Secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition caused by the hyperactivation of macrophages and T-cells, triggered by infection, malignancy, or underlying rheumatological conditions. It rarely presents as a first manifestation of a rheumatological condition. Macrophage activation syndrome (MAS) is secondary HLH associated with underlying hematological conditions. Here, we present a case of a previously healthy 29-year-old female who was admitted with fever, rash, and pancytopenia, found to have HLH, and a workup revealed underlying systemic lupus erythematosus (SLE). She was successfully treated with dexamethasone, etoposide, and belimumab, with complete recovery of her symptoms. This case highlights the importance of a thorough evaluation of rheumatological conditions in all patients with HLH despite their previous medical history and the use of belimumab for SLE.

UNASSIGNED: Serum ferritin (SF) is clinically found to be elevated in many disease conditions, and our research examines serum ferritin in patients with acute kidney injury (AKI) and its implication on the risk of short-term mortality in AKI.
UNASSIGNED: Data were extracted from the Medical Information Mart for Intensive Care IV 2.2 (MIMIC-IV 2.2) database. Adult patients with AKI who had serum ferritin tested on the first day of ICU admission were included. The primary outcome was 28-day mortality. Kaplan-Meier survival curves and Cox proportional hazards models were used to test the relationship between SF and clinical outcomes. Subgroup analyses based on the Cox model were further conducted.
UNASSIGNED: Kaplan-Meier survival curves showed that a higher SF value was significantly associated with an enhanced risk of 28-day mortality, 90-day mortality, ICU mortality and hospital mortality (log-rank test: p < 0.001 for all clinical outcomes). In multivariate Cox regression analysis, high level of SF with mortality was significantly positive in all four outcome events (all p < 0.001). This result remains robust after adjusting for all variables. Subgroup analysis of SF with 28-day mortality based on Cox model-4 showed that high level of SF was associated with high risk of 28-day mortality in patients regardless of the presence or absence of sepsis (p for interaction = 0.730). Positive correlations of SF and 28-day mortality were confirmed in all other subgroups (p for interaction>0.05).
UNASSIGNED: High level of SF is an independent prognostic predictor of 28-day mortality in patients with AKI.

BACKGROUND: The distribution of causes of hyperferritinemia in international series is heterogeneous. Also, the association between ferritin and prognosis is controversial. This study aims to describe the diagnosis associated with hyperferritinemia in a retrospective cohort at an academic healthcare network in Chile.
METHODS: A retrospective review of adult patients admitted to our academic medical center from June 2014 to February 2017 with ferritin ≥3,000 ng/mL. All patients were classified into nine diagnostic categories. Then, the association between ferritin level and disease category, as well as mortality, was evaluated.
RESULTS: Ninety-nine patients were identified. The mean age was 50.8 ± 19.9 years, 54.5% were men. The most frequent categories were \"inflammatory and autoimmune diseases\" (21.2%) and \"hematological malignancies\" (19.2%). The average ferritin was 10,539 ± 13,016.9 ng/mL, while the higher mean was 16,707 ng/mL in the \"inflammatory and autoimmune diseases\" category. There was a statistically significant association between the ferritin value and age but not between ferritin and diagnostic categories. In the group over 50, hematologic neoplasms (19%) and infections (19%) were more frequent. In those under 50, inflammatory and autoimmune diseases were more frequent (26.8%). There was no association between the ferritin level and mortality at 1, 3, and 12 months.
CONCLUSIONS: The most frequent categories were \"inflammatory and autoimmune diseases\" and \"hematological malignancies\", but ferritin level was similar in both. Further research could validate a prognostic role.

