Current approaches to diagnosing and treating macrophage activation syndrome in children with rheumatic diseases

Костик, М. М.; Дубко, М. Ф.; Масалова, В. В.; Snegireva, Ludmila; Чикова, И. А.; Исупова, Е. А.; Калашникова, О. В.; Chasnyk, Vyacheslav

doi:10.14412/1996-7012-2015-1-55-59

Cited by 6 publications

(2 citation statements)

References 16 publications

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“…Обнаружено также, что при ВГФС частота спленомегалии, уровень триглицеридов, ферритина, аланин-(АлАТ) и аспартатаминотрансферазы (АсАТ) статистически значимо выше, чем при сепсисе, а уровень гликозилированного ферритина (%ГФ), фибриногена, лейкоцитов, нейтрофилов и тромбоцитов -ниже. Приведены медианы (1-3-й квартиль) значений в группах пациентов с ВГФС и сепсисом соответственно: триглицериды (ммоль/л) -3,1 (2,3-3,8) и 1,5 (0,8-2,7), общий ферритин (нг/мл) -7170 (3159,2-12551,0) и 1274 (559,0-3041,5), %ГФ -26,5 (16,3) и 54,5 (37,8), фибриноген (г/л) -2,8 (1,4-4,4) и 5,3 (2,8), АлАТ (МЕ/л) -50 (20-102) и 30 (15,5), АсАТ (МЕ/л) -66 (40,6) и 36 (24,0), лейкоциты (×10 9 /л) -3,7 (2,1-5,5) и 8,9 (6,(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)5), тромбоциты (×10 9 /л) -56 (25,2-93,5) и 157 (97-308). По данным ROC-анализа, площадь под кривой для уровня нейтрофилов составила 0,88, общего ферритина, %ГФ, лейкоцитов и тромбоцитов -0,85, триглицеридов -0,74, фибриногена -0,71, натрия -0,65, АлАТ и АсАТ -0,61.…”

Section: клиническая онкогематологияunclassified

“…Наиболее частыми причинами ВГФС бывают лимфоидные опухоли, инфекции (вирус Эпштейна-Барр, лейшманиоз и др.) и системные ревматологические процессы [2,3]. Патофизиологическую основу ГФС составляет дефект иммунной системы, из-за которого под действием пускового фактора происходит избыточная активация цитотоксических лимфоцитов, Вторичный гемофагоцитарный синдром и сепсис естественных киллеров и макрофагов с последующим развитием гиперцитокинемии, тяжелого тканевого повреждения, полиорганной недостаточности, цитопении, вторичной инфекции.…”

Section: Introductionunclassified

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Clinical and Laboratory Characteristics and Differential Diagnosis between Secondary Hemophagocytic Syndrome and Sepsis

Потапенко¹,

Первакова²,

Титов³

et al. 2019

Клиническая онкогематология

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Актуальность. Вторичный гемофагоцитарный синдром (ВГФС) и сепсис при всей схожести клинических проявлений и лабораторных параметров принципиально различаются по применяемым при этих заболеваниях методам лечения. При ВГФС лечение направлено на достижение иммуносупрессии, а при сепсисе проводится противоинфекционная терапия. Для выбора правильной лечебной тактики необходима быстрая дифференциальная диагностика между заболеваниями. Цель. Поиск и анализ критериев дифференциальной диагностики ВГФС и сепсиса. Материалы и методы. Проанализированы данные 102 пациентов: 55-с ВГФС (медиана возраста 60 лет, диапазон 18-81 год) и 47-с сепсисом (медиана возраста 60 лет, диапазон 18-89 лет). Диагноз ВГФС установлен на основании критериев HLH-2004 и H-Score. Сепсис верифицирован при документированном очаге

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Section: клиническая онкогематологияunclassified

Section: Introductionunclassified

Clinical and Laboratory Characteristics and Differential Diagnosis between Secondary Hemophagocytic Syndrome and Sepsis

