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1.
Autops. Case Rep ; 11: e2021243, 2021. tab, graf
Article in English | LILACS | ID: biblio-1285402

ABSTRACT

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal syndrome resulting from a hyperactivated immune system. Diverse patient profiles and clinical presentations often result in misdiagnosis. This article describes the varied clinical presentations and autopsy findings in three patients with this entity. The etiopathogenesis of HLH, its disparate and confounding clinical features, the diagnostic criteria, and management principles are also briefly reviewed.


Subject(s)
Humans , Male , Adult , Middle Aged , Lymphohistiocytosis, Hemophagocytic/pathology , Autopsy , Hypertriglyceridemia , Macrophage Activation Syndrome , Ferritins , Immune System
3.
Arch. argent. pediatr ; 117(6): 676-678, dic. 2019.
Article in Spanish | LILACS, BINACIS | ID: biblio-1051372

ABSTRACT

La enfermedad de Kawasaki es una vasculitis febril, aguda y multisistémica, que afecta, principalmente, a niños menores de 5 años. Se describen las características clínicas, la evolución y las consideraciones terapéuticas en un paciente con diagnóstico de enfermedad de Kawasaki completo con manifestaciones multisistémicas graves, dentro de las cuales se resalta el síndrome de activación de macrófagos, que representa una complicación inusual y potencialmente mortal de la enfermedad


Kawasaki disease is a febrile, acute and multisystemic vasculitis that mainly affects children under 5 years of age. We describe the clinical characteristics, evolution and therapeutic considerations in a patient with a diagnosis of complete Kawasaki disease with severe multisystem manifestations, among which stands out the macrophage activation syndrome, which represents an unusual and potentially life-threatening complication of the illness


Subject(s)
Humans , Male , Child, Preschool , Macrophage Activation Syndrome , Meningitis, Aseptic , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/diagnostic imaging
4.
Rev. Paul. Pediatr. (Ed. Port., Online) ; 37(2): 252-256, Apr.-June 2019. tab, graf
Article in English | LILACS | ID: biblio-1013282

ABSTRACT

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.


RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.


Subject(s)
Humans , Female , Child , Cyclosporine/administration & dosage , Glucocorticoids/administration & dosage , Arthritis, Juvenile/complications , Arthritis, Juvenile/diagnosis , Arthritis, Juvenile/physiopathology , Arthritis, Juvenile/immunology , Chest Pain/diagnosis , Chest Pain/etiology , Tomography, X-Ray Computed/methods , Treatment Outcome , Immunoglobulins, Intravenous/administration & dosage , Pulse Therapy, Drug/methods , Electrocardiography/methods , Macrophage Activation Syndrome/etiology , Macrophage Activation Syndrome/physiopathology , Macrophage Activation Syndrome/blood , Macrophage Activation Syndrome/therapy , Immunosuppressive Agents/administration & dosage , Leukocytosis/diagnosis , Leukocytosis/etiology
5.
Article in Chinese | WPRIM | ID: wpr-351377

ABSTRACT

<p><b>OBJECTIVE</b>To study the clinical and laboratory features of macrophage activation syndrome (MAS) at the early stage of diagnosis, and to explore a method for early identification of MAS.</p><p><b>METHODS</b>A retrospective analysis was performed for the demographic data, clinical and laboratory features, and treatment outcomes of 21 MAS patients.</p><p><b>RESULTS</b>Of the 21 MAS patients, 14 had systemic juvenile idiopathic arthritis, 5 had Kawasaki disease (KD), and 2 had connective tissue disease (CTD) as primary diseases. The median time of MAS onset was 19 days. The KD patients had the shortest time of MAS onset, while the CTD patients had the longest onset time (P=0.009). The top 10 clinical symptoms were fever (95%), rash (86%), lymph node enlargement (67%), hemophagocytic phenomenon in bone marrow (63%), pulmonary disease (62%), serous effusion (62%), hepatomegaly (52%), cerebrospinal fluid abnormalities (50%), central nervous system damage (43%), and splenomegaly (38%). The median of hemoglobin level was lower than the normal value. The medians of C-reactive protein level and erythrocyte sedimentation rate were higher than the normal values. There were significant increases in serum ferritin, glutamic-pyruvic transaminase, aspartate aminotransferase, lactate dehydrogenase, and triglyceride. The median of fibrinogen level was lower than the normal value. There were significant increases in D-dimer, interleukin-6 (IL-6), interleukin-10 (IL-10), and interferon-γ (IFN-γ). Of the 21 patients, 20 were improved and discharged.</p><p><b>CONCLUSIONS</b>If patients with rheumatic disease have persistent fever, hepatic dysfunction, coagulation disorders, multiple organ impairment, significantly increased IL-10 and IFN-γ, and a persistent increase in serum ferritin, the development of MAS should be considered.</p>


