Dress por litio: reporte de un caso pediátrico / Lithium dress: a pediatric case report / Dress por Lítio: relato de caso pediátrico

La reacción a medicamentos con eosinofilia y síntomas sistémicos denominada DRESS(por sus siglas en inglés, Drug Reaction with Eosinophilia and Systemic Symptoms) hace parte de un amplio espectro denominado toxicodermias. La incidencia exacta no es conocida en niños; sin embargo, en la literatura se ha estimado una tasa de mortalidad que puede llegar a ser tan alta como el 10 %. Presentamos el caso de una paciente adolescente con antecedente personal de trastorno afectivo bipolar (TAB), quien recibía de forma ambulatoria sertralina, quetiapina y trazodona. Por presencia de alucinaciones se adicionó litio al manejo. Diez días después acude al servicio de urgencias por aparición de erupción cutánea y síntomas sistémicos, por lo que se sospechó un cuadro clínico secundario a hipersensibilidad a los medicamentos.

The reaction to drugs with eosinophilia and systemic symptoms called DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) is part of a broad spectrum called toxicodermias. The exact incidence is not known in children; However, a mortality rate that can be as high as 10% has been estimated in the literature. We present the case of a teenage patient with a personal history of bipolar affective disorder (BD), who received sertraline, quetiapine and trazodone on an outpatient basis. Due to the presence of hallucinations, lithium was added to the management. Ten days later she went to the emergency department due to the appearance of a skin rash and systemic symptoms, for which a clinical condition secondary to hypersensitivity to medications was suspected.

A reação a medicamentos com eosinofilia e sintomas sistêmicos denominada DRESS (Por suas siglas em inglês Drug Reaction with Eosinophilia and Systemic Symptoms) faz parte de um amplo espectro denominado toxicodermias. A incidência exata não é conhecida em crianças; No entanto, uma taxa de mortalidade que pode chegar a 10% foi estimada na literatura. Apresentamos o caso de um paciente adolescente com história pessoal de transtorno afetivo bipolar (TAB), que recebeu sertralina, quetiapina e trazodona em regime ambulatorial. Devido à presença de alucinações, foi adicionado lítio ao manejo. Dez dias depois, recorreu ao pronto-socorro devido ao aparecimento de erupção cutânea e sintomas sistêmicos, suspeitando-se de quadro clínico secundário a hipersensibilidade a medicamentos.

Cistitis eosinofílica en mujer con enfermedad renal crónica

Se presenta caso de una mujer de 70 años, con antecedentes de enfermedad renal crónica sin requerimiento dialítico que ingresa con descompensación aguda y que mejora con sesiones de hemodiálisis. Al examen físico llama la atención hematuria franca por lo que se solicita ecografía de pelvis donde aprecia vejiga distendida con coágulo en su interior de 7,2 cm. La tomografía muestra aparente tumor de pared de vejiga. La uretrocistografía confirma una cistitis crónica eosinofílica. Es manejada con hidroxicina teniendo respuesta favorable cediendo episodios de hematuria. La cistitis eosinofílica es una condición médica rara que se presenta con síntomas urinarios tales como disuria y hematuria. Es más común en niños que en adultos. El método diagnóstico es a través de una biopsia de pared vesical por cistoscopía. El manejo está dirigido a aliviar los síntomas. El interés por el caso se debe a la rareza de esta patología.

We present the case of a 70-year-old woman with a history of chronic kidney disease without dialysis who was admitted with acute decompensation and improved with hemodialysis sessions. On physical examination, frank hematuria was noticeable, therefore a pelvic ultrasound was requested, where a distended bladder with a 7.2 cm clot inside was observed. The tomography showed an apparent bladder wall tumor. Cyst urethrography confirmed chronic eosinophilic cystitis. It was managed with hydroxyzine, having a favorable response, reducing episodes of hematuria. Eosinophilic cystitis is a rare medical condition that presents with urinary symptoms such as dysuria and hematuria. It is more common in children than in adults. The diagnostic method is through a bladder wall biopsy by cystoscopy. Management is aimed at relieving symptoms. The interest in the case is due to the rarity of this pathology.

Clozapine-Induced Eosinophilia: a Case Report

Introduction: Clozapine is an atypical antipsychotic drug eligible for treatment-resistant schizophrenia. It frequently represents the best and the only choice in resistant schizophrenia. However, its use is feared by many professionals due to its possible adverse effects, such as eosinophilia. Case report: We report a case of a young white male suffering from treatment-resistant schizophrenia who rapidly developed eosinophilia after starting clozapine. Discussion: We present a case of a 26-year-old white man diagnosed with schizophrenia with poor clinical response to several antipsychotics owing to which clozapine was started. Psychotic symptoms improved dramatically but a progressively ascendant eosinophilia was reported during serial haematological analyses. The patient remained physically asymptomatic. An exhaustive assessment with ancillary diagnostic tests revealed no cause for eosinophilia. Thus, a diagnosis of clozapine-induced eosinophilia was made. The drug was discontinued and eosinophil count progressively returned to normal but psychotic symptoms worsened. Conclusions: Clozapine treatment is frequently feared due to its possible side effects and complications, delaying its use in refractory schizophrenia. Also, to our knowledge, there are no specific guidelines on how to manage haematological side effects such as eosinophilia. This is problematic as, in some cases, it may lead to an unnecessary withdrawal of clozapine with a worsening of psychotic symptoms. We present a brief discussion of the recent literature on the subject.

