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Fig. 2 | Pediatric Rheumatology

Fig. 2

From: Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

Fig. 2

HLH/MAS Evidence-Based Guideline Diagnostic Algorithm. The steps suggested in the HLH/MAS EBG diagnostic evaluation are depicted in the flow chart. HLH, hemophagocytic lymphohistiocytosis; MAS, macrophage activation syndrome; Neuro, neurology; MRI, magnetic resonance imaging; CNS, central nervous system; LP, lumbar puncture; BM, bone marrow; PET, positron emission tomography a. See Table 1. b. See Table 2. c. Neurologic symptoms include headaches, cognitive changes, focal examination findings, seizures, findings not explained by degree of illness/medications.d. MRI findings concerning for HLH/MAS include but are not limited to parenchymal lesions, diffuse brain edema, leptomeningeal enhancement, periventricular white matter changes, brain volume loss, and spinal lesions. A normal MRI does not rule out CNS HLH/MAS. Some patients may only have abnormalities in the cerebral spinal fluid. e. Concern for infection includes but is not limited to immunocompromised hosts, recent travel, known exposures, localizing signs/symptoms, and critically ill patients. f. Concern for malignancy includes atypical lymphadenopathy and cytopenias out of proportion of the clinical presentation. g. Indications for treatment include clinical deterioration, unremitting fevers, progressive worsening of laboratory parameters of HLH/MAS. h. See Table 3 *This guideline was developed for educational purposes only and for use in the Rheumatology Program at Boston Children’s Hospital. Decisions about evaluation and treatment are the responsibility of the treating clinician and should always be tailored to individual clinical circumstances

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