Subject(s)
Adolescent , C-Reactive Protein , Child , Child, Preschool , Cytokines , Blood , Female , Fibrin Fibrinogen Degradation Products , Humans , Infant , Macrophage Activation Syndrome , Blood , Diagnosis , Drug Therapy , Male , Retrospective Studies
6.
Med. Afr. noire (En ligne) ; 64(02): 79-84, 2017.
Article in French | AIM, AIM | ID: biblio-1266225

ABSTRACT

Le Syndrome d'Activation Macrophagique (SAM) est défini comme la traduction clinico-biologique d'une prolifération et d'une activation non-spécifique des macrophages du système réticulo-histiocytaire avec phagocytose des éléments figurés du sang. Nous rapportons 5 cas de SAM secondaires chez des enfants hospitalisés dans le service de pédiatrie de l'hôpital Aristide le Dantec entre août 2015 et avril 2016. Il s'agissait de 3 filles et 2 garçons âgés de 7 ans à 14 ans. Cliniquement, la fièvre, l'altération de l'état général et la splénomégalie étaient constantes. Quatre patients ont présenté des adénopathies et chez 2 patients une hépatomégalie a été retrouvée. Au niveau de l'hémogramme, l'anémie était constante, la thrombopénie et la leuco-neutropénie étaient retrouvées chez 3 patients et le frottis sanguin révélait 36% de blastes chez un patient. L'hémophagocytose médullaire était retrouvée chez tous les patients, l'hyper ferritinémie était constante et chez trois patients une hypertriglycéridémie avec un taux élevé de lactate déshydrogénase (LDH) ont été notés. Le diagnostic était surtout guidé par le médullogramme et basé sur les critères de l'hemophagocytic histiocytosis et les étiologies étaient infectieuses et néoplasiques. Chez trois patients, le SAM était d'origine infectieuse et les germes retrouvés étaient le Streptococcus, l'Escherichia coli et le Mycobacterium tuberculosis alors que les deux autres cas étaient d'origines néoplasiques secondaires à une leucémie aiguë myéloïde et à un lymphome hodgkinien. Le traitement était basé sur l'antibiothérapie (cas 1 et 2), les antituberculeux (cas 3) et la chimiothérapie (cas 4 et 5). L'évolution était favorable chez tous nos patients


Subject(s)
Child , Inpatients , Macrophage Activation Syndrome/diagnosis , Macrophage Activation Syndrome/drug therapy , Macrophage Activation Syndrome/etiology , Senegal
7.
Rev. colomb. reumatol ; 23(2): 137-143, Apr.-June 2016. ilus, tab
Article in English | LILACS | ID: biblio-830402

ABSTRACT

INTRODUCTION: Macrophage activation syndrome (MAS) is a pathological systemic inflammatory reaction that is often fatal and underdiagnosed. There may be multiple organ failure that could be triggered in association with rheumatic, neoplastic or infectious diseases and/or drugs. It has been reported more in children than adults, probably as it is often associated with genetic abnormalities not described yet undescribed, genetic abnormalities. In most cases the genetic defect is not recognized in adults, or has a different etiology. The signs and symptoms of macrophage activation syndrome have been defined. Not suspecting its presence may lead to not making the diagnosis and thus, an increase in mortality. Diagnosis is a challenge, treatment has to be started early and be aggressive to reduce the high mortality rate. OBJECTIVES: To describe four adult patients with five MAS episodes related to different under-lying diseases, with the aim of making it familiar to the reader, to look for the syndrome and make a diagnosis. MATERIALS AND METHODS: Patients evaluated in outpatients and while in the hospital. RESULTS: We present the characteristics of MAS, with the diagnostic approach and the ther-apeutic possibilities and their outcomes. CONCLUSIONS: MAS is not looked for in the adult and could be fatal. It requires identification and early treatment to reduce the risk of mortality. It still needs to be studied to define the genetic defect, or other causes that may be responsible for the development of the syndrome.


