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      Síndrome hemofagocítico secundario en el recién nacido Translated title: Secondary hemophagocytosis syndrome in the newborn

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          Abstract

          El síndrome hemofagocítico secundario o reactivo es una entidad poco frecuente, de etiología multifactorial, que clínicamente se manifiesta como un cuadro grave y de alta letalidad. Se caracteriza por activación benigna de los macrófagos, asociada a infecciones virales, bacterianas, fúngicas o parasitarias, a inmunodeficiencias y a neoplasias. Se presenta el caso clínico de un neonato varón, de 17 días de vida, que presentó fiebre, manifestaciones catarrales y digestivas. Evolucionó clínicamente con ictericia, hepatoesplenomegalia, trombocitopenia y anemia. Se diagnosticó un citomegalovirus en la orina. Egresó vivo a los 68 días de vida, con regresión del cuadro clínico.

          Translated abstract

          The secondary or reactive hemophagocytosis syndrome is an uncommon entity clinically manifested by a severe clinical picture and a high mortality rate. It is characterized by a macrophages benign activation, associated with viral, bacterial, fungoid or parasitic infections and immunodeficiencies and neoplams. This is the clinical case of a 17 days male neonate with fever, suffering from cold and digestive manifestations who clinically evolving with jaundice, hepatosplenomegaly and anemia. A cytomegalovirus was diagnosed in urine. Was discharged being alive at 68 days with a regression of clinical picture.

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          Multifactorial risk analysis of bone marrow histiocytic hyperplasia with hemophagocytosis in critically ill medical patients--a postmortem clinicopathologic analysis.

          Systematic studies of the prevalence and risk factors of histiocytic hyperplasia with hemophagocytosis (HHH) in critically ill patients are lacking. The aim of our study was a) to determine the frequency and intensity of HHH in the bone marrow of patients who died on the medical intensive care unit; b) to analyze morphologic bone marrow changes; and c) to identify possible risk factors and their interactions in the pathogenesis of HHH. A retrospective observational analysis of clinical data and autopsy findings including histologic and immunohistological analysis of bone marrow to characterize cellularity, siderosis, hemophagocytosis, and T-cell infiltrates. The medical intensive care unit of a university hospital. Patients were 107 consecutive patients who died and underwent autopsy. None. HHH was identified in 69 of the 107 patients (64.5%). Moderate to severe HHH was present in 35 of the 107 bone marrows. Univariate risk factor analysis showed that HHH was associated with various intrinsic and extrinsic factors. However, multivariate analysis identified the intensity of therapeutic interventions--represented by the Therapeutic Intervention Scoring System--as the only positive, and cardiovascular disease as the only significant negative, predictor of HHH (p <.05). Routine laboratory tests were of no value in predicting the presence of HHH. The intensity of HHH correlated significantly with siderosis and T-cell infiltrates (p <.05) but not with bone marrow cellularity. HHH is common in medical intensive care unit nonsurvivors. Treatment intensity and a noncardiovascular cause of death are predictors of HHH. Sepsis and blood transfusion may have a synergistic effect on the triggering of HHH. HHH in bone marrow is associated with enhanced T-cell infiltrates, suggesting that T cells may play an important role in its mediation.
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            INFECTION- AND MALIGNANCY-ASSOCIATED HEMOPHAGOCYTIC SYNDROMES

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              Haemophagocytic syndrome associated with infections.

              Haemophagocytic syndromes (HS) are the clinical manifestation of an increased macrophagic activity with haemophagocytosis. Pathophysiology is related to a deregulation of T-lymphocytes and excessive production of cytokines. The main clinicobiological features are fever, hepatosplenomegaly, adenopathies, skin rash, neurological features, cytopenias, hypertriglyceridaemia, hyperferritinaemia and coagulopathy. Diagnosis is based on examination of the bone marrow which shows benign histiocytes actively phagocytosing haemopoietic cells. Acquired HS are mostly associated with an underlying disease such as immunodeficiency, haematological neoplasias and autoimmune diseases. Infection-associated HS was originally described by Risdall in 1979, in viral disease. Since the initial description HS has also been documented in patients with bacterial, parasitic or fungal infections. Epstein-Barr virus (EBV) is the causative agent in most cases. In EBV-associated HS, which sometimes has a fatal course, unregulated T-cell reaction or uncontrolled B-cell proliferation may release cytokines. Management of HS consists of early diagnosis, careful screening for, and prompt treatment of, infections and detection and therapy of any underlying disease. Prognosis of infection-associated haemophagocytic syndrome (IAHS) is better than that in other types of secondary HS. Management of cytokine imbalance should be useful to improve the outcome and reduce the mortality rate in these cases.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                ped
                Revista Cubana de Pediatría
                Rev Cubana Pediatr
                Editorial Ciencias Médicas (Ciudad de la Habana )
                1561-3119
                June 2010
                : 82
                : 2
                : 0
                Affiliations
                [1 ] Hospital Pediátrico William Soler Cuba
                Article
                S0034-75312010000200012
                d9f78dc3-26cf-4526-b325-8fbff65745a5

                http://creativecommons.org/licenses/by/4.0/

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                SciELO Cuba

                Self URI (journal page): http://scielo.sld.cu/scielo.php?script=sci_serial&pid=0034-7531&lng=en
                Categories
                PEDIATRICS

                Pediatrics
                Newborn,cytomegalovirus,hemophagocytosis sindrome,Recién nacido,citomegalovirus,síndrome hemofagocítico

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