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Langerhans Cell Histiocytosis
(Haemophagocytic Histiocytosis, Familial Erythroid Lymphohistiocytosis)

Langerhans cell histiocytosis is a very rare disorder which can effect children of any age but most frequently those under two years of age. The name of the disease is derived from the type of body cell involved - the "histiocyte", - osis meaning increased numbers. Older textbooks still use the terms "Eosinophilic Granuloma" for disease that effects a single part of the body (eg. bones), Hans-Schuller-Christian disease (involving bones, pituitary gland and causing protuberance of the eyes) and Letterer-Siwe disease (affecting infants and involving liver, spleen, bone marrow and skin). They were named after the physicians first described them and were once thought to be separate diseases.

Histiocytes are normal tissue cells and are found in many organs in the body. They are derived from cells in the bone marrow (macrophages) and migrate to their various tissue sites via the blood stream. Those that migrate to the skin are called "Langerhans cells", to the liver "Kupffer cells", and those found in the bone are called "Osteoblasts". They are also found in the lungs, the lymph glands and spleen, the gut and in the brain especially around the pituitary gland.

WHAT IS THE DISEASE?

It is easier to say what it is not!

Histiocytosis is not a cancer: The cells involved in the disease probably do not multiply in an uncontrolled fashion as happens in cancer, but rather migrate to a site in abnormal numbers or wander outside their normal tissue compartment. Recently it has been shown that many patients with Histiocytosis have a deficiency of a certain type of white blood cell (suppressor Lymphocyte). Whether this is the cause or effect of the disease we don't yet know, but it seems possible that the primary disturbance is within the body's self-defence system - the immune system - and is caused by a disturbance of the way in which cells involved in the immune response (making of antibodies) "talk" to each other. The factors provoking this disturbance are unknown so prevention is at present not possible.

Histiocytosis is not infectious: (catching) but it may develop after an infection and may be inherited in some families. It can come and go spontaneously and is not life-threatening unless organs such as bone marrow, liver or lungs, which are vital to sustain life, are severely affected.

HOW WILL IT SHOW ITSELF?

The two commonest parts of the body in which Histiocytosis occurs are in the bones (commonly skull or limb) producing a painful lump or severe scalp rash like cradle cap it may present as a groin rash like severe nappy rash or as reddish brown pinhead spots that may appear on the body. Chronic ear discharge can be the presenting symptom and enlargement of the liver and spleen can cause abdominal swelling. When the disease affects the pituitary gland (as it does in 20-30% of cases, though not necessarily at the beginning of the illness) it causes a disturbance of water balance called diabetes insipidus. This produces excessive thirst both day and night and the need to pass urine frequently in large amounts. In some patients the eyes may become prominent ("exophthalmos"). The disease may be accompanied by generalised symptoms such as loss of appetite, failure to gain weight, anaemia and recurrent fevers. Occasionally, the lungs are affected and disease in this site may result in a somewhat increased breathing rate or pain in the chest and acute breathlessness due to the collapse of a lung.

Only some of these features (perhaps only two or three of them) occur in individual patients, and that some, such as liver, spleen and bone marrow involvement, are more likely in younger than in older patients. Others, such as bone involvement and exophthalmos, are more common in older children. We do not know why the "pattern" of disease involvement varies in this way.

WHAT INVESTIGATIONS ARE NEEDED TO DIAGNOSE IT?

Diagnosis is usually made by taking a small sample (a biopsy) of the tissue involved, such as skin rash, bony swelling or liver, for microscopic examination. It is then necessary to determine the extent of the disease. Information about the bones and lungs is obtained from x-rays. A blood test determines liver function and cellular production by the bone marrow, but a needle biopsy (sample) of the bone marrow is required and will be carried out under either sedation or anaesthesia. Breathing tests may be necessary to determine lung function while water balance may be tested by measuring the concentration of a urine specimen that passes after an overnight fast.

TREATMENT

When the disease was thought to be a cancer, the normal method of treatment involved radiotherapy and chemotherapy (cancer drugs). Cancer specialists therefore gained experience of the disease and an interest in treating it. This is why your child will be referred to a cancer clinic. Histiocytosis is a disease which can disappear spontaneously - we call this spontaneous remission. Because of this, there is a chance that no treatment need be given at all, especially if the disease is in a part of the body, such as a non-weight bearing bone, where no serious damage is being caused. Treatment will only be needed if there are symptoms such as pain, fever, or failure to gain weight, or if important organs such as liver, lungs and bone marrow are affected. If the disease is confined to one organ, local treatment is directed to that organ, whilst if the disease is generalised a systemic (generalised) treatment will be given.

The most useful drugs (chemotherapy) are Vincristine, Vinblastine, VP16 and steroids. These are given by intravenous injection, usually at 1-3 weekly intervals, depending on the individual patient. Radiotherapy is rarely needed as most bony lesions will respond to local operation or steroid injection.

Scalp rash is treated by coconut oil ointment which is put on and left overnight. Next morning it is washed off with a shampoo such as Cetavlon and the affected areas covered with a steroid lotion. Skin rash may respond to soaking in potassium permanganate followed by the application of a steroid lotion or cream. A cleansing cream preparation rather than soap and water should be used for bathing.

Replacement water balance pituitary hormone (pitressin) may be required. This is usually given in the form of nose drops, two to three times daily. This replacement treatment is likely to be required lifelong.

WHAT PRECAUTIONS SHOULD I TAKE?

Children with lesions in weight-bearing bones are at risk of fracture but usually the site is painful and therefore protected by the child by non-use. Provided there is evidence of healing and no pain there is usually no need to restrict activity. Children should be encouraged to live a normal life and attend school normally.

PROGNOSIS

An important feature of Histiocytosis is the way in which it can appear (relapse) and disappear (remission). Sometimes this is spontaneous and sometimes in response to treatment. Single system disease is associated with a high incidence of spontaneous remission. Multi-system disease may spontaneously remit (disappear) but usually needs treatment. Disease in the lungs, bone marrow, liver and spleen is very resistant to treatment. On the whole the younger the child at diagnosis and the more organs involved, especially if these organs are vital to sustain life, the poorer the outlook. The disease tends to run a more chronic course the older the child becomes and although it may not be life threatening may leave the child with chronic problems such as chronic ear discharge, deafness, unsightly scalp lumps, protuberant eyes (exophthalmos), diabetes insipidus or breathing problems.

SUMMARY

Because the cause is still not understood and because individual cases vary so much, Histiocytosis is a very difficult disease to explain and understand. Each child is an individual and discussion with the treating specialist will help you to understand about his/her individual problems.

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