INTRODUCCIÓN: El síndrome de activación macrofágica (SAM) es una reacción patológica inflamatoria sistémica, frecuentemente fatal y comúnmente no diagnosticada, que se acompaña de una falla multiorgánica y puede desencadenarse asociada a enfermedades reumáticas, neoplásicas, infecciosas o a drogas. Más descrita en niños que en adultos, probablemente en muchas ocasiones se relaciona con alteraciones genéticas aún no descritas. Sus síntomas y signos han sido definidos. El no sospecharlo conlleva a no diagnosticarlo y como consecuencia a un incremento importante del riesgo de mortalidad en el paciente; es por esto que el diagnóstico es un reto y el tratamiento debe de ser temprano y agresivo. OBJETIVOS: Describir 4 pacientes adultos con 5 episodios de SAM relacionado con diferentes enfermedades reumáticas, con el interés de familiarizar al lector con la búsqueda del síndrome y de realizar su diagnóstico. MATERIALES Y MÉTODOS: Estudio descriptivo de pacientes adultos evaluados en la consulta y hospitalizados. RESULTADO: Presentamos las características de los pacientes con SAM, el enfoque diagnóstico, las posibilidades terapéuticas y la evolución. CONCLUSIONES: El SAM es una enfermedad no buscada en el adulto que puede ser fatal, requiere ser identificada y tratada tempranamente para disminuir el riesgo de mortalidad. Aún requiere ser estudiada para definir defectos genéticos u otras etiologías que puedan ser responsables de este síndrome.


Subject(s)
Humans , Macrophage Activation Syndrome
8.
Article in English | WPRIM | ID: wpr-147090

ABSTRACT

As a new humanized monoclonal antibody against the interleukin-6 receptor, tocilizumab is currently used for the treatment of rheumatoid arthritis (RA) patients. Tocilizumab was reported to provoke drug-related liver toxicity, although there have been no reports on significant liver toxicity from tocilizumab in Korean patients with RA to date. Here, we describe the first case of tocilizumab-related liver toxicity in a patient with complicated RA, accompanied with macrophage activation syndrome, who had received tacrolimus and prednisolone and in whom both conventional disease modifying anti-rheumatic drugs, including methotrexate, leflunomide and sulfasalazine or tumor necrotizing factor-alpha blockades, were contraindicated due to drug eruption and a history of lung cancer.


Subject(s)
Antirheumatic Agents , Arthritis, Rheumatoid , Drug Eruptions , Humans , Interleukin-6 , Liver , Lung Neoplasms , Macrophage Activation Syndrome , Macrophage Activation , Macrophages , Methotrexate , Prednisolone , Sulfasalazine , Tacrolimus , Tranexamic Acid
9.
Article in English | WPRIM | ID: wpr-207500

ABSTRACT

Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3. We were unable to identify the cause for the HLH. He remained symptom-free until the development of back pain and right ankle joint pain with swelling at 15 years of age. He was diagnosed with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom aggravation despite taking naproxen and methotrexate, and the symptoms improved with etanercept. On admission, his laboratory data showed leukopenia with high ferritin and triglyceride levels. Bone marrow biopsy examination showed histiocytic hyperplasia with hemophagocytosis. There was no evidence of infection. He received naproxen alone, and his symptoms and laboratory data improved without any other immunomodulatory medications. Genetic study revealed no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune disease should have been considered as the cause of recurrent MAS in the young patient once primary HLH was excluded.