OBJECTIVE: To investigate the correlation factors of complete clinical response in idiopathic inflammatory myopathies (IIMs) patients receiving conventional treatment.
METHODS: Patients diagnosed with IIMs hospitalized in Peking University People\'s Hospital from January 2000 to June 2023 were included. The correlation factors of complete clinical response to conventional treatment were identified by analyzing the clinical characteristics, laboratory features, peripheral blood lymphocytes, immunological indicators, and therapeutic drugs.
RESULTS: Among the 635 patients included, 518 patients finished the follow-up, with an average time of 36.8 months. The total complete clinical response rate of IIMs was 50.0% (259/518). The complete clinical response rate of dermatomyositis (DM), anti-synthetase syndrome (ASS) and immune-mediated necrotizing myopathy (IMNM) were 53.5%, 48.9% and 39.0%, respectively. Fever (P=0.002) and rapid progressive interstitial lung disease (RP-ILD) (P=0.014) were observed much more frequently in non-complete clinical response group than in complete clinical response group. The aspartate transaminase (AST), lactate dehydrogenase (LDH), D-dimer, erythrocyte sedimentation rate (ESR), C-reaction protein (CRP) and serum ferritin were significantly higher in non-complete clinical response group as compared with complete clinical response group. As for the treatment, the percentage of glucocorticoid received and intravenous immunoglobin (IVIG) were significantly higher in non-complete clinical response group than in complete clinical response group. Risk factor analysis showed that IMNM subtype (P=0.007), interstitial lung disease (ILD) (P=0.001), eleva-ted AST (P=0.012), elevated serum ferritin (P=0.016) and decreased count of CD4+T cells in peripheral blood (P=0.004) might be the risk factors for IIMs non-complete clinical response.
CONCLUSIONS: The total complete clinical response rate of IIMs is low, especially for IMNM subtype. More effective intervention should be administered to patients with ILD, elevated AST, elevated serum ferritin or decreased count of CD4+T cells at disease onset.

BACKGROUND: Adult hemophagocytic lymphohistiocytosis (HLH) is a rare disease with a dismal prognosis. Early diagnosis and prompt management are necessary for improved outcomes.
METHODS: This multicenter retrospective study investigated the etiologies, survival, and prognostic factors of HLH, including the utility of HLH-2004 criteria and HScore in real-life clinical practice.
RESULTS: A total of 147 HLH patients were identified by using a combination of hemophagocytosis identification in bone marrow and the HLH-related international classification disease-10. A total of 116 (78.9%) patients fulfilled the HLH diagnosis by HScore, while 91 (61.9%) patients fulfilled 5 of 8 HLH-2004 criteria. In Thailand, the clinical application of HLH-2004 criteria needed to be reduced from 8 to 6 due to a lack of sCD25 and natural killer cell activity tests. Using the adapted HLH-2004 with a cutoff value of 4 resulted in 132 (89.9%) cases meeting the diagnostic criteria. Among these 132 confirmed HLH patients by using adapted HLH-2004, HLH was triggered by infection (29.5%), autoimmune disease (12.9%), malignancy (40.9%), and unknown cause (16.7%). Median overall survival of HLH patients was extremely short (67 days). Ferritin >6,000 μg/L, HLH from infection, malignancy, and unknown etiology were demonstrated as independent prognostic factors for inferior survival (hazard ratio [HR] 2.47; 95% confidence interval [CI] 1.39-4.37, HR 4.69; 95% CI 1.38-15.92, HR 6.09; 95% CI 1.84-20.14, and HR 6.02; 95% CI 1.64-22.05, respectively).
CONCLUSIONS: Ferritin is a helpful biomarker for HLH diagnosis and prognostic prediction. Autoimmune disease-triggered HLH has favorable outcomes. Future prospective study is required to verify the use of the adapted HLH-2004 criteria.

