Hemophagocytic Lymphohistiocytosis
Hemophagocytic Lymphohistiocytosis 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.
Hemophagocytic Lymphohistiocytosis 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.
Hemophagocytic Lymphohistiocytosis 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.
Hemophagocytic Lymphohistiocytosis Symptoms and Signs
The symptoms of HLH include jaundice, fever, low blood count, enlarged spleen or liver, rash, paleness, fatigue, weight loss, and irritability.
Hemophagocytic Lymphohistiocytosis 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.