Subject(s)
Abscess , Adolescent , Ankle Joint , Autoimmune Diseases , Back Pain , Biopsy , Bone Marrow , Etanercept , Ferritins , HLA-B27 Antigen , Humans , Hyperplasia , Immunoglobulins , Inflammasomes , Leukocytes , Leukopenia , Lymphohistiocytosis, Hemophagocytic , Macrophage Activation Syndrome , Macrophage Activation , Macrophages , Malaria , Male , Methotrexate , Naproxen , Pancytopenia , Sacroiliitis , Splenomegaly , Spondylitis, Ankylosing , Steroids , Triglycerides
10.
Rev. bras. reumatol ; 55(1): 79-82, Jan-Feb/2015. tab, graf
Article in Portuguese | LILACS | ID: lil-744676

ABSTRACT

A síndrome de ativação macrofágica (SAM) é uma doença rara e potencialmente fatal, normalmente associada às doenças reumáticas crônicas, em especial a artrite idiopática juvenil. É incluída no grupo das formas secundárias de síndrome hemofagocítica, cujas outras causas podem ser as doenças linfoproliferativas e infecções. As manifestações clínicas e laboratoriais mais importantes são a febre não remitente, esplenomegalia, hemorragias, disfunção hepática, citopenias, hipoalbuminemia, hipertrigliceridemia e hiperferritinemia. O tratamento deve ser iniciado rapidamente, e a maioria dos casos responde bem aos corticosteroides e à ciclosporina (CSA). O vírus Epstein-Barr (EBV) é descrito como possível gatilho para muitos casos de SAM, especialmente naqueles em tratamento com bloqueadores do fator de necrose tumoral (TNF). Nos casos refratários ao tratamento convencional, etoposide (VP16) deve ser administrado, em associação com corticosteroides e CSA. Nosso objetivo foi descrever um caso raro de síndrome hematofagocítica provavelmente secundária à infecção pelo vírus Epstein-Barr (EBV), em paciente com artrite idiopática juvenil sistêmica, confirmada pelas manifestações clínicas e laboratoriais típicas, mielograma e sorologia positiva contra o EBV, que atingiu remissão completa após inclusão no protocolo de tratamento HLH-04.


Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein–Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemiconset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol.


Subject(s)
Humans , Female , Child , Arthritis, Juvenile/complications , Macrophage Activation Syndrome/etiology
11.
Korean Journal of Medicine ; : 525-534, 2015.
Article in Korean | WPRIM | ID: wpr-162285

ABSTRACT

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome involving defective apoptosis in which the pathways regulating the termination of immune and inflammatory responses are disrupted. Fever, cytopenia, splenomegaly, and hemophagocytosis are typical findings of this syndrome. HLH can be induced by genetic disorders (familial) or secondary causes. While familial HLH is rare, secondary causes include infection, autoimmune disease, and malignancy in adults. Adult onset HLH may be confused with or misdiagnosed as sepsis or macrophage activation syndrome due to similar clinical manifestations and laboratory findings. Consequently, it is difficult to diagnose HLH promptly to initiate adequate immunosuppressive treatment or chemotherapy. A pediatric HLH treatment protocol such as HLH-2004 or multi-agent chemotherapy can be given to adults after adjusting the drug dosage and type. After the initial treatment, refractory or reactivated patients should undergo allogenic hematopoietic stem cell transplantation as soon as possible to improve survival. Clinical trials should determine more suitable therapeutic options for adults with HLH.


Subject(s)
Adult , Apoptosis , Autoimmune Diseases , Clinical Protocols , Diagnosis , Drug Therapy , Fever , Hematopoietic Stem Cell Transplantation , Humans , Lymphohistiocytosis, Hemophagocytic , Macrophage Activation Syndrome , Sepsis , Splenomegaly
12.
Korean Journal of Medicine ; : 372-376, 2015.
Article in Korean | WPRIM | ID: wpr-216637

