TAILIEUCHUNG - Chapter 061. Disorders of Granulocytes and Monocytes (Part 6)

Tham khảo tài liệu 'chapter 061. disorders of granulocytes and monocytes (part 6)', y tế - sức khoẻ, y học thường thức phục vụ nhu cầu học tập, nghiên cứu và làm việc hiệu quả | Chapter 061. Disorders of Granulocytes and Monocytes Part 6 Hereditary Neutropenias Hereditary neutropenias are rare and may manifest in early childhood as a profound constant neutropenia or agranulocytosis. Congenital forms of neutropenia include Kostmann s syndrome neutrophil count 100 pL which is often fatal due to mutations in the anti-apoptosis gene HAX-1 severe chronic neutropenia neutrophil count of 300-1500 pL due to mutations in neutrophil elastase hereditary cyclic neutropenia or more appropriately cyclic hematopoiesis also due to mutations in neutrophil elastase the cartilage-hair hypoplasia syndrome due to mutations in the mitochondrial RNA-processing endoribonuclease RMRP Shwachman-Diamond syndrome associated with pancreatic insufficiency due to mutations in the Shwachman-Bodian-Diamond syndrome gene SBDS the WHIM warts hypogammaglobulinemia infections myelokathexis retention of WBCs in the marrow syndrome characterized by neutrophil hypersegmentation and bone marrow myeloid arrest due to mutations in the chemokine receptor CXCR4 and neutropenias associated with other immune defects such as X-linked agammaglobulinemia Wiskott-Aldrich syndrome and CD40 ligand deficiency. Mutations in the G-CSF receptor can develop in severe congenital neutropenia and are linked to leukemia. Maternal factors can be associated with neutropenia in the newborn. Transplacental transfer of IgG directed against antigens on fetal neutrophils can result in peripheral destruction. Drugs . thiazides ingested during pregnancy can cause neutropenia in the newborn by either depressed production or peripheral destruction. In Felty s syndrome the triad of rheumatoid arthritis splenomegaly and neutropenia Chap. 314 spleen-produced antibodies can shorten neutrophil life span while LGLs can attack marrow neutrophil precursors. Splenectomy may increase neutrophil count in Felty s syndrome and lower serum neutrophil-binding IgG. Some Felty s syndrome patients also have neutropenia .

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