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Background: Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HL) is an immune hyperactivation of multifactorial etiology, characterized by excessive activation of lymphocytes and macrophages, as well as numerous pro-inflammatory cytokines. It has a non-specific and highly variable clinical presentation, with splenomegaly being one of the clinical manifestations. Due to its nature, it can manifest during childhood or adult life, which is why it is a disease of diagnostic and therapeutic complexity. Clinical case: 38-year-old male patient without comorbidities, who presented with abdominal pain, choluria, fever > 38 °C and diaphoresis of more than 10 days of evolution. A bone marrow aspirate was performed as part of the diagnostic approach with data compatible with hemophagocytosis and cytopenias. The immunosuppressive management did not show the expected response, which is why an open splenectomy was performed as the last therapeutic option with adequate hematological control. A documentary review of the disease was carried out, and of the therapeutic options, emphasizing surgical management in case of refractoriness to medical treatment. Conclusions: Splenectomy increases the overall survival rate and the time free of HL progression, even though there are still no studies to determine with certainty the ideal time to perform a splenectomy in patients with pancytopenia without splenomegaly who suffer from hemophagocytic syndrome. [ABSTRACT FROM AUTHOR]

Adult-onset Still's disease (AOSD) and Schnitzler syndrome (SchS) are rare, not fully understood, multisystemic and autoinflammatory disorders, with a challenging differential diagnosis. The authors report the case of an elderly man with unexplained fever, arthralgia, weight loss, spleen enlargement, lymphadenopathy, anemia, hyperferritinemia and IgG monoclonal gammopathy. Autoimmunity, infection, haematological disease and malignancy were excluded. The clinical spectrum fulfilled both AOSD diagnostic criteria and IgG-variant SchS Strasbourg criteria. Symptom resolution was achieved with immunosuppressive therapy, supporting the diagnosis of an autoinflammatory disorder, a diagnostic challenge for the medical team emerging as an unexpected cause of fever in the elderly. [ABSTRACT FROM AUTHOR]

The current study aimed at characterizing the dynamics of SARS-CoV-2 nucleocapsid (N) antigenemia in a cohort of critically ill adult COVID-19 patients and assessing its potential association with plasma levels of biomarkers of clinical severity and mortality. Seventy-three consecutive critically ill COVID-19 patients (median age, 65 years) were recruited. Serial plasma (n = 340) specimens were collected. A lateral flow immunochromatography assay and reverse-transcription polymerase chain reaction (RT-PCR) were used for SARS-CoV-2 N protein detection and RNA quantitation and in plasma, respectively. Serum levels of inflammatory and tissue-damage biomarkers in paired specimens were measured. SARS-CoV-RNA N-antigenemia and viral RNAemia were documented in 40.1% and 35.6% of patients, respectively at a median of 9 days since symptoms onset. The level of agreement between the qualitative results returned by the N-antigenemia assay and plasma RT-PCR was moderate (k=0.57; p < 0.0001). A trend towards higher SARS-CoV-2 RNA loads was seen in plasma specimens testing positive for N-antigenemia assay than in those yielding negative results (p=0.083). SARS-CoV-2 RNA load in tracheal aspirates was significantly higher (p<0.001) in the presence of concomitant N-antigenemia than in its absence. Significantly higher serum levels of ferritin, lactose dehydrogenase, C-reactive protein, and D-dimer were quantified in paired plasma SARS-CoV-2 N-positive specimens than in those testing negative. Occurrence of SARS-CoV-2 N-antigenemia was not associated with increased mortality in univariate logistic regression analysis (odds ratio, 1.29; 95% confidence interval, 0.49-3.34; p = 0.59). In conclusion, SARS-CoV-2 N-antigenemia detection is relatively common in ICU patients and appears to associate with increased serum levels of inflammation and tissue-damage markers. Whether this virological parameter may behave as a biomarker of poor clinical outcome awaits further investigations. [ABSTRACT FROM AUTHOR]

A prospective study was carried out to determine if hyperferritinemia is a predictive factor for immunosuppression in patients with acquired immunodeficiency syndrome (AIDS). The population consisted of 40 patients hospitalized at the University Hospital of Maracaibo (Hospital Uni-versitario de Maracaibo), from January to October, 2010. Twenty-seven (67.50%) patients were male and 13 (32.50%) were female. 22.50% had a T CD4+lymphocyte count between 200-400 cells/mm3 with a mean ferritin of 144.2 ± 127.1 ng/mL; 77.50% had a T CD4+ lymphocyte count of <200 cells/mm3 and a ferritin average of 1100.0 ± 984.7 ng/mL (p = 0.01). An inverse negative correlation was found between high ferritin levels and low T CD4+ lymphocyte count (r = 0.3135, p = 0.030), low numbers of leukocytes (r = o, 7458 p = 0.012), low levels of protein (r = 0.5814, p = 0.01), and a directly proportional relation with the increase of ESR (r = 0.7422, p = 0.001). In patients who died, the mean ferritin level (1180.0 ± 1,072) statistically (p = 0.018) was higher compared with the average ferritin level (474 ± 440.2) of patients who survived. Conclusions are that these results are sufficiently relevant to take hyperferritinemia into account as a prognostic factor for the immunosuppression of diagnosed AIDS patients. [ABSTRACT FROM AUTHOR]