Introducción: La clozapina es un fármaco antipsicótico atípico eligible para la esquizofrenia resistente al tratamiento. Con frecuencia representa la mejor y la única opción para la esquizofrenia resistente. Sin embargo, muchos profesionales temen utilizarla por sus posibles efectos adversos, como la eosinofilia. Reporte de caso: Se expone el caso de un joven blanco que sufre esquizofrenia resistente al tratamiento y desarrolló eosinofilia rápidamente tras comenzar el tratamiento con clozapina. Discusión: Varón de 26 años con diagnóstico de esquizofrenia y mala respuesta clínica a varios antipsicóticos, por lo que se inició clozapina. Los síntomas psicóticos mejoraron drásticamente, pero los análisis hematológicos seriados informaron una eosinofilia en ascenso progresivo. El paciente permaneció físicamente asintomático. Una evaluación exhaustiva con pruebas de diagnóstico complementarias no reveló ninguna causa de eosinofilia. Por lo tanto, se diagnosticó eosinofilia inducida por clozapina. Se suspendió el fármaco, el recuento de eosinófilos volvió progresivamente a la normalidad, pero los síntomas psicóticos empeoraron. Conclusiones: A menudo se teme tratar con clozapina por sus posibles efectos secundarios y sus complicaciones, lo cual retrasa su uso en la esquizofrenia refractaria. Además, hasta donde sabemos, no existen pautas específicas sobre cómo tratar los efectos secundarios hematológicos como la eosinofilia. Esto es problemático porque, en algunos casos, puede conducir a suspender innecesariamente la clozapina y que empeoren los síntomas psicóticos. Se presenta una breve discusión de la literatura reciente sobre el tema.

Reação a drogas com eosinofilia e sintomas sistêmicos (DRESS): desafio no diagnóstico e tratamento / Drug reaction with eosinophilia and systemic symptoms (DRESS): a diagnostic and treatment challenge

Introdução: A reação a medicamentos com eosinofilia e sintomas sistêmicos (DRESS) trata-se de uma doença grave, sendo sua gravidade relacionada ao grau de acometimento visceral, e sua taxa de mortalidade de cerca de 10%. Seu diagnóstico é desafiador, e a utilização do escore RegiSCAR como ferramenta facilita a formação deste diagnóstico. Objetivo: Analisar os aspectos clínicos, laboratoriais, evolução e classificação dos casos segundo o RegiSCAR dos pacientes internados no serviço de Alergia e Imunologia do Hospital do Servidor Público Estadual de São Paulo, com o diagnóstico de DRESS. Método: Trata-se de um estudo retrospectivo baseado na análise de prontuários de pacientes atendidos no período entre janeiro de 2006 a janeiro de 2020. Resultados: Neste estudo verificou-se maior prevalência do sexo feminino, e a DRESS acometeu principalmente adultos e idosos, tendo como comorbidades mais frequentes as doenças cardiovasculares. Dos sintomas clínicos, 69,2% dos pacientes apresentava febre, e a alteração laboratorial mais encontrada foi a presença de eosinofilia. A lesão cutânea mais frequente foi o exantema maculopapular, e os medicamentos, os anticonvulsivantes. O tempo prévio de uso do medicamento foi de 2,1 semanas, e todos os pacientes receberam corticoide sistêmico como tratamento principal, e 3 pacientes fizeram uso da imunoglubulina humana como tratamento adicional. A mortalidade foi de 7% na fase aguda, e 14% por causas secundárias. Conclusão: A DRESS é uma síndrome complexa grave e potencialmente fatal, cujo diagnóstico é desafiador. O uso do escore preconizado pelo RegiSCAR demonstrou ser importante auxílio na confirmação do diagnóstico e na diferenciação de outras doenças. A mortalidade encontrada destaca a gravidade da doença. Reconhecer e excluir a droga implicada e iniciar um tratamento precoce permite maior chance de sobrevida para estes pacientes.

