Anthony's Little Brother, Nicholas

   Please sign our Guest Book, we like to know who stopped by
Sign     View

 

Follow us on Facebook  Click on logo to go to that Website

What Is LCH?

Histiocytosis is a rare, mysterious and sometimes fatal disease that primarily affects children.   The estimated annual incidence ranges from 0.5-5.4 per million persons per year. Approximately 1200 new cases per year are reported in the United States.   Similar in many ways to cancer, Histiocytosis is treated with radiation and chemotherapy.  Histiocytosis X is an uncontrolled growth of cells called histiocytes.  Histiocytes are scavenger cells, the cells responsible for removing tissue debris from the body.  Histiocytosis X can vary from a localized and mild form, with one or several bones involved, to a widespread disease involving the liver, lungs, skin, bones, and bone marrow.  Nicholas has multiple organ involvement (liver, bone marrow) and a scalp rash.  More than half the patients are diagnosed before the age of 2 years.  Although the cause and best treatment of this disease are unknown, our doctor told us that patients have too many histiocytes.  These cells widely multiply and can attack any part of the body.  A majority of patients will survive the disease.  Some may develop lifelong chronic problems such as diabetes insipidus.  In some cases, the disease is fatal.  Usually these are very young infants.  They have a rapid downhill course, and don’t respond to any known treatment.  According to our Hematologist/Oncologist, Dr. Anne Warwick, Nicholas has responded very well to the treatment and she is very excited she sees him improving.  My parents has given permission to have his records released to research projects and any other medical group so that hopefully his success can help others.

The following taken from "Langerhans Cell Histiocytosis Historical Perspectives" by Fritz Lampert, MD 

The Langerhans Cell

Paul Langerhans was born July 25, 1847 in Berlin.  The name Langerhans became firmly established in the history of medicine as a result of two microscopic discoveries he made as a young medical student whilst at the Institute of Pathology of the University of Berlin.  These were: 

1. The islands of cells in the body of the pancreas described in his doctoral thesis (1869) which were names ilôts de langerhans by E. Laguesse in 1893
2. The dendritic cells in the stratum germinativum of the epidermis.  He had made these cells visible by means of a gold chloride technique in 1868.  They were named Langerhans' sche Zellen by Sigmund Merkel in 1875.

Langerhans first thought that the structures he saw in the microscope (knob-like swellings with branching) were the endpoints of nerves, but he corrected himself - "that my cells are not important for nerve endings".  The sketch by Paul Langerhans of the microscopic picture of the skin with these structures and his quantitation - 500 to 900 per cm3 - are still valid today.

In contrast to the Langerhans Islands, the Langerhans Cells did not receive much attention in the 100 years following the first description. Between 1868 and 1973 fewer than 100 articles in medical literature dealt with them.

The symptoms of LCH will depend upon which part of the body is affected, and whether the disease is affecting more than one part of the body.

If LCH affects the bone, this can cause pain in the bone and/or swelling/lumps on the skull. If the skin is affected, a skin rash, such as cradle cap and nappy rash, may occur. A discharge from the ear or hearing problems can occur if the ear is affected. If LCH affects the lungs or chest, the child may have breathing difficulties. LCH within the abdomen may cause tummy problems (diarrhea) and liver problems, including possible jaundice. The lymph glands may be enlarged. Children may be irritable and have a poor appetite.

In 2–3 out of every 10 children with multi-system disease, the pituitary gland at the base of the brain is affected, causing hormonal problems. This can lead to the child passing larger amounts of urine and being very thirsty. This is called diabetes insipidus (which is different from sugar diabetes) and can be well controlled with specific medicines. Occasionally, other pituitary hormones may be affected causing poor growth or delayed puberty, which again can be treated.

Excerpts from CancerBACUP