Hello Insomniacs.
A little update on the 3yo grand nephew... he has been officially diagnosed with Hemophagocytic Lymphohistiocytosis (HLH) - and Kawasaki disease made it reveal itself. At this time he is roid raging because they had put him on a very high dose of steriods. Over the next 6-8 weeks they'll taper that off while also conducting chemotherapy. He now has a PICC line installed and should be able to go home and have weekly treatments. Apparently, only after the chemo will they be able to determine if it's Familial FLH/Genetic HLH or Secondary HLH. If it's genetic, he will have to have bone marrow transplant(s) (if they can find a donor) and his twin will have to also be checked. So, overall... not good news.
Excerpt from the Internet: HLH is rare disorder mainly affecting children at an early age. The illness consists of the malfunctioning of the hystiocyte, one of the primary cells to fight infections. Bone marrow produces these cells that then travel throughout the body to fight infections and destroy foreign material.
FLH/HLH patients have overactive histiocytes and lymphocytes, which are white blood cells that can cause swelling, redness, heat, pain and malfunction/damage of organs when they attack and accumulate on healthy lymph tissue (eg: Liver, Spleen, lymph nodes). There are two forms of HLH: 'Familial' and 'Secondary', both require Chemotherapy treatment but:
1) Familial FLH/genetic HLH is the worst of the two. Often young age onset is an indicator of FLH, however exceptions exist. If FLH, there is a 25% chance of other siblings having the same illness. The danger of FLH resides in the necessity of a bone marrow transplant in order for the patient to be cured.
2) Secondary HLH is none genetic and presents itself when the body's immune system goes into overdrive following a major infection. Secondary HLH is treated with more relative ease than FLH as although Chemo is required, generally following treatment the underlying infection is cured and no bone marrow transplant is needed.
A little update on the 3yo grand nephew... he has been officially diagnosed with Hemophagocytic Lymphohistiocytosis (HLH) - and Kawasaki disease made it reveal itself. At this time he is roid raging because they had put him on a very high dose of steriods. Over the next 6-8 weeks they'll taper that off while also conducting chemotherapy. He now has a PICC line installed and should be able to go home and have weekly treatments. Apparently, only after the chemo will they be able to determine if it's Familial FLH/Genetic HLH or Secondary HLH. If it's genetic, he will have to have bone marrow transplant(s) (if they can find a donor) and his twin will have to also be checked. So, overall... not good news.
Excerpt from the Internet: HLH is rare disorder mainly affecting children at an early age. The illness consists of the malfunctioning of the hystiocyte, one of the primary cells to fight infections. Bone marrow produces these cells that then travel throughout the body to fight infections and destroy foreign material.
FLH/HLH patients have overactive histiocytes and lymphocytes, which are white blood cells that can cause swelling, redness, heat, pain and malfunction/damage of organs when they attack and accumulate on healthy lymph tissue (eg: Liver, Spleen, lymph nodes). There are two forms of HLH: 'Familial' and 'Secondary', both require Chemotherapy treatment but:
1) Familial FLH/genetic HLH is the worst of the two. Often young age onset is an indicator of FLH, however exceptions exist. If FLH, there is a 25% chance of other siblings having the same illness. The danger of FLH resides in the necessity of a bone marrow transplant in order for the patient to be cured.
2) Secondary HLH is none genetic and presents itself when the body's immune system goes into overdrive following a major infection. Secondary HLH is treated with more relative ease than FLH as although Chemo is required, generally following treatment the underlying infection is cured and no bone marrow transplant is needed.
