Acquired aplastic anemia and paroxysmal nocturnal hemoglobinuria: studies on clonality [see comments]
| Autor: | T. R. Rutherford, Jennifer A. Tooze, Borthwick-Clarke C, K. M. Josten, Edward C. Gordon-Smith |
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| Rok vydání: | 1991 |
| Předmět: |
Lymphocyte
Population Immunology Biology Biochemistry hemic and lymphatic diseases White blood cell medicine Aplastic anemia education Decay-accelerating factor education.field_of_study business.industry Myeloid leukemia Cell Biology Hematology medicine.disease Haematopoiesis medicine.anatomical_structure Hypoxanthine-guanine phosphoribosyltransferase Monoclonal Paroxysmal nocturnal hemoglobinuria Stem cell Clone (B-cell biology) business |
| Zdroj: | Blood. 78:3162-3167 |
| ISSN: | 1528-0020 0006-4971 |
| Popis: | DIOPATHIC, ACQUIRED aplastic anemia (AA) is a I failure of the hematopoietic system affecting multiple lineages. The pathogenesis is not understood, but may involve intrinsic failure of the hematopoietic stem cells, disturbance of the hematopoietic microenvironment, immunological suppression, or a combination of factors.’ More than 50% of patients may enter (partial) remission following therapy, but on long-term follow-up late complications may arise in up to 57% of cases.‘ Abnormal populations of hematopoietic cells arise, including myelodysplastic (MDS), acute myeloid leukemia (AML), and, in particular, paroxysmal nocturnal hemoglobinuria (PNH) cells.334 AA is not usually thought to involve a clonal proliferation within the hematopoietic system with suppression of normal proliferation, but this has not been directly investigated. PNH, in contrast, is generally assumed to be a clonal stem cell disorder: although the direct evidence for this is limited. PNH presents with a variety of clinical syndromes and is commonly associated with preexisting AA (for recent reviews see ref 4-6). Among the most typical features of PNH are nocturnal episodes of intravascular hemolysis, an increased risk for thrombotic events, and a tendency for infections, but the PNH cells may be detected only as a laboratory phenomenon arising in patients with AA. PNH red blood cells (RBCs) are abnormally susceptible to complement-mediated lysis. It has been shown that at least nine different membrane proteins are deficient or completely lacking in the “PNH cells” of both RBC and white blood cell (WBC) lineages. At least three of these proteins are involved in the regulation of complement activity and |
| Databáze: | OpenAIRE |
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