ABSTRACT

Macrophage activation syndrome (MAS) is a secondary hemophagocytic lymphohistiocytosis caused by autoimmune diseases, such as systemic lupus erythematosus (SLE). It is characterized by fever, cytopenia, coagulopathy, hepatosplenomegaly, elevated liver enzyme, and high ferritin, typically combined with hemophagocytic histiocyte proliferation in the bone marrow. Here, we report a case of MAS in a patient with SLE treated successfully by tocilizumab. She was transferred to our hospital due to persistent fever of unknown origin. Initial blood tests revealed cytopenia, elevated liver enzyme, and high ferritin. Bone marrow histology revealed the presence of hemophagocytic histiocytes. The patient was initially treated with high dose corticosteroids; however, fever and cytopenia were not controlled. Additional treatments with cyclosporine, intravenous immunoglobulin, and rituximab were applied consecutively, but the fever and cytopenia persisted. Symptom resolution was finally achieved following treatment with tocilizumab, resulting in rapid improved of fever, and resolution of pancytopenia within 2 months.


Subject(s)
Adrenal Cortex Hormones , Autoimmune Diseases , Bone Marrow , Cyclosporine , Ferritins , Fever , Fever of Unknown Origin , Hematologic Tests , Histiocytes , Humans , Immunoglobulins , Liver , Lupus Erythematosus, Systemic , Lymphohistiocytosis, Hemophagocytic , Macrophage Activation Syndrome , Macrophage Activation , Macrophages , Pancytopenia , Rituximab
13.
Article in English | WPRIM | ID: wpr-104099

ABSTRACT

Macrophage activation syndrome (MAS) is a rare complication in systemic lupus erythematosus (SLE) that can be triggered by infections. Due to the fact that MAS may mimic clinical features of underlying rheumatic disease, or be confused with an infectious complication, its detection can prove challenging. This is particularly true when there is an unknown/undiagnosed disease; and could turn into an even greater challenge if MAS and SLE are combined with a viral infection. A-14-year-old female came to the hospital with an ongoing fever for 2 weeks and a painful facial skin rash. Hepatomegaly, pancytopenia, increased aspartate aminotransferase, elevated serum ferritin and lactate dehydrogenase were reported. No hemophagocytic infiltration of bone marrow was reported. The patient was suspected for hemophagocytic lymphohistiocytosis. Her skin rashes were eczema herpeticum, which is usually associated with immune compromised conditions. With the history of oral ulcers and malar rash, positive ANA and low C3, C4 and the evidence of hemolytic anemia, she was diagnosed as SLE. According to the diagnostic guideline for MAS in SLE, she was diagnosed MAS as well, activated by acute HSV infection. After administering steroids and antiviral agent, the fever and skin rash disappeared, and the abnormal laboratory findings normalized. Therefore, we are reporting a rare case of MAS triggered by acute HSV infection as the first manifestation of SLE.


Subject(s)
Anemia, Hemolytic , Aspartate Aminotransferases , Bone Marrow , Exanthema , Female , Ferritins , Fever , Hepatomegaly , Humans , Kaposi Varicelliform Eruption , L-Lactate Dehydrogenase , Lupus Erythematosus, Systemic , Lymphohistiocytosis, Hemophagocytic , Macrophage Activation Syndrome , Macrophage Activation , Macrophages , Oral Ulcer , Pancytopenia , Rheumatic Diseases , Steroids
14.
Article in Korean | WPRIM | ID: wpr-66599

ABSTRACT

Macrophage activation syndrome (MAS) is a severe complication in patients with autoimmune disease. We should consider MAS in patients with autoimmune disease, who present with newly developed fever, and MAS needs proper management due to grave outcome. We report a case of MAS in a 15-year-old adolescent girl, who was newly diagnosed with systemic lupus erythematosus 1 month before the diagnosis of MAS. Her MAS was improved by intensive treatment, including etoposide.


Subject(s)
Adolescent , Autoimmune Diseases , Diagnosis , Etoposide , Female , Fever , Humans , Lupus Erythematosus, Systemic , Macrophage Activation Syndrome
15.
Article in Korean | WPRIM | ID: wpr-11340

ABSTRACT

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of childhood systemic inflammatory disorder, primarily systemic onset juvenile rheumatoid arthritis (SoJRA). It is characterized by pancytopenia, liver insufficiency, coagulopathy, and neurologic symptoms. The clinical manifestations are caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to cytokine overproduction including tumor necrosis factor-alpha (TNF-alpha). Methylprednisolone pulse therapy and cyclosporine A have made a considerable progress in the treatment of MAS. However, the mortality rate remains high suggesting the need of another therapeutic agent. Several cases of MAS successfully treated with TNF-alpha inhibitor (etanercept) have been reported. We report the first Korean case of MAS successfully treated with combination therapy of corticosteroid, cyclosporine A and etanercept.