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a serious disease. Its severity is related to the degree of visceral involvement and its mortality rate is approximately 10%. Diagnosis is a challenge, although RegiSCAR scores can facilitate the process. Objective: To analyze clinical and laboratory data, clinical course, and classify cases according to RegiSCAR scores among patients diagnosed with DRESS who were admitted to the Allergy and Immunology service of the Hospital do Servidor Público Estadual de São Paulo. Method: This retrospective study analyzed the medical records of patients seen between January 2006 and January 2020. Results: There was a higher prevalence of women, with DRESS mainly affecting adults and older adults; cardiovascular diseases were the most frequent comorbidity. The most common clinical symptom was fever (69.2%), while the most common laboratory finding was eosinophilia. The most frequent skin lesion was maculopapular rash, and anticonvulsants were the main prescribed drug class. The drug was used for a mean of 2.1 weeks, and all patients received systemic corticosteroids as the main treatment. Human immunoglobulin was used as an additional treatment in 3 patients. Mortality was 7% in the acute phase and 14% due to secondary causes. Conclusion: DRESS is a severe, complex, and potentially fatal syndrome whose diagnosis is challenging. RegiSCAR scores helped confirm diagnosis and differentiate it from other diseases. The disease's mortality highlights its severity. Recognizing and excluding the implicated drug and initiating early treatment led to a greater chance of survival for these patients.

DRESS, una reacción alérgica no mediada por IgE / DRESS, a Non-IgE Mediated Allergic Reaction

Resumen El síndrome de reacción a medicamentos con eosinofilia y síntomas sistémicos (DRESS, por sus siglas en inglés) es una respuesta de hipersensibilidad multisistémica poco frecuente inducida por uno o varios medicamentos que puede inducir una reacción adversa cutánea grave, la cual es difícil de diagnosticar y pone en peligro la vida del paciente si no es identificada y no se recibe tratamiento. Frecuentemente, se manifiesta como una erupción cutánea amplia, linfadenopatía, signos de afectación de órganos viscerales y alteraciones hematológicas, como leucocitosis, eosinofilia y, en ocasiones, linfocitosis atípica que se presentan de 2 a 8 semanas posterior a la administración del fármaco responsable. Los medicamentos responsables con mayor número de reportes son la fenitoína, la carbamazepina, el alopurinol y el abacavir. Se han identificado algunos alelos específicos del antígeno leucocitario humano (HLA) que se asocian a la hipersensibilidad de estos fármacos. La fisiopatología del síndrome de DRESS aún no se conoce por completo, generalmente se trata de una respuesta de hipersensibilidad mediada por células T, al interactuar con el receptor del complejo principal de histocompatibilidad en individuos con factores de susceptibilidad genética, como ocurre en otros cuadros de reacciones graves secundarias a la ingesta de fármacos. Los criterios del European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) son los más utilizados para su diagnóstico. El síndrome de hipersensibilidad inducido por fármacos (DiHS), el síndrome de Stevens-Johnson (SSJ), la necrólisis epidérmica tóxica (NET), y la pustulosis exantemática generalizada aguda (PEGA) deben considerarse ante cualquier exantema que aparezca posterior a la administración de cualquier fármaco. La terapia incluye la eliminación del agente causal lo antes posible, así como los corticosteroides sistémicos, los cuales son los pilares del tratamiento.. Los agentes ahorradores de esteroides, como la ciclosporina, las inmunoglobulinas intravenosas (IVIGs) y otros agentes inmunosupresores, se han utilizado con éxito para contribuir al tratamiento.

Abstract DRESS (drug reaction syndrome with eosinophilia and systemic symptoms) is a rare drug-induced multisystemic hypersensitivity response that can induce a severe cutaneous adverse reaction that is difficult to diagnose and treat. It frequently manifests as an extensive skin rash, systemic symptoms, lymphadenopathy, visceral organ involvement, and hematological alterations, mainly leukocytosis, eosinophilia, and sometimes atypical lymphocytosis that manifest 2 to 8 weeks after continuous administration of the responsible drug. The most prevalent drugs related with this syndrome are phenytoin, carbamazepine, allopurinol, and abacavir. Some specific human leukocyte antigen (HLA) alleles have been identified that are associated with hypersensitivity to these drugs. The pathophysiology of DRESS syndrome is not yet fully understood; the main hypothesis is a T-cell mediated hypersensitivity response when interacting with the major histocompatibility complex receptor in individuals with genetic susceptibility factors. The criteria of the European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) are the most commonly used for the diagnosis of DRESS syndrome. Drug-induced hypersensitivity syndrome (DiHS), Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP) should be considered for any rash that appears following the administration of any drug. Therapy of DRESS includes the elimination of the causative agent as soon as possible, as well as systemic corticosteroids which are the cornerstones of treatment. Steroid-sparing agents such as cyclosporine, intravenous immunoglobulins (IVIGs), and other immunosuppressive agents have been used successfully to contribute to treatment.

Subserous Eosinophilic Colitis: A Case Report in a Private Hospital in Lima, Peru

Eosinophilic colitis is a rare gastrointestinal disease that belongs to the group of so-called primary eosinophilic diseases of the digestive tract. There are three types: mucosa, transmural (muscular), and subserous. We present the case of a 23-year-old male patient with a clinical picture of abdominal pain, nausea, chronic diarrhea, and ascites. Parasitic and other secondary etiologies were ruled out. Upper digestive endoscopy was not helpful. Colonoscopy revealed characteristics of inflammation in the distal ileum and ascending colon, the histological findings of which were consistent with eosinophilic colitis. The study of ascitic fluid was suggestive of eosinophilic ascites. The patient received induction treatment with prednisone 40 mg daily orally; remission was achieved after two weeks, and maintenance therapy based on prednisone was continued with the progressive withdrawal of the dose. Control of the disease was successful.

