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(C96.0) Multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease]
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123 676 in individuals diagnosis multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease] confirmed
37 151 deaths with diagnosis multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease]
30% mortality rate associated with the disease multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease]
Diagnosis multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease] is diagnosed Men are 17.09% more likely than Women
72 407
Men receive the diagnosis multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease]
17 751 (24.5 %)Died from this diagnosis.
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Women receive the diagnosis multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease]
19 400 (37.8 %)Died from this diagnosis.
Risk Group for the Disease multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease] - Men aged 75-79 and Women aged 70-74
In Men diagnosis is most often set at age 0-94
Less common in men the disease occurs at Age 95+in in women, the disease manifests at any age
In Women diagnosis is most often set at age 0-95+
Disease Features multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease]
Absence or low individual and public risk
Multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease] - what does this mean
Letterer-siwe disease is a rare disorder caused by an abnormal proliferation of langerhans cells, a type of white blood cell. it is characterized by the presence of lesions in multiple organs, including the skin, lungs, liver, spleen, bones, and central nervous system. the disease can be localized or disseminated, and may be acute or chronic in nature. symptoms can include fever, rash, weight loss, anemia, and organ dysfunction. treatment typically involves a combination of chemotherapy, immunotherapy, and/or surgery.
What happens during the disease - multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease]
Langerhans-cell histiocytosis (lch) is a disorder of histiocytes, a type of white blood cell, which leads to the accumulation of these cells in various organs or tissues. this accumulation of cells can cause inflammation and tissue damage, leading to a variety of symptoms depending on the organs affected. in the case of multifocal and multisystemic (disseminated) lch, the disease is caused by a mutation in the braf gene, leading to the overproduction of histiocytes, which can then accumulate in multiple organs and cause inflammation.
Clinical Pattern
More details coming soon
How does a doctor diagnose
- Complete physical examination
- Laboratory tests to determine if the patient has an elevated white blood cell count, elevated ESR, and elevated LDH
- Imaging studies such as X-rays, CT scans, or MRI scans to look for any signs of the disease
- Biopsy of the affected tissue to confirm the diagnosis
- Bone marrow aspiration and biopsy to look for evidence of the disease
- Lymph node biopsy to look for evidence of the disease
- Skin biopsy to look for evidence of the disease
- Pulmonary function tests to look for any signs of the disease
- Genetic testing to look for any mutations associated with the disease
Treatment and Medical Assistance
Main goal of the Treatment: To reduce the symptoms of the Letterer-Siwe disease and prevent complications.
- Administering systemic corticosteroids
- Administering systemic chemotherapy
- Administering radiation therapy
- Administering biologic therapies such as interferon-alpha
- Administering immunomodulatory agents such as hydroxychloroquine
- Surgery to remove affected organs or tissues
- Providing supportive care, including pain management, nutrition, and physical therapy
- Providing psychosocial support to the patient and their family
16 Days of Hospitalization Required
Average Time for Outpatient Care Not Established
Multifocal and multisystemic (disseminated) langerhans-cell histiocytosis [letterer-siwe disease] - Prevention
The best way to prevent letterer-siwe disease is to avoid exposure to environmental toxins, such as cigarette smoke, asbestos, and other air pollutants. it is also important to maintain good hygiene and to avoid contact with individuals who may be infected with the disease. vaccination is also recommended for those at risk of contracting the disease. additionally, regular health check-ups and early diagnosis are important for the prevention and treatment of the disease.