Acquired Hemophagocytic Lymphohistiocytosis

Acquired Hemophagocytic Lymphohistiocytosis

Article
Diseases & Conditions
Cancer & Benign Tumors
+3
Contributed byKrish Tangella MD, MBAAug 29, 2023

The topic Acquired Hemophagocytic Lymphohistiocytosis you are seeking is a synonym, or alternative name, or is closely related to the medical condition Hemophagocytic Lymphohistiocytosis (HLH).

Quick Summary:

  • Hemophagocytic Lymphohistiocytosis (HLH) is a type of immune disorder, called cytokine storm syndrome (CSS). Cytokine storm syndromes exhibit excessive stimulation of the immune system, which results in a widespread inflammatory response that is often life-threatening
  • Cytokines are small signaling proteins that various bodily cells use to coordinate an immune response. This includes immune cell activation and recruitment; it also includes inflammation
  • Inflammation is associated with many of the familiar symptoms of injury and infection. These include redness, swelling, warmth, and pain. The purpose of an inflammation is to draw the body’s resources to the site of injury or infection
  • When tissues are injured or infected, cells in the immune system that are located at that site, secrete cytokines and other chemicals that signal the body that there is a need to ‘fight-off’ an infection or repair tissue injury. The newly arriving immune cells then secrete additional cytokines, to activate even more of immune cells throughout the body. In such a manner, the immune response builds on itself. This process  is known as a positive feedback
  • Proper regulation is necessary to prevent this process from going out of control. This is accomplished using other signals that limit the effect of the cytokines in a large number of complex ways
  • Cytokine storm syndromes result, when the immune response system gets out of control. The inflammatory response that results from CSS is body-wide and debilitating. It is characterized by overabundant and hyperactive immune cells
  • Hemophagocytic Lymphohistiocytosis is a CSS that is characterized by an excessive stimulation of inflammation and attack of the body’s own blood cells (termed as hemophagocytosis)

Please find comprehensive information on Hemophagocytic Lymphohistiocytosis (HLH) regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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