La colitis eosinofílica es una patología gastrointestinal infrecuente que pertenece al grupo de las denominadas enfermedades primarias eosinofílicas del tracto digestivo. Existen 3 tipos: mucosa, transmural (muscular) y subserosa. Presentamos el caso de un paciente varón, de 23 años de edad, con un cuadro clínico de dolor abdominal, náuseas, diarrea crónica y presentación de ascitis. Se descartan etiologías parasitarias y otras secundarias. La endoscopia digestiva alta no fue contribuidora. Mediante una colonoscopia se observaron características de inflamación en el íleon distal y el colon ascendente, cuyos hallazgos histológicos son compatibles con colitis eosinofílica. El estudio de líquido ascítico es sugestivo de ascitis eosinofílica. El paciente recibió tratamiento de inducción con prednisona a 40 mg diarios por vía oral, se logró la remisión a las 2 semanas y se continuó con terapia de mantenimiento a base de prednisona con retiro progresivo de la dosis. Se logró el control de la enfermedad de manera exitosa.

An Unusual Case of DRESS Syndrome Presenting with Marked Leucocytosis.

Drug reaction with eosinophilia and systemic symptom (DRESS) syndrome is a rare severe drug-induced idiosyncratic hypersensitivity characterized by maculopapular and/or erythrodermic eruption, fever, peripheral lymphadenopathy, eosinophilia or atypical lymphocytosis, and visceral organ involvement. The estimated incidence of this syndrome ranges from 1/1000 to 1/10,000 drug exposures. In this report, we describe a case of DRESS syndrome in a young female with a unique presentation. The DRESS syndrome can be difficult to diagnose as its clinical findings can mimic those of other systemic diseases. This case emphasizes the importance of incorporation of the patient’s clinical and medication history in the interpretation of hematological investigations.

Angiolymphoid hyperplasia with eosinophilia: A report of a rare entity in an elderly male

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign proliferation of blood vessels of uncertain etiology. It primarily affects the head‑and‑neck region. Histologically, it is characterized by the prominent proliferation of plump endothelial cells, and accompanying eosinophilic and lymphocytic infiltration. Herein, we report the case of ALHE in a 65‑year‑old male.

Clinical characteristics, ultrasonic diagnosis, treatment and outcomes of eosinophilic fasciitis: a retrospective single-center analysis of 45 cases / 南方医科大学学报

OBJECTIVE@#To evaluate the clinical features, laboratory and imaging results, treatment and outcomes of eosinophilic fasciitis (EF) and assess the value of ultrasound in the diagnosis of EF.@*METHODS@#We retrospectively analyzed the clinical data of 45 patients with EF treated in our center from January 1, 2006 to February 28, 2022. The consistency between the diagnoses of EF based on ultrasound and MRI findings was assessed.@*RESULTS@#In the 45 EF patients (male/female ratio 3.5:1), the age of onset ranged from 16 to 64 years with a mean disease course of 22.6 months. The average time from symptom onset to diagnosis was 16 months. The most common possible trigger of the disease was vigorous exercise (10/45), causing symmetrical lesions in the limbs, most commonly in the forearms (86.7%) and lower legs (80%). Clinical features of EF included subcutaneous swelling and induration (95.6%), arthralgia and arthritis (55.6%), groove sign (42.2%), hand joint contractures (42.2%), skin pigmentation (37.8%), and peau d'orange appearance (13.3%). Eosinophilia was found in 31 patients (68.9%). Hypergammaglobulinemia was seen in 23/44 (52.3%) and positive antinuclear antibodies in 9 (20%) of the patients. Twentyone of the patients were treated with high-dose methylprednisolone (≥200 mg daily for 3 to 5 consecutive days), and compared with the patients who did not receive this treatment, these patients more frequently experienced relapse before admission, had more extensive involvement, and had a higher rate of hypergammaglobulinemia without fever, but these differences were not statistically significant. Of the 31 patients (68.9%) with follow-up data (for a median of 3.2 years [range 0.2-15.9]), complete remission was achieved in 12 (38.7%) patients, and the accumulative complete remission rate was 44.1% at 5.5 years. No specific baseline characteristics or immunosuppressants were found to correlate with the treatment response. A total of 26 patients underwent both ultrasound and MRI examination, and the Kappa value of the diagnostic results between ultrasound and MRI was 0.91.@*CONCLUSION@#EF is characterized by symmetrical subcutaneous swelling and induration in the limbs, accompanied by eosinophilia and hypergammaglobulinemia. Glucocorticoid is effective for treating EF. Ultrasound examination can identify thickening of subcutaneous fascia for an early diagnosis of EF.