Потапенко¹,

Первакова²,

Титов³

et al. 2019

Клиническая онкогематология

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Secondary hemophagocytic syndrome in adult patients. Study of 91 patients

et al. 2020

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Background. Secondary hemophagocytic lymphohystiocytosis (sHLH) is a hyperinflammatory reaction provoked by some trigger (cancer, autoimmune or infection). The majority of affected patients are at high risk of fatal multiple organ failure without getting immunsupressive treatment.Objective. Clinical and laboratory profile of sHLH patients.Materials and methods. Retrospective study included clinical, instrumental and lab data from the 91 patients followed between June 2009 and June 2019. Diagnosis sHLH had been based on HLH-2004 and H-Score criteria. The analyzed parameters had been fever chart, liver and spleen enlargement, changes in the bone marrow; values levels of glutamic pyruvic transaminase, serum glutamic oxaloacetic transaminase, alkaline phosphatase, bilirubin, triglycerides, total ferritin with percentage of glycosylation. All patients with rheumatic disorders or malignancies had received either immunosuppressive or cytotoxic therapy. Febrile patients received anti-infective treatment according to the local routine protocols.Results. The data from 91 patients (41 male and 50 female) had been analyzed. Median age was 58 (2–90) years. The sHLH trigger-diseases spectrum included leukemia/lymphoma (n = 52), infection diseases (n = 11), autoimmune disorders (n = 5), allogenic bone marrow transplantation (n = 13), unidentified (n = 10). A fever with an unknown origin and refractory to antibacterial treatment had been observed in 87 (96 %) patients. Morphological hemophagocytic evidences in the bone marrow had been found in 83 %. Breath shortening, liver failure, neurologic disturbances, systemic effusions, rash, heart failure had been registered in 83 % patients. Detected splenomegaly presented in 56 %. Laboratory changes, median were as following: serum glutamic-pyruvic transaminase (alanine aminotransferase, SGPT) – 92 (39.2–1060.8) IU/L; serum glutamic oxaloacetic transaminase (aspartate aminotransferase, SGOT) – 105 (40–4177) IU/L; alkaline phosphatase – 225 (120.9–989) IU/L; bilirubin – 50.5 (22–559) µmol/L; triglycerides – 3.2 (1.95–8.6) mmol/L; total ferritin – 10000 (597–255000) ng/mL with glycosylation percentage – 20.45 (0–37.8) %. 71 patients received various of HLH-directed therapy courses. The overall survival rate was 27 %, median follow-up – 540 days.Conclusion. The main clinical and instrumental findings in sHLH are fever, refractory to anti-infective treatment, elevation of transaminases, serum alkaline phosphatase, triglycerides, total ferritine with low glycosylated fraction. Early diagnosing and immunesupression are the main factors of survival.

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Hemophagocytic syndrome associated with leishmaniasis: case report

et al. 2022

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Secondary hemophagocytic syndrome (sHLH) is a hyperinflammatory reaction which manifests with fever, cytopenia and organ damage. possible causes of sHLH include leishmaniasis. The article describes a clinical case of sHLH in patient with visceral leishmaniasis. A female 30 years old developed hectic daily fever up to 40 °C, pancytopenia, cytolytic syndrome, hyperferritin- and hypertriglyceridemia, immunoglobulin G to capsid antigens of the Epstein-Barr virus, enlarged liver and spleen a one and a half month after returning from Spain. based on the HLH-2004 and H-Score criteria, a sHLH was diagnosed, presumably associated with the Epstein-Barr virus. Immunosuppressive treatment with dexamethasone, cyclosporin-A and etoposide was started under the HLH-2004 program. Apyrexia, reduction of splenomegaly and resolution of cytolysis were achieved. The fever resumed 20 days after the start of chemotherapy, the spleen enlarged again, and therefore a diagnostic splenectomy was performed. Morphological analysis of the removed spleen revealed leishmania. After amphotericin-B therapy, the patient recovered. Chemotherapy of sHLH led to a temporary improvement for a period sufficient to verify the diagnosis and conducting of successful treatment.

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Current approaches to diagnosing and treating macrophage activation syndrome in children with rheumatic diseases

Cited by 6 publications

References 16 publications

Clinical and Laboratory Characteristics and Differential Diagnosis between Secondary Hemophagocytic Syndrome and Sepsis

Clinical and Laboratory Characteristics and Differential Diagnosis between Secondary Hemophagocytic Syndrome and Sepsis

Secondary hemophagocytic syndrome in adult patients. Study of 91 patients

Hemophagocytic syndrome associated with leishmaniasis: case report

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