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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.
It most commonly affects individuals over the age of 65, due to the accumulation of genetic mutations that occur over time. [3] [18] CLL is rarely seen in individuals less than 40 years old. [19] Men are more commonly affected than women, although the average lifetime risk for both genders are similar (around 0.5-1%) .
It occurs primarily in elderly adults, far less frequency in younger adults, and rarely in children. Elderly adults present with B symptoms (i.e. fever, night sweats, and weight loss), swollen lymph nodes, and symptoms due to malignant cell infiltrations into the upper gastrointestinal tract, lungs, upper airways, and/or other organs. Younger ...
In approximately 51–70% of people with this disease, serum IgG4 concentrations are elevated during an acute phase. [ 1 ] [ 2 ] [ 3 ] It is a relapsing-remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved.
Acute lymphoblastic leukemia (ALL) is a cancer of the lymphoid line of blood cells characterized by the development of large numbers of immature lymphocytes. [1] Symptoms may include feeling tired, pale skin color, fever, easy bleeding or bruising, enlarged lymph nodes, or bone pain. [1]
Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal B lymphocytes. [1] The incidence of hairy cell leukemia (HCL) is 0.28-0.30 cases per 100,000 people in Europe and the United States and the prevalence is 3 cases per 100,000 in Europe with a lower prevalence in Asia, Africa and the Middle East.
The signs and symptoms of non-Hodgkin lymphoma vary depending upon its location within the body. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredness. Other symptoms may include bone pain, chest pain, or itchiness. Some forms are slow growing, while others are fast growing. [1]
Although lymphocytes are usually considered mature, as seen in blood tests, they are certainly not inert. Lymphocytes can travel around the body wherever there is a need. When such needs arise, new rounds of downstream lymphopoiesis, such as cell multiplication and differentiation, may occur, accompanied by intense mitotic and metabolic activity.