~:: JoM SaMa2 KiTa ::~

~Jom KLiK Cni ~

Thursday, February 23, 2012

~:: WHaT Is HLH ?? apa itu HLH??::~

assalamulaikum semua..saya baru lepas lunch & skg tgh buat 

pumping utk noon session..


berkesempatan ber blog walking...

baru jugak dapat tau pasal pemergian adik Faris yang tabah......

apa yang saya rasa waktu dapat tahu?Allah saja yang Maha Memahami...

saya dah x mampu nk teruskan entry ni..

Semoga keluarga arwah dalam perlindungan Allah hendaknya...


* saya xmampu nk tgk wajah2 adik Faris lama2..tgk je kt blog en mazidul ni...

* saya mmg sorg yg sgt yg saya dapat tentang HLH (penyakit yg dideritai adik Faris)waktu saya mula2 dapat tau pasal arwah...ringkasnya..

What is hemophagocytic lymphohistiocytosis?
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder primarily affecting young children at a very early age, but it is found in patients of all ages.

The human body contains many types of cells which help fight infection. One type of cell is called the histiocyte. Histiocytes are produced by the bone marrow, and they may travel throughout the body. At a later stage, these cells remain in place within various tissues in the body. Their job is to help destroy certain foreign materials and to fight infections. This is done in cooperation with other important cells.

Patients with active HLH have too many of these histiocytes, as well as lymphocytes (another kind of infection-fighting cell), both of which are so-called white blood cells that may cause inflammation (swelling, redness, heat, pain, and loss of function). These cells then begin to penetrate and accumulate in good tissue and can cause damage to a variety of organs.

What causes this disease?
Scientists do not yet know all the complicated processes involved in this disease.

There are two major forms of HLH. One, known as the primary form, is inherited. The other is known as the secondary form. In the secondary form, the disease develops secondary to inappropriate (abnormal) activity of the immune system. This can occur after the use of immunosuppressive therapy and/or infections.

What does familial mean?
Familial hemophagocytic lymphohistiocytosis (also known as FHL, FEL or FHLH) means that the particular form of the disease is genetic.

How will I know if it is familial?
It is extremely difficult, if not impossible, to distinguish by testing between a primary (familial) and secondary form of HLH in each patient. However, onset at an early age is suggestive of a familial form, whereas onset later than 6 years of age is rare in the familial form.

What genes cause familial HLH (FHL)?
Thus far, two of an expected three or four genetic causes of familial HLH have been described. One genetic mutation that causes FHL results in a deficiency of perforin, a critical protein responsible for the function of immune cells involved in control of viral infections. The gene is named PRF1. Perforin is required for natural-killer lymphocytes (NK cells) and T lymphocytes (cytotoxic T cells) to fight viral infections and also to keep the immune system, especially histiocytes, under control. A second genetic mutation responsible for FHL involves the gene-encoding Munc13-4 protein, which is also involved in the function of NK and T cells.

Approximately 50% of FHL in North America is caused by mutations in the gene-encoding perforin, PRF1. In other parts of the world, approximately 20-30% of FHL cases are caused by the PRF1 mutations. Perforin mutations are a particularly common cause of FHL in patients of African descent. Cases of FHL due to mutations in the gene for Munc13-4 have been found worldwide, but it is not yet clear what percentage of FHL is due to this gene.


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