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Date of last update: 10/12/2017.

Forum Name: Hematology Topics

Question: neutophils too large - Chedial-Hagashi

 mermaid_ g-ma - Tue Feb 12, 2008 10:04 pm

My two month old grandson was diagnosed with Chedial-Hagashi.
His neutophils are too large. Since neutophils are constantly being replenished, why aren't his new ones smaller. My daughter in law was told he would need a bone marrow transplant in the next few months. Is this common?
 Dr. Chan Lowe - Mon Feb 18, 2008 8:06 pm

User avatar Hello Mermaid_g-ma,

Chediak-Higashi syndrome (CHS) is a genetic condition that affects many cells of the body, including the white blood cells. It is quite rare. You are correct that the body is constantly making new WBC's; however, the problem is that the genetic make up of the cells that become new WBC's is abnormal. As a result, all the new cells made are also abnormal.

Unfortunately, these children often will die early due to complications from infection or problems with the WBC's themselves. Those that survive tend to develop neurological problems as they get older.

The treatment of choice is a bone marrow transplant. The bone marrow holds the precursor cells that become WBC's. A bone marrow transplant replaces the genetically abnormal bone marrow cells with new, normal marrow cells that then produce normal white blood cells. Unfortunately, this fixes the immune problem but not the neurological problems, which often can continue to develop. At this point there is not a great treatment for this part.

Your grandson needs to be under the care of a pediatric hematologist that can help be sure that he is getting the correct care for this disorder.

I would also suggest that your son and daughter-in-law consider talking with a geneticist about this condition. It is an autosomal recessive disorder. This means that there is a 25% chance of future children also having this disorder. The geneticist can give them all the latest information and answer any questions about this that they may have.

I hope this helps some. Best wishes.
 mermaid_ g-ma - Mon Feb 18, 2008 10:47 pm

Dear Dr. Lowe, thank you for your precious time to respond to my question.
I went to see my g-baby this weekend. His physical features in no way indicate CHS.
I do know that my son and his wife take extra precautions and see a hematologist.
I think there is one genetic doctor in their area.
Thanks again and God Bless

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