Diagnostic value of fractional exhaled nitric oxide combined with small airway function test for chronic cough / 中国基层医药

Objective:To investigate the value of fractional exhaled nitric oxide (FeNO) combined with small airway function test to replace bronchial provocation test and induced sputum test in differentiating cough variant asthma (CVA) from eosinophilic bronchitis (EB).Methods:The clinical data of 105 patients with chronic cough admitted to The Third People's Hospital of Hubei, Jianghan University from January 2018 to December 2021 were retrospectively analyzed. These patients consisted of 40 patients with CVA (CVA group), 25 patients with EB (EB group), and 40 patients with other chronic coughs (other chronic cough group). FeNO and lung function were compared between groups. The value of FeNO, small airway function, and their combination in differentiating CVA from EB were analyzed using the receiver operating characteristic curves.Results:FeNO level was the highest in the CVA group [33.0 (30.0, 37.8) ppb], followed by the EB group [28.0 (25.5, 32.0) ppb], and the lowest in other chronic cough group [13.0 (11.0, 15.0) ppb]. There was significant difference in FeNO level between groups ( H value = 79.00, P < 0.05). There were no significant differences in forced vital capacity (FVC), forced expiratory volume in 1 second (FEV 1), FEV 1/FVC, peak expiratory flow (PEF) between groups (all P > 0.05). Maximal mid-expiratory flow (MMEF) [74 (66.0, 77.4) in the CVA group, 80 (79.0, 83.3) in the EB group, 88.0 (86.4, 90.0) in other chronic coughs group], FEF25 (%) [70.0 (60.3, 75.1) in the CVA group, 78.0 (74.1, 85.0) in the EB group, 81.7 (78.9, 86.3) in other chronic coughs group], FEF50 (%) [75.2 (67.1, 80.8) in the CVA group, 80.6 (75.7, 85.9) in the EB group, 89.4 (87.0, 90.5) in other chronic coughs group], FEF75 (%) [76.4 (68.7, 85.8) in the CVA group, 80.9 (77.4, 89.7) in the EB group, 90.8 (87.2, 94.2) in other chronic coughs group] were significantly lower in the CVA group than those in other chronic coughs group. With the exception of FEF25 (%), MMEF (%), FEF50 (%), and FEF75 (%) were significantly lower in the EB group compared with other chronic coughs group. MMEF (%) and FEF25 (%) in the CVA group were significantly lower compared with the EB group. There were significant differences in MMEF (%), FEF50 (%), and FEF75 (%) between groups ( H = 62.82, 47.04, 47.41, 49.11, all P < 0.01). There were significant differences in FEF50 (%) and FEF75 (%) between CVA and EB groups (both P > 0.05). In binary logistic regression equation, FeNO and MMEF (%) were important indexes to distinguish CVA from EB ( P < 0.05). Bronchial provocation test and induced sputum test were used as the gold standard to distinguish CVA from EB. When FeNO and MMEF (%) were used separately to distinguish CVA from EB, the optimal threshold value was 30.0 ppb and 77.7 respectively, the area under the receiver operating characteristic curve was 0.77 and 0.82 respectively, the diagnostic sensitivity was 70% and 77.5% respectively, and the diagnostic specificity was 72% and 88% respectively. When FeNO and MMEF (%) were used in combination to distinguish CVA from EB, the area under the receiver operating characteristic curve was 0.89, and the diagnostic sensitivity and specificity was 75% and 96% respectively. Conclusion:FeNO and MMEF (%) can be used to distinguish CVA from EB. FeNO combined with MMEF (%) has a higher value in distinguishing CVA from EB than FeNO and MMEF alone.

Periostin as an important biomarker of inflammatory phenotype T2 in Brazilian asthma patients / Periostina como um importante biomarcador do fenótipo inflamatório T2 em pacientes brasileiros com asma

ABSTRACT Objective: The aim of this study was to assess the laboratory performance of periostin associated with a panel of biomarkers to identify the inflammatory phenotype of Brazilian asthma patients. Methods: We evaluated 103 Brazilian individuals, including 37 asthmatics and 66 nonasthmatic controls. Both groups underwent analyses for serum periostin, eosinophil levels in the peripheral blood, the fraction of exhaled nitric oxide (FeNO), total serum IgE, urinary leukotriene E4, and serum cytokines. Results: Higher levels of periostin (p = 0.005), blood eosinophils (p = 0.012), FeNO (p = 0.001), total IgE (p < 0.001), and IL-6 (p ≤ 0.001) were found in the asthmatic patients than the controls. Biomarker analyses by the ROC curve showed an AUC greater than 65%. Periostin (OR: 12,550; 95% CI: 2,498-63,063) and IL-6 (OR: 7,249; 95% CI: 1,737-30,262) revealed to be suitable asthma inflammation biomarkers. Blood eosinophils, FeNO, total IgE, IL-6, TNF, and IFN-g showed correlations with clinical severity characteristics in asthmatic patients. Periostin showed higher values in T2 asthma (p = 0.006) and TNF in non-T2 asthma (p = 0.029). Conclusion: The panel of biomarkers proposed for the identification of the inflammatory phenotype of asthmatic patients demonstrated good performance. Periostin proved to be an important biomarker for the identification of T2 asthma.

