Histiocytosis refers to several syndromes or serious blood diseases that affect around 1,400 people a year in the US, mostly children. Histiocytosis typically involves an abnormal increase in the number of white blood cells (called histiocytes). The histiocytes cluster together and can attack the skin, bones, lung, liver, spleen, gums, ears, eyes, and/or the central nervous system. The disease can range from limited involvement that spontaneously regresses to progressive multiorgan involvement that can be chronic and debilitating. In some cases, the disease can be life-threatening.

In some ways, histiocytosis is similar to cancer and has historically been treated by oncologists with chemotherapy and radiation. Unlike cancer, histiocytosis sometimes goes into remission without treatment. It is approximated that histiocytosis affects 1 in 200,000 children born each year in the United States. It is estimated that 8.9 of every 1,000,000 children under the age of 15 have histiocytosis. Seventy-six (76) percent of the cases occur before ten (10) years of age, but the disease is also seen in adults of all ages. Over the years, cancer treatments have been used in patients with histiocytosis. Consequently, hematologists and oncologists, who treat cancer, also treat children with Langerhans cell histiocytosis.  However, the disease is not cancer.  Radiation therapy, if used, is given in much lower doses than that which cancer patients receive.

There are three major classes of histiocytoses:

  1. Langerhans cell histiocytosis (LCH), which is also called histiocytosis X, is a rare disease involving clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Clinically, its manifestations range from isolated bone lesions to multisystem disease. The rarity of LCH in adults makes its incidence difficult to assess, but if we can draw from research which has suggested that a little less than one third of LCH occurs in adults, then about 1 in 560,000 adults might be affected. This illness is so rare, there is little research into its cause and treatment, and it is often referred to as an “orphan disease,” meaning it strikes too few people to generate government – supported research. The cause of LCH is unknown.  It may be triggered by an unusual reaction of the immune system from something commonly found in the environment.  It is not a known infection or cancer.  It is not known to be hereditary or communicable.
  2. Malignant histiocytosis syndrome (now known as T-cell lymphoma) T-cell lymphoma describes several different types of Non-Hodgkin lymphoma which affect T cells. T cells are involved in four classes of lymphoma. Together with lymphomas involving NK cells, these account for perhaps one in eight cases of NHL. They can be associated with Epstein Barr virus and Human T-cell leukemia. Lymphomas result when DNA damage or changes occurs to an immune cell (a lymphocyte) that alters the behavior of the cells. The damage to DNA results in the abnormal production of proteins that prevents the cells from dying when they should, or causes sustained rapid cell division that produces more of its kind. These malignant cells then may accumulate to form tumors in areas where the normal counterparts of the cell type reside – lymph nodes, skin, mucosal linings, etc.
  3. Non-Langerhans cell histiocytosis (aka hemophagocytic syndrome)

There are two subtypes of non-Langerhans cell histiocytosis:

* Class IIa: histiocytosis involving dermal dendritic cells
* Class IIb: histiocytosis involving cells other than Langerhans cells and dermal dendrocytes

The subtypes can be told apart by their different appearance and special staining of tissue examined under a microscope. Like other forms of histiocytosis, non-Langerhans cell histiocytosis tends to cause reddish-brown or reddish-yellow bumps in the skin and may affect internal organs (such as liver, kidneys, lungs).

Erdheim-Chester disease is an example of non-Langerhans cell histiocytosis. The disease involves an infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.

Patients Against Lymphoma

Lymphoma Research Foundation

The Leukemia & Lymphoma Society

Histiocytosis and Diabetes

Histiocytosis is frequently associated with diabetes insipidus, even after several years of diagnosis and successful therapy. Some patients with Langerhans cell histiocytosis (LCH) develop loss of control of water balance through a deficiency or lack of a hormone, vasopressin, secreted by the pituitary gland. Areas in the middle of the brain secrete this anti-diuretic hormone that is then stored in the pituitary gland.  In most cases, the portion of the brain that secretes this hormone or the pituitary, which stores the anti-diuretic hormone, has been damaged by the presence of histiocytes.  The hormone is necessary to maintain a proper water balance within the body’s cells and blood. Without normal secretion of the anti-diuretic hormone from the pituitary gland – vasopressin, the kidneys lose excessive water (polyuria) causing increased concentration of the blood and dehydration which leads to thirst.

Not all patients with Langerhans cell hisitocytosis develop Diabetes Insipidus.  Estimates of the percentage of LCH patients that do develop Diabetes Insipidus is around 30% with a range of 5% to 50% reported in different studies.

NYTimes Health

Histiocytosis Association of America

The Histiocytosis Research Trust

Histiocyte Society

Sofia Marcile Wardle SofiaMaze.com
Sofia was a sweet, fun, 7 year old girl, who lost her battle with histiocytosis in September 2008. She had been on treatment since age 2 1/2 and never remembered a life without pain and disease. Even so, she lived her life to the
fullest, making loads of friends wherever she went. Before she died, Sofia helped design this site and she wanted you to play the game for free, as her gift to you. SofiaMaze.com
See News Article About Sofia and Launch of SofiaMaze HERE

~ by marbleroad on April 28, 2009.

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