I will try my best to give you a simple version of the illness for easy reading --not the wordy medical term filled version

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Introduction to Histiocytosis
Typical Symtoms and Complications
Tests and Treatment
Quick FACTS
Explaining Some Terms

Introduction to Histiocytosis

Alternate names for Histiocytosis X:eosinophilic granuloma; nonlipid reticuloendotheliosis; Langerhans Cell Histiocytosis (LCH); Letterer-Siwe disease; Hand-Schuller-Christian disease; pulmonary langerhans cell granulomatosis; pulmonary histiosytosis X;

There are three common types of Histiocytosis X which all characterizes an increase in the number of histiocyte cells in the blood. (histiocytes are a form of white blood cell in the body which widely multiply when possibly threatening foreign cells enter our body... they are sort of like the defence unit)

In order of increasing severity, these three types are Eosinophilic Granuloma, Hand-Schuller-Christian Disease, and Letterer- Siwe disease.

In the case of patients with Histiocytosis X, the histiocytes in their bodies multiply uncontrollably for an unknown reason, forming masses of tumor like tissue, attacking and causing damage to the patients bones, spleen, liver, lungs, skin and lymph nodes. In many ways, Histiocytosis X is like cancer --although remission may take place without treatment.

Eosinophilic Granuloma are more commonly found in children and young adults. Hand-Schuller-Christian Disease is present mainly in children under 5 years old and Letterer-Siwe disease is mostly present in children 2 and under. (which is often fatal)

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Typical Symtoms & Complications

Patients often experience multiple symtoms.

• Bone Lesions (Fractures) --often characterized by abnormal PAIN;
• Spontaneous Pneumothorax --often characterized in chest pains and shortness of breath due to lung collapsion;
• Skin rashes
• Weight loss
• Diabetes Insipidus --loss of control of water balance due to lack of hormone Vasopressin (an ADH hormone) secreted by pituitary gland
• Failure to thrive --growth failure due to pituitary dysfunction
• Fever and Coughing
• Anemia
• damage to different organs (i.e. liver, lungs etc.) --depending on the extent of histiocyte growth throughout the body

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Tests & Treatment

TESTS
Depending on the location of suspected histiocyte growth, different tests and treatments are administered.

• Chest X-ray
• Biopsy of area with suspected histiocyte presence (this may range from a bone in the body; skin etc.)
• Bone Scan (with radioactive isotopes)
• Skeletal Survey X-ray (of entire skeleton)
• Bone Marrow Biopsy
• Pulmonary function tests / bronchoscopy
• CBC

TREATMENT *
There is a wide range of treatment for this illnesses depending on the severity AT the time of treatment.

• Corticosteroid Therapy
• Radiation Therapy / Chemotherapy

* It is very important to note that Spontaneous Healing of Lesions is possible. Depending on the severity of the symtoms, treatment may not be necessary. Please consult your physician and obtain a second opinion if you have any questions.

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Quick Facts
Facts and Figures gathered may not be entirely up to date - but close.

• this illness stabilizes or improves in about one half of the affected people
• Histiocytosis X affects an estimated 1 in every 200,000 children and adults each year
• The disorder is more common in Caucasians, especially in young men
• Remission occurs in about 30% of patients, stabilization in 30% and progression in 30% (don't ask me where the other 10% went... )
• There have been links of smoking and pulmonary involvement in patients with the illness
• In children, bone involvement occurs 80% of the time
• Infants (especially 6 - 18 months old) have the most severe involvement of this illness

It is a fact that, even though the pain and suffering associated with this disease is just as severe as that with diseases which are well known, the illness is receiving very minimal ongoing research and funding.

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Explaining Some Terms

Anemia	Abnormally low level of red blood cells in the blood.
Biopsy	Surgical removal of tissue for examination.
CT or CAT	Computed tomography, a radiologic imaging that uses computer processing to generate an image of tissue density in slices through the patient's body.
Eosinophils	A leukocyte with coarse, round granules present.
Histiocyte	A type of white blood cell whose purpose is to remove foreign material from the blood and tissues. In Histiocytosis, there is an increase in number of these histiocytes in the patient. Through research, it was also found that Langerhan cells are also involved. That's why the illness has been renamed from Histiocytosis X to Langerhan Cell Histiocytosis.
Inflammatory	A localized protective response of the body caused by injury or destruction of tissues.
Langerhan Cells	The function of Langerhan Cells is to recognize foreign material, including bacteria, and stimulate the immune system to react to them. Langerhans cells are usually found in skin, lymph nodes, lungs, and the gastrointestinal tract. In Langerhan Cell Histiocytosis, Langerhan Cells accumulate with other immune cells in various parts of the body and cause damage by the release of chemicals.
MRI	Magnetic resonance imaging, a noninvasive nuclear procedure that uses electromagnetic energy to create images of structures inside the body.
Pituitary gland	The master gland located in the middle of the head that controls the endocrine glands and affects most bodily functions.

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