OBJECTIVE: To determine the incidence of hyperferritinemia in VLBW infants, and its association with neonatal morbidity.
METHODS: Prospective cohort study in a tertiary-level hospital in Bangkok, from March 2022 to January 2023. Serum ferritin (SF) was measured in VLBW infants at one month and repeated monthly for those with hyperferritinemia (SF > 300 ng/mL).
RESULTS: Gestational age and birth weight were 29.7 ± 2.4 weeks (mean ± SD) and 1100 g (IQR, 830, 1340). Hyperferritinemia was identified in 30.1% (95% CI, 20.8-41.4). After adjustment, only packed red cell transfusion >15 mL/kg was associated with hyperferritinemia (RR 3.1; 95% CI, 1.5-6.4). All elevated SF levels returned to normal within four months. Hyperferritinemia was associated with severe bronchopulmonary dysplasia (RR 2.3, 95% CI, 1.0-5.4) and retinopathy of prematurity (RR 3.5, 95% CI, 1.4-8.6).
CONCLUSIONS: Hyperferritinemia is common among our VLBW infants, particularly after transfusion, and is associated with severe BPD and ROP.

UNASSIGNED: Angiotensin-converting enzyme 2 (ACE2) is not only the entry route of SARS-CoV-2 infection but also triggers a major mechanism of COVID-19 aggravation by promoting a hyperinflammatory state, leading to lung injury, hematological and immunological dysregulation. The impact of ACE2 inhibitors on the course of COVID-19 is still unclear. The effect of ACE2 inhibitors on the course of acute respiratory distress syndrome (ARDS) during COVID-19 and other severe respiratory infections in conditions of hyperferritinemia (HF) was investigated.
UNASSIGNED: A cohort study of critically ill patients with COVID-19 and other respiratory diseases (widespread infection, pneumonia) who underwent treatment in The Critical Care Unit of the First University Clinic (Tbilisi, Georgia) during the 2020-2021 years was conducted. The impact of the ACE2 inhibitors on the course of the ARDS developed during COVID-19 and other severe respiratory infections in conditions of different severity of HF was evaluated.
UNASSIGNED: In COVID-19-infected (I) and uninfected (II) patients with ARDS, ACE2 inhibitors reduce the levels of Ang II, C reactive protein (CRP) and D-dimer (I: from 1508.07  ±  26.68 to 48.51  ±  24.35, from 233.92  ±  13.02 to 198.12  ±  11.88, from 7.88  ±  0.47 to 6.28  ±  0.43; II: from 1000.14  ±  149.49 to 46.23  ±  88.21, 226.48  ±  13.81 to 183.52  ±  17.32, from 6.39  ±  0.58 to 5.48  ±  0.69) at moderate HF and Ang II, CRP levels (I: from 1845.89  ±  89.37 to 49.64  ±  51.05, from 209.28  ±  14.41 to 175.37  ±  9.84; II: from 1753.29  ±  65.95 to 49.76  ±  55.74, 287.10  ±  20.50 to 214.71  ±  17.32) at severe HF, reduce interleukin-6 (IL-6) expression at moderate HF (I: from 1977.23  ±  354.66 to 899.36  ±  323.76) and cause reduction of pCO2 index at severe HF (I: from 69.80  ±  3.22 to 60.44  ±  2.20) in COVID-19-infected patients.
UNASSIGNED: Study results show that the ACE2 inhibitors play an important role in the regulation of inflammatory processes in both COVID-19-infected and uninfected patients with ARDS. ACE2 inhibitors decrease immunological disorders, inflammation, and lung alveoli dysfunction, especially in COVID-19-infected patients.

OBJECTIVE: Hyperferritinemia in the critical phase of dengue infections may correlate with severe dengue ( sd ) disease, and our primary objective was to examine the association between ferritin level on day 1 of PICU admission and 2009 World Health Organization (WHO) criteria for sd . Our secondary objective was outcome in relation to care. It is unclear whether immunomodulatory therapy during the critical phase may restore immune homeostasis and mitigate disease severity.
METHODS: Retrospective cohort study of children with dengue 1 month to 16 years old with admission ferritin greater than or equal to 500 ng/mL requiring PICU admission. Demographics, clinical, and laboratory parameters, presence of the 2009 WHO sd criteria and outcomes were analyzed. Immunomodulatory therapy was used when there was persistent hyperinflammation beyond the critical phase of plasma leakage.
METHODS: None.
RESULTS: Fifty-five patients were admitted in the critical phase of dengue with median (interquartile range) ferritin levels of 8,105 ng/mL (2,350-15,765 ng/mL). Patients with at least one WHO sd category had higher ferritin levels compared to those without any sd criteria, with the highest levels in eight patients with all three sd categories. In our cohort of 55, 52 patients (94%) recovered with standard supportive therapy. Recovery was associated with decreased ferritin levels that occurred in parallel with improved circulation and platelet counts; this included 22 of 24 patients with admission ferritin levels greater than or equal to 10,000 ng/mL and two with ferritin greater than 1,00,000 ng/mL. Immunomodulation was used in three patients with unremitting fever, persistent hyperferritinemia, and progressive multiple organ dysfunction beyond the critical phase, of whom two died.
CONCLUSIONS: Hyperferritinemia in the critical phase of sd is associated with the number of 2009 WHO sd criteria present. Our data also indicate that many patients with sd recover well with supportive care.

