What is Langerhans Histiocytosis?
Langerhans histiocytosis is also known as histiocytosis X, is a rare condition. This has some cancer characteristics but, unlike any other cancer, it may resolve completely or may be life-threatening. It is a condition where an abnormal proliferation of histiocytes (a type of white blood cell) occurs, which is termed as histiocytosis which resulted in the development of tumours, lesions, and damage of tissues and organs. One or more granulomas develop in the bones in about 80% of affected individuals, causing pain and swelling. The granulomas which typically occur in the skull, arms, or legs can cause bone fracture. Langerhans cell histiocytosis affects the lungs, liver, or blood-forming organs like bone marrow, thymus, and spleen. It may be fatal in 15-20% of cases. There are two types of histiocytes; macrophage/monocytes which fight and kill harmful proteins, viruses and bacteria, and dendritic cells, which act as antigen-presenting cells and help to stimulate the immune system. Langerhans cells are dendritic cells that are normally present in the outer layer of the skin; respiratory, genitourinary tract, and in bone marrow. It is most common in infants and younger children but can also affect the adult population.
It is a rare cancer type and its annual occurrence rate ranges from 0.5-5.4 cases per million persons per year. Nearly 1200 cases are reported in the USA every year. It is more common in infants and among younger children than adults and older population. Males are more affected than females; with a male to female ratio is 2:1.
There is no well-known cause of LHC. Rapid abnormal growth of LHC may be due to a viral infection, or due to certain genetic changes. Some research studies showed the positive association between infection with Human herpesvirus 6 (HHV-6) and the development of Langerhans cell histiocytosis. Cigarette smoking is also a risk factor for the development of LCH of the lungs.
Signs and Symptoms
Signs and symptoms vary according to the involvement of organs for example;
- Congenital (by birth) LCH malignancy presents itself as skin lesions that are present at birth or develop after birth.
- 25% of patients with multiple origin lesions of MCH show signs and symptoms of recurrent otitis media (inflammation and infection of the middle ear), mastoiditis(swelling of mastoid bone or process which is present under the ear), polyuria(excessive urination) and polydipsia( excessive thirst).
- In some cases, there is a single bone or skin lesion.
Diagnosis and Treatment
Baseline recommended diagnostic tests are;
- Complete blood count (CBC) with peripheral smear
- Liver function tests (LFTs)
- Complete Urine Examination (UCE)
Radiological and imaging tests to locate the tumour are;
- X-Ray Chest
- CT Scan
- PET Scan
Definite diagnostic test/Procedure
- Biopsy of the skin or lesion
- Histocytology of the lesion
Treatment options are;
- A wide local surgical excision or curettage of the tumour is preferred in the case of a single lesion.
- Administration of Intralesional (inside the lesion) steroid injection is recommended for painful bony lesions. Local single or multiple bone and skin lesions are best treated with local or systemic steroidal medicine or with radiotherapy.
- Systemic chemotherapy is suggested for a multisystem disease or non-responsive cases of the single-system disease to other treatments. Usually, the combination of cytotoxic drugs with systemic steroids is effective. Methotrexate, prednisone, and vinblastine are used at low to moderate doses.
What support can we give for Langerhans Histiocytosis?
Langerhans Histiocytosis is a rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. We provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Langerhans Histiocytosis, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
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