ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that is characterized by hyperferritinemia, cytopenia, disseminated intravascular coagulopathy and functional disorders of the liver and the central nervous system. The term macrophage activation syndrome is predominantly used for secondary HLH in the context of autoimmune diseases (e.g., systemic juvenile idiopathic arthritis). In addition, malignancies and genetic inborn errors of immunity can predispose to the development of HLH. Infections (e.g., Epstein-Barr virus) in turn represent possible triggers of an acute episode. Due to the unspecific manifestation of the disease, a systematic evaluation of the organ systems is recommended in the clinical and laboratory analytical clarification of hyperinflammatory syndromes. In general, the treatment should be carried out by a multidisciplinary team with expertise in rheumatology, hematological oncology, infectious diseases and intensive care medicine. The primary treatment of HLH usually consists of glucocorticoids and in cases of a rapid deterioration of the condition anakinra (interleukin 1 block) and intravenous immunoglobulins can be employed. Treatment of the underlying disease should be consequently carried out in parallel, together with antimicrobial treatment.
Subject(s)
Lymphohistiocytosis, Hemophagocytic , Macrophage Activation Syndrome , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/therapy , Lymphohistiocytosis, Hemophagocytic/immunology , Humans , Macrophage Activation Syndrome/diagnosis , Macrophage Activation Syndrome/therapy , Macrophage Activation Syndrome/immunology , Macrophage Activation Syndrome/etiology , Patient Care Team , Glucocorticoids/therapeutic use , Evidence-Based Medicine , Diagnosis, Differential , Treatment Outcome , Immunoglobulins, Intravenous/therapeutic use , Rheumatology , Interleukin 1 Receptor Antagonist Protein/therapeutic useABSTRACT
BACKGROUND: Prognostic counselling is a sensitive issue in medicine and especially so in MS due to the highly heterogeneous disease course. However, people with MS (pwMS) seek prognostic information. The web-based 'Evidence-Based Decision Support Tool in Multiple Sclerosis' (EBDiMS) uses data of 717 patients from the London/Ontario cohort to calculate personalized long-term prognostic information. OBJECTIVE: The aim of this study was to investigate the feasibility and effect of long-term prognostic counselling in pwMS using EBDiMS. METHODS: Ninety consecutive pwMS were provided with personalized estimations of expected time to reach Expanded Disability Status Scale (EDSS) scores of 6 and 8 and time to conversion to secondary-progressive MS. Participants gave estimates on their own putative prognosis and rated the tool's acceptability on six-step Likert-type scales. RESULTS: Participants rated EBDiMS as highly understandable, interesting and relevant for patient-physician encounters, coping and therapy decisions. Although it provoked a certain degree of worry in some participants, 95% would recommend using the tool. Participants' own prognosis estimates did not change significantly following EBDiMS. CONCLUSION: Long-term prognostic counselling using an online tool has been shown to be feasible in a clinical setting. EBDiMS provides pwMS with relevant, easy-to-understand, long-term prognostic information without causing relevant anxiety.
