Contents
Histiocytosis is a very rare disease that affects children between 1 and 3 years of age. It is a disease of the hematopoietic system, associated with an excessive multiplication of histiocytes called Langerhans cells – the name of the person who first observed them. The direct cause of the development of histiocytosis, as scientists believe, may be: a disease of the immune system and the accumulation of intracellular defects, or a reaction to external factors. What are the symptoms of histiocytosis? how can it be treated?
Characteristics of the disease in histiocytosis
Langerhans cell histiocytosis (LCH) is a complex disease. In the past, due to the huge variation in the clinical picture, various forms of histiocytosis were described separately as Hand-Schuller-Christian disease, characterized by, inter alia, characteristic changes in bones, exophthalmos and excessive thirst with the production of large amounts of urine, and Letterer-Siwe disease characterized by a severe, often fatal course with typical changes not only in the bones but also in the skin, liver and spleen. Currently, we use this term to refer to hematological disorders in childhood. It has been given the common name histiocytosis X, where “X” signified a lack of clarity as to the origin and causes of its formation. Langerhans cell histiocytosis affects children of all ages, but is most common between the ages of 1 and 3, more often in boys. This disease affects many different organs and systems, e.g. bones, skin, lymph nodes, liver, lungs, spleen, haematopoietic and central nervous system. This means that histiocytosis is not one disease, but a diverse group of more or less malignant diseases characterized by the formation of abnormal cellular infiltration in various tissues.
Histiocytosis – symptoms
The symptoms of histiocytosis depend on the organs in which they originated, and which organs are occupied by the abnormal cells. In most patients, the disease usually affects one sphere and these are bones, while in the remaining patients the infiltrates are observed in various tissues. In children under 3 years of age, histiocytosis becomes disseminated in many organs.
The most common symptoms of histiocytosis include:
- single or multiple bone changes with a frequently palpable or even visible tumor around and above the affected bone and pain;
- recurrent otitis media, chronic ear discharge, hearing impairment%;
- changes on the skin characterized by lumpy spots or erythematous, sometimes yellow-brown, peeling skin
- enlargement of the lymph nodes;
- enlargement of the liver or spleen;
- fever;
- decrease in hemoglobin level, decrease in the number of white blood cells, decrease in the number of platelets;
- cough, breathing problems, spontaneous pneumothorax;
- symptoms of diabetes insipidus.
Histiocytosis – treatment
Patients with single system LCH either require minimal treatment or can only be monitored. If the bones are involved, the patient undergoes a biopsy, curettage of the lesion and treatment with steroids or radiotherapy in a more progressive form. People who have multiple organ involvement or a specific form of multiple organ involvement (e.g. with involvement of the nervous system or in the case of multifocal bone lesions) are eligible for the treatment of histiocytosis in children and adults.
If systemic therapy is necessary, the most frequently used drugs in the first phase of treatment are vimblastine and prednisone. Jełka does not bring any effect, the oncologist decides about further treatment. Treatment is long, consisting of 1-2 cycles of 6 weeks of chemotherapy followed by maintenance treatment for up to 12 months. Patients who do not respond to standard chemotherapy are candidates for allogeneic hematopoietic cell transplantation.