise de Sobrevida , Terbutalina/uso terapêutico , Teofilina/uso terapêuticoRESUMO
The infective agent responsible for cat scratch disease, Bartonella henselae, is a rare cause of hepatic granulomatosis in immunocompetent adults. Clinical features include a prolonged fever or more typical symptoms such as lymphadenopathy associated with painful hepatomegaly and a fever following a cat scratch or bite. Images of micronodular hepatosplenic lesions on abdominal ultrasonography or computed tomography scan along with epithelioid granulomas in a liver biopsy can suggest this diagnosis. It is established with a serology by indirect immunofluorescence or by ELISA and/or the presence of Bartonella henselae DNA evidenced by PCR in the liver biopsy. We report two cases of hepatosplenic localizations of cat scratch disease in a 41-year-old woman and a 44-year-old man presenting asthenia and fever associated with a biological inflammatory syndrome and elevated liver enzymes.