RESUMO Objetivo: O objetivo deste estudo foi de avaliar o desempenho laboratorial da periostina associada a um painel de biomarcadores para identificar o fenótipo inflamatório de pacientes brasileiros com asma. Métodos: Foram avaliados 103 indivíduos brasileiros, incluindo 37 asmáticos e 66 controles não asmáticos. Ambos os grupos foram submetidos a análises de periostina sérica, níveis de eosinófilos no sangue periférico, a fração exalada de óxido nítrico (FeNO), IgE sérica total, leucotrieno E4 urinário e citocinas séricas. Resultados: Maiores níveis de periostina (p = 0,005), eosinófilos periféricos (p = 0,012), FeNO (p = 0,001), IgE total (p < 0,001) e IL-6 (p ≤ 0,001) foram encontrados nos pacientes asmáticos do que nos controles. As análises de biomarcadores pela curva ROC mostraram uma AUC superior a 65%. A periostina (OR: 12.550; IC 95%: 2.498-63.063) e a IL-6 (OR: 7.249; IC 95%: 1.737-30.262) se mostraram biomarcadores adequados da inflamação da asma. Eosinófilos periféricos, FeNO, IgE total, IL-6, TNF e IFN-g apresentaram correlação com características clínicas de gravidade em pacientes asmáticos. A periostina teve valores mais elevados na asma T2 (p = 0,006) e o TNF na asma não T2 (p = 0,029). Conclusão: O painel de biomarcadores proposto para a identificação do fenótipo inflamatório de pacientes asmáticos demonstrou bom desempenho. A periostina provou ser um importante biomarcador para a identificação da asma T2.

Combined Allergic Bronchopulmonary Aspergillosis and Tropical Pulmonary Eosinophilia: A Rare Co-occurrence

Allergic bronchopulmonary aspergillosis (ABPA) and tropical pulmonary eosinophilia (TPE) are common lung diseases presenting with peripheral blood eosinophilia. Although these have been widely reported both from India and outside, simultaneous co-occurrence of the two diseases has not been reported so far. We hereby present a case of an elderly male, a known case of asthma, who was diagnosed to have concurrent ABPA and TPE. Partial clinical response as well as the persistence of eosinophilia after ABPA treatment raised the suspicion that subsequently led to the diagnosis of TPE. The concurrent treatment of both conditions led to satisfactory clinical and serological improvement.

DRESS syndrome: Tricky to diagnose but easy to treat

A 27-year-old female presented to us with a short history of fever, jaundice, rash, and worsening hepatic dysfunction subsequent to treatment with intravenous antibiotics and alternative medicine for a urinary tract infection. The eosinophilia, lymphadenopathy, and transaminitis prompted us to consider a diagnosis of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) which can be fatal if not treated. The patient showed improvement in clinical and laboratory parameters after a course of steroids. This case is presented as DRESS syndrome that can prove rapidly fatal if not diagnosed and treated immediately.

Granuloma ulcerativo traumático con eosinoflia estromal: lesión simuladora de difícil diagnóstico. Presentación de caso / Traumatic ulcerative granuloma with stromal eosinophilia: Simulator lesión of difcult diagnosis. Presentation of a case

Introducción: el granuloma ulcerativo traumático con eosinofilia estromal es una afección benigna, crónica y autolimitante, que por su evolución clínica puede estar sujeta a confusión diagnóstica. Por ello, el caso que aquí se comparte expone particularidades de esta afección y su respuesta al tratamiento para permitir un mejor conocimiento de esta lesión. Se describen las características clínicas e histopatológicas y su evolución ante la terapéutica empleada. Se presenta una paciente femenina de seis años, con antecedentes de salud y de dientes neonatales, que hace tres meses manifiesta dos úlceras en mucosa sublingual que no cicatrizan ni mejoran al tratamiento anterior. Se indicaron estudios hematológicos, se realizó biopsia incisional de la lesión con su estudio histopatológico e inmunohistoquímico. Se obtuvo eosinofilia estromal y ausencia de linfocitos anómalos CD30. El tratamiento incluyó aplicación de corticosteroides tópicos e intralesionales, experimentando remisión de la lesión. Se mantiene la paciente bajo seguimiento clínico, sin recidiva de lesión. Conclusiones: el granuloma ulcerativo traumático con eosinofilia estromal es una lesión autolimitante que puede ser confundida con otras lesiones ulcerativas de la cavidad bucal, por lo que su estudio histopatológico resulta imprescindible para su diagnóstico. Asimismo, su análisis inmunohistoquímico es indispensable para precisar su naturaleza y probable evolución. El adecuado y exhaustivo proceso diagnóstico constituye una herramienta vital para lograr su identificación.

