Definition: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease affecting infants and young childen, but in some cases adolescents. It is characterized by the pathological findings of hemophagocytosis, jaundice, fever, and splenomegaly.
HLH is usually associated with Epstein-Barr virus, genetic, malignant, autoimmune diseases, as well as other viruses or fungal infections. Diagnosis: Diagnostic procedures needed to examine for abnormal or excessive immune cells includes bone x-ray, biopsy of the affected tissue (bone marrow, bone, lymph node, skin, and/or liver), urinalysis, CT or MRI scan, blood samples, and genetic tests. Treatment: Corticosteroids are usually provided for treatment of HLH, in combination with chemotherapy which helps suppress the immune system. Supportive treatment is also provided for the immune system, including transfusions, antibiotics, hormone replacement therapy, nutritional support, and physical therapy.
Other cases of HLH may require different forms of treatment, such as radiation therapy, stem cell treatment, surgery, and immune based therapy. Symptoms and Signs: The symptoms of HLH include jaundice, fever, low blood count, enlarged spleen or liver, rash, paleness, fatigue, weight loss, and irritability. Causes: HLH has an autosomal recessive nature, and thus may be caused by genetics. It may also be caused by infections, immunodeficiency, or an underlying malignancy. : hemophagocyticlymphohistiocytosis The information on this site is for research purposes only and can not substitute for the advice of a medical professional. Ailments.com is not run by doctors and does not engage in the practice of medicine. Our site is not a medical authority, it is just a research tool for you to use in preparation for consulting with a doctor. We can not guarantee our information is accurate or up to date. Even if a statement made about a medical condition is accurate, it may not apply to you or your symptoms, so you should always consult a doctor. Nothing on our site should be construed as an attempt to offer or render a medical opinion or otherwise engage in the practice of medicine.
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