Subject(s)
Arthritis, Juvenile , Cyclosporine , Hepatic Insufficiency , Immunoglobulin G , Macrophage Activation Syndrome , Macrophages , Methylprednisolone , Neurologic Manifestations , Pancytopenia , Receptors, Tumor Necrosis Factor , T-Lymphocytes , Tumor Necrosis Factor-alpha , Etanercept
16.
Iranian Journal of Pediatrics. 2011; 21 (4): 557-562
in English | IMEMR | ID: emr-137380

ABSTRACT

Pleuritic pain is not an unusual problem in children. Other concomitant symptoms should be considered for diagnostic approach in a child with pleuritic chest pain. In this report we discuss chest pain in a 6-year-old child with regard to other signs and symptoms. Finally, we found a rare life-threatening complication of juvenile systemic lupus erythematosus [JSLE] in our patient


Subject(s)
Humans , Male , Chest Pain/etiology , Chest Pain/diagnosis , Macrophage Activation Syndrome/etiology , Macrophage Activation Syndrome/physiopathology , Macrophages/metabolism
17.
Article in English | IMSEAR | ID: sea-145549

ABSTRACT

Hemophagocytic lymphohistiocytosis is a potentially fatal condition characterized by pathologic immune activation, which can complicate infections, childhood systemic rheumatologic diseases and malignancies. Here we report a case of reactive hemophagocytic lymphohistiocytosis [macrophage activation syndrome] complicating systemic onset juvenile idiopathic arthritis, which was treated successfully with dexamethasone and cyclosporine. Reactive hemophagocytic lymphohistiocytosis or macrophage activation syndrome should be considered in patients of juvenile idiopathic arthritis with prolonged fever of unknown origin and cytopenias. Early diagnosis with high index of suspicion and prompt, aggressive treatment are needed for successful outcomes.


Subject(s)
Adolescent , Arthritis, Juvenile/complications , Cyclosporine/therapeutic use , Dexamethasone/therapeutic use , Fever of Unknown Origin/drug therapy , Fever of Unknown Origin/etiology , Humans , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/drug therapy , Macrophage Activation Syndrome/complications , Macrophage Activation Syndrome/drug therapy , Male
18.
Article in Korean | WPRIM | ID: wpr-219039

ABSTRACT

Few cases of macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) during the acute febrile phase of Kawasaki disease (KD) have been reported. We report on a case of a 19 month-old girl with MAS or reactive HLH during the course of KD. Despite immunoglobulin and steroid therapy, she showed persistent fever with hepatosplenomegaly and evidence of hemophagocytosis in the bone marrow. A high index of suspicion for clinical features associated with MAS is necessary for KD patients in order to provide appropriate treatment.


Subject(s)
Bone Marrow , Fever , Humans , Immunoglobulins , Lymphohistiocytosis, Hemophagocytic , Macrophage Activation , Macrophage Activation Syndrome , Macrophages , Mucocutaneous Lymph Node Syndrome , Organic Chemicals
19.
Chinese Journal of Pediatrics ; (12): 806-811, 2006.
Article in Chinese | WPRIM | ID: wpr-349526