Background: Traumatic ulcerative granuloma with stromal eosinophilia is a benign, chronicle and self-healing lesion, which can be misdiagnosed for its clinical evolution, for this reason, this case report is carried out, showing particularities of this disease and its response in front to the treatment, giving a better identifcation of the lesion, were described the clinical and histopathological fndings of a case. A feminine patient, six years old, with neonatal health and dental history. She has presented during three months two ulcerative lesions in sublingual mucosa, which do not improve with the previous treatment. Hematological studies and biopsy were carried out, the incisional biopsy was analyzed with immunohistochemical test, the results were stromal eosinophilia and absence of anomalous lymphocytes CD30. She was treated with topical and intralesional corticosteroids, experiencing remission of the lesion. The patient had a long clinical follow up without recidive. Traumatic ulcerative granuloma with stromal eosinophilia is a self-healed lesion that needs a histopathological and inmunohistochemical analysis for an adequate diagnosis. The correct diagnostic sequence is a vital tool to achieve its identification.

Reacción a fármacos con eosinofilia y síntomas sistémicos (DRESS) / Drug reaction with eosinophilia and systemic symptoms (DRESS)

La reacción a fármacos con eosinofilia y síntomas sistémicos (del acrónimo en inglés DRESS: drug reaction with eosinophilia and systemic symptoms) o síndrome de hipersensibilidad inducida por fármacos (del acrónimo en inglés DIHS: drug induced hypersensitivity syndrome), es una reacción adversa a fármacos (RAF) grave e infrecuente. Los mecanismos involucrados en su fisiopatogenia incluyen diversas alteraciones de las enzimas metabolizadoras de fármacos, con la consecuente acumulación de metabolitos reactivos, la reactivación secuencial de virus de la familia del herpes, la predisposición genética asociada a ciertos alelos de antígenos leucocitarios humanos (HLA) y una respuesta de hipersensibilidad retardada de tipo IV. En la actualidad, se han ido incorporando nuevos fármacos responsables de este cuadro como medicamentos biológicos, inmunosupresores y quimioterápicos. La presentación clínica del DRESS es variable. Tiene una morbimortalidad alta y supone costos elevados en la atención médica. Su tratamiento consiste, en primer lugar, en la suspensión de los fármacos causales o sospechosos de desencadenar el síndrome. Luego, según la gravedad del cuadro, se pueden indicar corticosteroides sistémicos o inmunoglobulina (IGIV) combinada con corticosteroides, plasmaféresis, ciclosporina, micofenolato de mofetilo y rituximab. El objetivo de este trabajo fue realizar una revisión sobre el DRESS y destacar los aspectos nuevos y relevantes de los últimos 5 años.

Drug reaction with eosinophilia and systemic symptoms (DRESS) or drug-induced hypersensitivity syndrome (DIHS), is a serious and rare adverse drug reaction. Among the mechanisms involved in its pathophysiology, there are various alterations in drugmetabolizing enzymes with the consequent accumulation of reactive metabolites, sequential reactivation of viruses of the herpes family, genetic predis-position associated with certain HLA, and a type IV hypersensitivity response. Currently, new drugs responsible for this pathology have been incorporated, such as biologicals, immunosuppressants and chemotherapy. The clinical presentation of DRESS is variable. It has a high morbidity and mortality and involves high costs in medical care. Its treatment consists, in the first place, in the suspension of the causal or suspected drugs. Then, depending on severity, systemic corticosteroids or IVIG combined with corticosteroids, plasmapheresis, cyclosporine, mycophenolate mofetil, and rituximab may be indicated.The objective of this work was review DRESS and highlight the new and relevant aspects of the last 5 years.

Kimura disease with renal impairment: case series and literature review / 中华肾脏病杂志

Objective:To analyze the clinical and pathological characteristics, treatment and prognosis of renal changes in patients with Kimura disease and improve the clinicians′ understanding on renal manifestations of Kimura disease.Methods:The clinical data of Kimura disease patients with definite diagnosis and detailed data in Peking Union Medical College Hospital from January 1980 to August 2020 were retrospectively analyzed. The patients were divided into renal impairment group and non-renal impairment group according to whether the kidney was involved or not and the related clinical data between the two groups were compared. The patients presenting with nephrotic syndrome were followed up.Results:There were 60 patients with Kimura disease confirmed by pathological diagnosis with 48 males. The median age was 33(3, 62) years old, and the median duration was 36(12, 111) months. There were 18 cases complicated with renal injury in 49 patients with complete routine urine and renal function examination and the main manifestations of renal injury were proteinuria and/or microscopic hematuria. There was no significant difference at age, sex and absolute value of eosinophils between the two groups (all P>0.05). Compared with the renal inpairment group, patients in non-renal inpairment group had longer course of disease, higher levels of hypersensitive C-reactive protein and erythrocyte sedimentation rate, and lower median values of total eosinophils and total IgE, but there was no statistically significant difference (all P>0.05). Among the patients with renal involvement, 6 patients met the diagnostic criteria for nephrotic syndrome, and 5 of them completed renal biopsies. The renal pathological diagnosis was membranous nephropathy in 2 cases and minimal change disease in 3 cases, and no interstitial eosinophil infiltration was found in renal biopsy tissues. These patients had a good response to glucocorticoids and/or immunosuppressive therapy, and achieved complete remission of nephrotic syndrome; at the same time, lymphadenopathy caused by Kimura disease could be well controlled. Conclusions:Kimura disease can combine with various renal lesions, and the pathology of nephrotic syndrome can be membranous nephropathy or minimal change nephropathy. After energetic treatment of glucocorticoids and/or immunosuppressive therapy, nephrotic syndrome can be completely relieved, and lymphadenopathy can be well controlled. The relationship between Kimura disease and renal disease needs further study.

