Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recovery of bone marrow function in aplastic anemia patients treated with immunosuppressive therapy first suggested a role for the immune system in bone marrow failure. High recovery rates in patients treated with immunosuppressive therapy suggested that an immune mechanism may be a final common pathway of marrow failure in this disease. In vitro studies have shown that aplastic peripheral blood and marrow cells and their supernatants are capable of suppressing hematopoiesis by autologous and normal marrow. Soluble factors identified in this system include gamma interferon and lymphotoxin. The interaction of these molecules with positive growth factors, the role of synergy with other negative regulators, and their role in the pathogenesis of bone marrow failure are discussed. Lymphokine and lymphocyte abnormalities in aplastic anemia may be manifestations of an underlying viral etiology. Three examples are discussed: Epstein-Barr virus-associated aplastic anemia; B19 parvovirus bone marrow failure; and HIV-induced neutropenia.
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PMID:Autoimmune aspects of aplastic anemia. 297 24

This study reports the characterization of a spontaneous lymphoblastoid cell line (LCL) raised from the peripheral blood of a patient with Kostmann's congenital neutropenia. The LCL was composed of EBV-infected polyclonal B cells and displayed surface markers and pattern of growth in vitro typical of normal LCLs. The supernatant of the LCL contained a colony inhibiting activity (CIA) that decreased the cloning efficiency of normal committed haemopoietic progenitors and was identified as immunoreactive transforming growth factor beta 1 (TGF-beta 1) by neutralization experiments with a specific antiserum. Control studies with a panel of LCLs spontaneously derived from the peripheral blood of patients seropositive for Epstein-Barr virus (EBV) infections showed that 5/30 LCLs produced a CIA. This CIA was not identifiable as TGF-beta 1 but rather was due to the combined effects of tumour necrosis factor alpha (TNF alpha), tumour necrosis factor beta (TNF beta) and interferon alpha (IFN alpha), that were present in the LCL supernatants. The hypothesis that the B cells latently infected by EBV in vivo and possibly expanded as a consequence of the infection may have contributed to the inhibition of the patient granulopoiesis by releasing TGF-beta 1 will be discussed.
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PMID:Transforming growth factor beta-1 (TGF-beta 1) released by an Epstein-Barr virus (EBV) positive spontaneous lymphoblastoid cell line from a patient with Kostmann's congenital neutropenia inhibits the growth of normal committed haemopoietic progenitors in vitro. 791 30