ABSTRACT

<p><b>OBJECTIVE</b>To review and analyze the clinical features, treatment, and outcome of macrophage activation syndrome (MAS) in children with systemic onset juvennil rheumatoid arthritis (SOJRA).</p><p><b>METHOD</b>Retrospective review and analysis were performed on cases with MAS from a prospectively collected database of children with SOJRA from the year of 2003 to 2006 in the Hospital.</p><p><b>RESULTS</b>Twenty four patients (21 boys, 3 girls) were diagnosed as having MAS with SOJRA. Mean age of the patients with MAS at diagnosis was 7 years, and the duration prior to diagnosis of MAS was 12 months. No trigger factors were found except in one case whose MAS was triggered by use of methotrexate and in another by parvovirus B19 infection. High grade fever, new onset hepatosplenomegaly and lymphadenopathy, pancytopenia, liver dysfunction were common clinical features in all the 24 cases (100%). Bleeding from skin, mucous membrane and gastrointestinal tract were noted in 9 cases (38%). Twelve (50%) cases had CNS dysfunction (high intracranial pressure, seizure and coma). Six cases (25%) developed ARDS. One patient suffered from renal damage. The laboratory test revealed elevated live enzymes and ferritin, decreased value of ESR, albumin, complete blood count and fibrinogen in all the 24 cases. Bone marrow examination supported the diagnosis of definite hemophagocytosis in the 24 cases. Lymph node biopsy was done for one case and histopathological examination showed that the node was full of activated macrophage. As to treatment, five cases only received high dose steroids (three of them died), 14 cases were treated with high dose steroids plus cyclosporine (one died), two were treated with steroids plus cyclosporine and etoposide (none died). The causes of deaths were ARDS and CNS involvement. In three of the cases who died, treatment was given up by their parents.</p><p><b>CONCLUSIONS</b>MAS is a rare and potentially fatal complication of SOJRA. Most of our patients were male. Bone marrow studies support the diagnosis. CNS involvement and ARDS were poor prognostic signs. Early diagnosis and aggressive therapy are essential.</p>


Subject(s)
Adolescent , Arthritis, Juvenile , Drug Therapy , Pathology , Child , Child, Preschool , Female , Humans , Infant , Macrophage Activation Syndrome , Drug Therapy , Pathology , Male , Retrospective Studies
20.
Chinese Journal of Pediatrics ; (12): 812-817, 2006.
Article in Chinese | WPRIM | ID: wpr-349525

ABSTRACT

<p><b>OBJECTIVE</b>Macrophage activation syndrome (MAS) is a rare but life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile idiopathic arthritis (SOJIA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study reviewed the data of MAS in 13 cases with SOJIA.</p><p><b>METHODS</b>Retrospective review was performed on the precipitating events, clinical manifestations, laboratory data, treatment, and outcome of macrophage activation syndrome in 13 children with SOJIA seen from 1996 to 2005.</p><p><b>RESULTS</b>Over the past 10 years the unit has had 90 new patients with SOJIA. Thirteen of those patients (14.4%) developed MAS during the course of their primary SOJIA, of whom ten were male. All patients were noted to have active SOJIA prior to developing MAS; 3 patients had medications, which were considered as trigger factors; 8 had infections prior to MAS, in two of them the infections were possible triggers. All the patients had high grade fever; 12 cases (92.3%) had hepatomegaly; 10 patients (76.9%) had coagulopathy, and eight patients (61.5%) had central nervous system dysfunction. The counts of platelet, white blood cells and the mean erythrocyte sedimentation rate fell dramatically in all patients; hyperferritinemia was identified in 8 patients, in 5 of whom serum ferritin (SF) was >or= 10,000 microg/L; in 8 (72.7%) of 11 cases fibrinogen was <or= 2.5 g/L; triglyceride (TG) was >or= 2.5 mmol/L in 9 (69.2%) of 13 cases.</p><p><b>CONCLUSION</b>MAS is a rare and potentially fatal complication of children with SOJIA. Primary disease activity, medications and infections preceding MAS were all important triggers. The strongest clinical discriminators were hepatomegaly, hemorrhages and central nervous system dysfunction. The strongest laboratory tests were decreased counts of platelet and white blood cells, decreased ESR and fibrinogen, dramatically increased SF and TG. It calls for the immediate treatments, particularly with cyclosporin A, which are often effective.</p>


Subject(s)
Arthritis, Juvenile , Drug Therapy , Pathology , Child , Child, Preschool , Female , Humans , Infant , Macrophage Activation Syndrome , Drug Therapy , Pathology , Male , Retrospective Studies
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