IL3-IGH fusion gene-positive pediatric acute lymphoblastic leukemia with hypereosinophilia as the first presentation: report of 1 case and review of literature / 白血病·淋巴瘤

Objective:To investigate the clinical characteristics and prognosis of IL3-IGH fusion gene-positive pediatric acute lymphoblastic leukemia (ALL) with hypereosinophilia as the first presentation.Methods:The clinical data of 1 pediatric IL3-IGH fusion gene-positive ALL patient with hypereosinophilia as the first presentation in January 2021 in Fujian Medical University Union Hospital was retrospectively analyzed and relevant literature was reviewed.Results:This 11-year-old male patient underwent bone marrow examination, and results showed that the proportion of eosinophils was increased; immunophenotyping disclosed that there were about 49.4% abnormal naive B lymphocytes in bone marrow; 43 leukemia fusion genes showed all negative; the whole transcriptome sequencing showed IL3-IGH fusion gene-positive. The patient was finally diagnosed as B-ALL with IL3-IGH fusion gene. According to the Chinese Children Cancer Group (CCCG)-ALL 2020 regimen, eosinophils returned to normal after induction therapy. Bone marrow examination on day 19 of induction showed that the proportion of promyelocytes was 0.005, the proportion of eosinophils was 0.05, and the minimal residual disease (MRD) was 23.02%. Bone marrow examination on day 46 of induction showed remission, and MRD was 0.18%. Consolidation chemotherapy used CAT (cyclophosphamide 1 g/m 2 once; cytarabine 50 mg/m 2, 12 h once, 7 days in total; mercaptopurine 40 mg/m 2, once per night, 7 days in total) regimen. Then the patient was added with lusotinib (75 mg 12 h once) orally and continued to receive high-dose methotrexate (5 g/m 2) regimen chemotherapy for 2 courses, the MRD was 0.20%. Chimeric antigen receptor T-cell (CAR-T) regimen was administered, followed by negative MRD. Conclusions:IL3-IGH fusion gene ALL is more frequently found in males, and more common in older children and young adults. It is prone to organ infiltration damage, and it has a high rate of induction failure and recurrence as well as poor prognosis.

Research progress on the relationship between hypereosinophilia and ischemic stroke / 中华神经科杂志

Hypereosinophilia (HE) is a kind of hematology disorder affecting multiple organs. Multiple studies demonstrated that HE was correlated with ischemic stroke, and it could be a rare and reversible cause for ischemic stroke. Therefore, more and more concerns have been concentrated by neurologists in recent years. Based on the research data, the definition, typical characteristics, possible mechanism, diagnosis and treatment principles of HE related ischemic stroke were summarized systematically, in order to provide possible personalized treatment strategies for this disease.

Immunoanalysis of different antigenic preparations of Angiostrongylus cantonensis for neuroangiostrongyliasis diagnosis improvement

BACKGROUND Angiostrongylus cantonensis is the etiological agent of neuroangiostrongyliasis in humans, which is developed in gastropods and vertebrate species, mainly rodents. Human transmission occurs through consumption of molluscs and paratenic hosts infected with L3, and the migration of larvae to the central nervous system causes eosinophilic meningitis. Laboratory diagnosis is based on molecular and immunological tests, using young or adult females as a source of antigens. However, these tests give positive results only after several weeks of symptoms onset and also cross-reactions with others parasite infections may occur. OBJECTIVES The purpose of this work was to study different antigenic preparations of distinct evolutionary phases of A. cantonensis, in order to improve serological techniques for disease immunodiagnosis. METHODS For this purpose, antigenic fractions of different evolutionary forms were evaluated by Dot-enzyme-linked immunosorbent assay (Dot-ELISA) and Western blot using serum samples. FINDINGS All analysed fractions showed reactivity with serum samples from patients with neuroangiostrongyliasis, especially female membrane alkaline (FAM) and female soluble alkaline (FAS) fractions together with female soluble saline (FSS), improving the technique specificity. MAIN CONCLUSIONS The results point to the possibility of use of raw female antigens in association with alkaline membrane antigens extracted from adult worms to aid in diagnosis and helps initiate neuroangiostrongyliasis surveillance and control actions.