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The vast majority of patients who meet these criteria will not have a genetic cause of their disease, but rather HLH will be triggered by infection, malignancy, rheumatic disease, and/or certain treatments (as in the cytokine release syndrome associated with CAR T cell therapy). [citation needed]
Chédiak–Higashi syndrome [1] (CHS) is a rare autosomal recessive disorder that arises from a mutation of a lysosomal trafficking regulator protein, [2] which leads to a decrease in phagocytosis. The decrease in phagocytosis results in recurrent pyogenic infections, albinism , and peripheral neuropathy .
Phagoptosis occurs when signals on the surface of a (target) cell activate phagocytic receptors on a phagocyte, inducing uptake into a phagosome, where the cell is killed and digested. Phagoptosis (cell death by phagocytosis) is a type of cell death caused by the cell being phagocytosed (i.e. eaten) by another cell, and therefore this form of ...
Hodgkin's disease; Non-Hodgkin's lymphoma; Myelomas. Multiple myeloma (Kahler's disease) Solid tumor cancers Neuroblastoma; Desmoplastic small round cell tumor; Ewing's sarcoma; Choriocarcinoma; Hematologic disease. Phagocyte disorders. Myelodysplasia; Anemias. Paroxysmal nocturnal hemoglobinuria (PNH; severe aplasia) Aplastic anemia; Acquired ...
Efferocytosis can be performed not only by 'professional' phagocytic cells such as macrophages or dendritic cells, but also by many other cell types including epithelial cells and fibroblasts. To distinguish them from living cells, apoptotic cells carry specific 'eat me' signals, such as the presence of phosphatidyl serine (resulting from ...
Immune system cells: These assessments are used to measure the levels of various immune cells. Genetic testing involves collecting samples from patients for molecular analysis when there is a suspicion of inborn errors in immunity. Most Primary Immunodeficiency Disorders (PIDs) are inherited as single-gene defects. [25]
Follicular hyperplasia (FH) is a type of lymphoid hyperplasia and is classified as a lymphadenopathy, which means a disease of the lymph nodes. It is caused by a stimulation of the B cell compartment and by abnormal cell growth of secondary follicles. This typically occurs in the cortex without disrupting the lymph node capsule. [1]
B-cell prolymphocytic leukemia, referred to as B-PLL, is a rare blood cancer.It is a more aggressive, but still treatable, form of leukemia.. Specifically, B-PLL is a prolymphocytic leukemia (PLL) that affects prolymphocytes – immature forms of B-lymphocytes and T-lymphocytes – in the peripheral blood, bone marrow, and spleen.