UNASSIGNED: Ferritin has been recognized as a predictor of severity among Coronavirus-19 disease (COVID-19) patients. Studies have shown higher levels of ferritin in patients with COVID-19 than in healthy children. Patients with transfusion-dependent thalassemia (TDT) basically have high ferritin level due to iron overload. It is uncertain whether serum ferritin level in these patients is associated with COVID-19 infection.
UNASSIGNED: To evaluate ferritin levels in TDT with COVID-19 before, during, and after the course of infection.
UNASSIGNED: This retrospective study enrolled all TDT children with COVID-19 infection that were hospitalized in Ulin General Hospital Banjarmasin during the COVID-19 pandemic (March 2020 to June 2022). Data were collected from medical records.
UNASSIGNED: There were 14 patients included in this study, 5 patients had mild symptoms and 9 patients were asymptomatic. The mean of hemoglobin level upon admission was 8.1 ± 3 g/dL and serum ferritin level were 5148.5 ± 2651.8 ng/mL. The average serum ferritin level during COVID-19 infection was 2373.2 ng/mL higher than before infection and then decreased by 952.4 ng/mL after infection. We found no association of increasing serum ferritin with patients\' symptoms (p = 0.27). The severity of anemia also was not correlated with the presentation of COVID-19 infection (p = 0.902).
UNASSIGNED: Serum ferritin levels in TDT children may not reflect disease severity or predict poor outcomes during COVID-19 infection. However, the presence of other co-morbid conditions/confounders warrants cautious interpretation.

UNASSIGNED: Mucormycosis is a serious fungal infection associated with uncontrolled diabetes and immunocompromised patients. This angioinvasive infection emerged as a post-covid complication worldwide especially in developing countries. Due to the common socio-demographic status of South Asian countries, we expected a surge in mucormycosis cases in Pakistan. This study aims to observe the frequency and survival of Covid associated mucormycosis patients at tertiary care hospitals in Pakistan during the third wave of Covid-19 in 2021.
UNASSIGNED: In this retrospective study, we collected the data of clinically and histopathologically confirmed cases of rhino-occipito-cerebral mucormycosis from three tertiary care hospitals of Lahore. These cases were analysed for history of Covid-19 and other associated comorbidities using SPSS28. History of steroid medication was also taken. Data were retrieved from May to July 2021 after the approval from the ethical review board.
UNASSIGNED: Out of the total 43 reported patients of mucormycosis in the set time frame only 22 cases had a history of Covid-19. The mean age was 50 ± 13.27 years with slight male predilection (60%). Diabetes mellitus was the most common comorbidity (88.4%) and all the patients with covid associated mucormycosis (CAM) had taken corticosteroid regimen for covid management (p < 0.0001). The survival of the patient was not significantly different between CAM and non-CAM patients of Mucormycosis (p = 0.747).
UNASSIGNED: Covid-19 and mucormycosis make a lethal duo against the weakened health system of Pakistan. This problem can be prevented by avoiding nonjudicial use of corticosteroids and proper diabetes control program following Covid-19 infection. Furthermore, large-scale epidemiological studies should be carried out to evaluate the true burden of Mucormycosis in the population.