• Global Team Science Award Lupus Research Alliance
• 2R01-AR-065538 NIAMS NIH HHS
• R01-AR-075906 NIAMS NIH HHS
• R01-AR-073201 NIAMS NIH HHS
• P30-AR-070253 NIAMS NIH HHS

• immunoglobulins
• rheumatology
• sepsis

• Hyperferritinemic sepsis
• Macrophage activation syndrome
• Macrophage activation-like syndrome
• Multiple organ dysfunction syndrome
• Sepsis

• Humans
• Multiple Organ Failure/etiology
• Multiple Organ Failure/immunology
• Sepsis/immunology
• Sepsis/complications
• Cytokine Release Syndrome/immunology
• Cytokine Release Syndrome/etiology
• Lymphohistiocytosis, Hemophagocytic/immunology
• Lymphohistiocytosis, Hemophagocytic/etiology
• Macrophage Activation Syndrome/immunology
• Macrophage Activation Syndrome/etiology
• Killer Cells, Natural/immunology

This is a case analysis of a 73-year-old Chinese man admitted to the cardiac intensive care unit (ICU) with fever and general pain. Based on the patient’s initial condition of multi-organ function impairment and increased serum ferritin, and after a series of examinations, the patient was diagnosed with Klebsiella pneumonia-induced hemophagocytic lymphohistiocytosis (HLH). Meropenem and dexamethasone were used in combination to treat the patient, and the results were very successful. In this case report, it is further suggested that Klebsiella pneumoniae is a possible trigger of HLH, and a combination of antibiotics and corticosteroids can be effective in treating HLH. It is also recommended that doctors in the ICU of each department should pay attention to the role of hyperferritinemia in the diagnosis of HLH, and ICU admission teams should include ferritin in their monitoring.

• Klebsiella pneumoniae
• ferritin
• hemophagocytic lymphohistiocytosis
• hemophagocytic syndrome
• hyperferritinemia
• intensive care unit

• 血液病
• 肿瘤性疾病
• 静脉注射用免疫球蛋白
• 专家共识
• 儿童
• hematological disease
• neoplastic disease
• intravenous immunoglobulin
• expert consensus
• child

• Child
• China
• Consensus
• Hematologic Neoplasms/drug therapy
• Hematopoietic Stem Cell Transplantation
• Humans
• Immunoglobulins, Intravenous/therapeutic use

Secondary haemophagocytic lymphohistiocytosis (sHLH) is a rare life-threatening condition mainly associated with underlying infections, malignancies, and autoimmune or immune-mediated diseases.

To analyse all sHLH cases that were diagnosed and managed under real-world circumstances in our department focusing on the treatment schedule and the outcome.

Prospectively collected data from all adult patients fulfilling the criteria of sHLH who diagnosed and managed from January 1, 2010 to June 1, 2018, in our department of the tertiary care university hospital of Larissa, Greece, were analysed retrospectively (n = 80; 52% male; median age: 55 years). The electronic records and/or written charts of the patients were reviewed for the demographic characteristics, clinical manifestations, underlying causes of sHLH, laboratory parameters, treatment schedule and 30-d-mortality rate. Most of patients had received after consent intravenous γ-immunoglobulin (IVIG) for 5 d (total dose 2 g/kg) in combination with intravenous steroid pulses followed by gradual tapering of prednisolone.

Seventy-five patients (94%) reported fever > 38.5 °C, 47 (59%) had liver or spleen enlargement and 76 (95%) had ferritin > 500 ng/mL including 20 (25%) having considerably high levels (> 10000 ng/mL). Anaemia and thrombocytopenia occurred in 72% and leucopoenia in 47% of them. Underlying infections were diagnosed in 59 patients (74%) as follows: leishmaniasis alone in 15/80 (18.9%), leishmaniasis concurrently with Coxiella Burnetti or non-Hodgkin lymphoma in 2/80 (2.5%), bacterial infections in 14/80 (17.5%) including one case with concurrent non-Hodgkin lymphoma, viral infections in 13/80 (16.3%), fungal infections in 2/80 (2.5%), infections by mycobacteria in 1/80 (1.3%) and unidentified pathogens in 12/80 (15%). Seventy-two patients (90%) had received combination treatment with IVIG and intravenous steroids. Overall, sHLH resolved in 76% of patients, 15% died within the first month but 82.5% of patients were still alive 6 mo after diagnosis. Univariate analysis showed older age, anaemia, thrombocytopenia, low fibrinogen, disseminated intravascular coagulation (DIC), and delay of diagnosis as factors that negatively affected remission. However, multivariate analysis showed low platelets and DIC as the only independent predictors of adverse outcome.

sHLH still carries a remarkable morbidity and mortality. Underlying infections were the major cause and therefore, they should be thoroughly investigated in patients with sHLH. Early recognition and combination treatment with IVIG and corticosteroids seem an efficient treatment option with successful outcome in this life-threatening condition.

• Haemophagocytic syndrome
• Haemophagocytic lymphohistiocytosis
• γ-immunoglobulin
• Leishmaniasis
• Ferritin

We present the case of an 8-year-old girl with hemophagocytic lymphohistiocytosis secondary to a Salmonella typhi infection. She received antibiotic treatment and intravenous immunoglobulin with complete resolution of the symptoms. We present a review of previously reported pediatric cases and propose a gradual approach to treatment.

• Epstein-Barr virus
• Hemophagocytic lymphohistiocytosis
• Lymphoma
• Soluble IL2 receptor

• Central nervous system (CNS)
• Cerebrospinal fluid
• Hemophagocytic lymphohistiocytosis (HLH)
• Intrathecal treatment
• Long-term effects
• Neuroradiology
• Overall survival
• Treatment

• Karolinska Institute

Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin. In this manuscript, we present the case of a patient with primary Sjögren's syndrome who developed HLH after an acute infection by Cytomegalovirus. We will describe and discuss the pathogenesis, differential diagnosis and a pragmatic approach to the treatment for this critically important and, when diagnosed early, potentially curable syndrome.

• CMV
• Cytomegalovirus
• HLH
• Hemophagocytic lymphohistiocytosis
• Sjögren's syndrome

• Systemic lupus erythematosus
• macrophage activation syndrome
• rituximab