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Severe congenital neutropenia (SCN), also often known as Kostmann syndrome or disease, is a group of rare disorders that affect myelopoiesis, causing a congenital form of neutropenia, usually without other physical malformations.
The condition is usually caused by Gram-positive enteric commensal bacteria of the gut (). Clostridioides difficile is a species of Gram-positive bacteria that commonly causes severe diarrhea and other intestinal diseases when competing bacteria are wiped out by antibiotics, causing pseudomembranous colitis, whereas Clostridium septicum is responsible for most cases of neutropenic enterocolitis.
Neutropenia is an abnormally low concentration of neutrophils (a type of white blood cell) in the blood. [4] Neutrophils make up the majority of circulating white blood cells and serve as the primary defense against infections by destroying bacteria, bacterial fragments and immunoglobulin-bound viruses in the blood. [5]
Cyclic neutropenia; Other names: Periodic neutropenia, cyclic leucopenia, cyclic hematopoesis: Blood film with a striking absence of neutrophils, leaving only red blood cells and platelets. Specialty: Hematology Symptoms: Fever, malaise, inflammation and infection of oral mucosa, respiratory tract, digestive tract, skin, and abdominal pain [1 ...
Neutropenia, a subtype of leukopenia, refers to a decrease in the number of circulating neutrophil granulocytes, the most abundant white blood cells. The terms leukopenia and neutropenia may occasionally be used interchangeably, as the neutrophil count is the most important indicator of infection risk. Agranulocytosis is an acute form of ...
Neutropenia is a hematological disorder characterized by an abnormally low number of neutrophils in the blood. Neutrophils usually make up 50-70% of circulating white blood cells and serve as the primary defense against infections.
Signs and symptoms are nonspecific and generally related to the blood cytopenias: Anemia (low RBC count or reduced hemoglobin) – chronic tiredness, shortness of breath, chilled sensation, sometimes chest pain [6] Neutropenia (low neutrophil count) – increased susceptibility to infection [7]
Evans syndrome is an autoimmune disease in which an individual's immune system attacks their own red blood cells and platelets, the syndrome can include immune neutropenia. [1] [2] These immune cytopenias may occur simultaneously or sequentially.