Der Anaesthesist
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Hemophagocytic lymphohistiocytosis (HLH) has well been studied as a genetic disorder in children (primary HLH). Mutations in the regulatory complex of the cellular immune synapse lead to a loss of function of cytotoxic T‑cells and natural killer cells with excessive inflammation based on a cytokine storm. During the last decade, an increasing number of adult HLH patients without a family history of HLH (secondary or acquired HLH) have been reported. Various triggers - infections, malignancies or autoimmune diseases - result in an acquired loss of function of these cells and a sepsis-like disease. Missed or late diagnosis is believed to be a major cause of the high mortality. ⋯ Because of the similar clinical presentation to that of sepsis, HLH is often not recognized, resulting in a fatal outcome. In "sepsis" patients on the ICU with deterioration despite a standard of care, HLH needs to be considered by testing for ferritin when considering differential diagnoses. The complexity of the illness requires interdisciplinary patient care with specific integration of the hematologist in the diagnostic workup and therapeutic management, because of the frequent use of chemotherapy-based immunosuppression.
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Decompression of the chest is a life-saving invasive procedure for tension pneumothorax, trauma-associated cardiopulmonary resuscitation or massive haematopneumothorax that every emergency physician or intensivist must master. Particularly in the preclinical setting, indication must be restricted to urgent cases, but in these cases chest decompression must be executed without delay, even in subpar circumstances. The methods available are needle decompression or thoracentesis via mini-thoracotomy with or without insertion of a chest tube in the midclavicular line of the 2nd/3rd intercostal space (Monaldi-position) or in the anterior to mid-axillary line of the 4th/5th intercostal space (Bülau-position). ⋯ In extremely urgent cases opening the pleural membrane by thoracostomy without inserting a chest tube is sufficient in mechanically ventilated patients. Complications are common and mainly include ectopic positions, which can jeopardise effectiveness of the procedure, sometimes fatal injuries to adjacent intrathoracic or - in case of too inferior placement - intraabdominal organs as well as haemorrhage or infections. By respecting the basic rules for safe chest decompression many of these complications should be avoidable.