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Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Symptoms range from isolated bone lesions to multisystem disease . [ 1 ]
Before the Histiocyte Society classified histiocytoses in the 1980s, the condition was also known as "Histiocytosis X", where "X" denoted the then unknown cause. [9] [10] [11] It is now known as chronic multifocal Langerhans cell histiocytosis, a subtype of LCH. [3] The disease is rare. [12] Most present between the ages of two and six. [2]
In the United States during 2013–2017, the age-adjusted mortality rate for all types of cancer was 189.5/100,000 for males, and 135.7/100,000 for females. [1] Below is an incomplete list of age-adjusted mortality rates for different types of cancer in the United States from the Surveillance, Epidemiology, and End Results program.
The American Cancer Society reports 5-year relative survival rates of over 70% for women with stage 0-III breast cancer with a 5-year relative survival rate close to 100% for women with stage 0 or stage I breast cancer. The 5-year relative survival rate drops to 22% for women with stage IV breast cancer. [3]
Five-year survival rates can be used to compare the effectiveness of treatments. Use of five-year survival statistics is more useful in aggressive diseases that have a shorter life expectancy following diagnosis, such as lung cancer, and less useful in cases with a long life expectancy, such as prostate cancer.
Erdheim–Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis). It was declared a histiocytic neoplasm by the World Health Organization in 2016. [1]
Because of the prolonged survival, which was typically about 10 years in past decades, but which can extend to a normal life expectancy, [2] the prevalence (number of people living with the disease) is much higher than the incidence (new diagnoses). CLL is the most common type of leukemia in the UK, accounting for 38% of all leukemia cases.
It is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation of benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of HLH.
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