Langerhans’ Cell Histiocytosis

Langerhans’ Cell Histiocytosis General Information

Langerhans’ Cell Histiocytosis is a rare affection encountered that usually occurs in children between 1 and 15 years of age; it has been noted that the most affected are children in the 5 to 10 year old age group. While it is far more common in children, with an incidence rate of 1 in 200,000, adults are also affected by this condition however the incidence rate in adult patients is of 1 in 560,000. In elder patients, the disease is very rare. Also, studies have shown that Caucasian patients are more likely to be affected by this condition than patients of any other race, and that incidence rate in males is twice as high as in female patients. It is a rare affection that has not been proven to be genetically transmitted, however in some cases it has been noted that it occurred in patients that had a family history of such affections. A congenital form of the condition, the Hashimoto-Pritzker disease, has also been known to be self-healing.

Langerhans’ Cell Histiocytosis affects the clonal replication of the Langerhans cells, which are abnormal cells that derive from the bone marrow and which have the capability of migrating from their usual location in the skin to the lymph nodes, and causing multiple manifestations in the patient, from bone lesions that occur infrequently and are generally isolated incidents to multi-systemic disease. While related to other diseases which are characterized by abnormal reproduction and functioning of white blood cells (for example leukemia and lymphoma), this affection is part of the histiocytose syndromes group of syndromes. The term “histiocytes” is the archaic denomination for macrophages and dendritic cells.

Langerhans’ Cell Histiocytosis Symptoms

Generally, the specialized health care professionals divide histiocytoses into three groups. These are Langerhans’ Cell Histiocytosis, true histiocytic lymphomas (also known as the malignant versions) and benign histiocytoses. The benign histiocytoses are also called reactive, as they may be caused by a normal reaction of the body to an infection; an example of such a benign affection is leukocytosis, which refers to the replication of white blood cells as a normal reaction of the organism when fighting an infection, and in this case the histiocytes are the white blood cells.

Also, the complex of syndromes that are grouped into the term Langerhans’ Cell Histiocytosis can be further divided into three classifications, each group with its particular symptoms. The Unifocal type is typically a disease that progresses slowly, and is characterized by the Langerhans cell proliferation in bones, skin, stomach or lungs. The multifocal unisystem variation is generally accompanied by fever and bone lesions. The patient will also present eruptions in the ear canals and on the scalp. The Hand-Schuller-Christian triad is formed by diabetes insipidus , lytic bone lesions and proptosis.

The third type of Langerhans’ Cell Histiocytosis is the multifocal multi-system variation, also known under the name of Latterer-Siwe disease. It is a disease that typically evolves rapidly, and the Langerhans cells proliferate in multiple various tissues throughout the body. This version of the affection is typically encountered in children, becoming less common in patients over the age of 2. However, the mortality rate of this disease is as high as 50%, even when the patient undergoes aggressive chemotherapy treatment.

Langerhans’ Cell Histiocytosis Treatment

In order to correctly diagnose a Langerhans’ Cell Histiocytosis, extensive testing may be in order. The examining health care specialists may require blood tests in order to obtain a blood cell count and maybe coagulation studies also. Urine sample testing may be useful in detecting any signs of diabetes insipidus. In cases where the patient presents disturbances in the proper functioning of the liver, a biopsy may be required in order to rule out the possibility of cirrhosis. Chest X-rays and skeletal radiographs may be useful in further observing the condition, while CT scans and MRI may reveal abnormalities in the hypothalamic or pituitary gland regions. In most cases, a biopsy of the skin is performed as it may be conclusive in determining the diagnosis.

There are several types of treatment and therapy that may be employed in treating patients who suffer from Langerhans’ Cell Histiocytosis. The treatment may vary depending on what type of disease is affecting the patient, as well as on the patient’s age, general health condition and medical history. In the case of single-system histiocytosis, the solitary lesions of the bone tissue may be locally treated. Typically the procedures employed are curettage or excision, however if the lesions are painful they may require intra-lesional injections with a steroid type of drug, such as Triamcinolone Acetonide. Indomethacin has been administered in cases where the patient presents polyostotic lesions, but in certain cases this may be replaced by a short term treatment with systemic steroids. Localized disease of the skin requires treatment with topical steroids under the form of creams. Topical nitrogen mustard administration and phototherapy have also been used in patients with skin affected by Langerhans’ Cell Histiocytosis. Excision is typically employed in cases where the disease has affected the lymph nodes. The enlargement of the lymph nodes can usually be countered with systemic steroids, however if the nodes prove to be resistant to treatment, in most cases systemic chemotherapy is the best option.

In multi-systemic variations of Langerhans’ Cell Histiocytosis, systemic chemotherapy is the treatment of choice. Cytotoxic drugs may be administered in combination with systemic steroids for best effects. Low doses of Prednisone, Vinblastine and Methotrexate have been used with positive results. In Langerhans’ Cell Histiocytosis cases where the disease proves to be highly resistant to treatment, the health care specialists typically employ a treatment with Prednisolone, Antithymocyte globulin and Cyclosporin A. The treatment is usually employed if there is no matched donor for the patient, making bone marrow transplantation impossible. In severe cases where the survival chances are very low, the only possible choices are bone marrow transplantation or reduced-intensity condition stem cell transplantation. In cases where the patients have developed diabetes insipidus, the specialized health care professionals may prescribe Desmopressin Acetate to treat the affection symptomatically. For further information contact your personal physician.