Eric Embrey

Newton County High School

Class of 2000

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First Name	Eric
Last Name	Embrey
Graduation Year	Class of 2000
Gender	Male
Hometown	Chunky, Mississippi
Relationship Status	Single
About Me	i'm 31. I did have a few health issues in the past where i died (yes you read that right) but now im alot better now. I would like to start a family some day. Other than that i'm just me laid back, I try to help people as much as possible even thou it usually bites me in the ass. What is HLH? HLH, Hemophagocytic Lymphohistiocytosis, is a life-threatening immunodeficiency. It affects people of all ages and ethnic groups. Common symptoms are fevers, enlarged spleen, low blood counts and liver abnormalities. At least half of the patients with HLH will also experience inflammation of the brain which can lead to a wide variety of neurologic problems. HLH has traditionally been divided into genetic forms (Familial HLH or FHLH) or acquired forms which may be associated with infections, autoimmune conditions or cancer. To date, 9 genetic causes of FHLH have been discovered, accounting for about 50% of childhood HLH cases in the U.S.A. FHLH can be inherited in either an autosomal recessive manner (where both parents are carriers) or in an x-linked manner affecting only males. HLH qualifies as an orphan or rare disease with a reported prevelance ranging from 1/150,000, although this may be an underestimate. The symptoms of HLH result from an overactive and poorly controlled immune response involving T lymphocytes and macrophages. This abnormally intense immune response results in prolonged and highly elevated levels of cytokines (molecules which, in minute quantities, normally regulate immune functions). This so-called "hypercytokinemia" is potentially damaging to all the major vital organs. The hallmark of hypercytokinemia is Hemophagocytosis, the process for which the disorder is named. Hemophagocytosis is an abnormal consumption of blood cells by macrophages which are turned on by the high cytokine levels, resulting in the low blood counts. While some patients experience HLH symptoms which can wax and wane or respond well to steroids initially, many patients suffer from progressive illness which can lead to death if appropriate immune suppressive treatment is not begun in time. Ultimately, children and young adults who have HLH on the basis of known genetic defects will require a hematopoietic cells transplant (BMT) to cure them. Patients without a known genetic cause who experience progressive HLH or recurrent episodes are also considered for BMT. At present, with the more widespread use of Reduced Intensity Conditioning (RIC) protocols accompanied by careful selection of donor and stem cell product to be used - long term survival rates after HCT have improve...(read more)