- 1 On being a patient
- 2 Modern medicine: foundations, achievements, and limitations
- 3 Global patterns of disease and medical practice
- 4 Cell biology
- 5 Immunological mechanisms
- 6 Principles of clinical oncology
- 7 Infection
- 8 Sexually transmitted diseases and sexual health
- 9 Chemical and physical injuries and environmental factors and disease
- 10 Clinical pharmacology
- 11 Nutrition
- 12 Metabolic disorders
- 13 Endocrine disorders
- 14 Medical disorders in pregnancy
- 15 Gastroenterological disorders
- 16 Cardiovascular disorders
- 17 Critical care medicine
- 18 Respiratory disorders
- 19 Rheumatological disorders
- 20 Disorders of the skeleton
- 21 Disorders of the kidney and urinary tract
- 22 Disorders of the blood
- 22.1 Introduction
- 22.2 Haemopoietic stem cells
- 22.3 The leukaemias and other bone marrow disorders
- 22.3.1 Cell and molecular biology of human leukaemias
- 22.3.2 The classification of leukaemia
- 22.3.3 Acute lymphoblastic leukaemia
- 22.3.4 Acute myeloid leukaemia
- 22.3.5 Chronic lymphocytic leukaemia
- 22.3.6 Chronic myeloid leukaemia
- 22.3.7 Myelodysplasia
- 22.3.8 The polycythaemias
- 22.3.9 Primary myelofibrosis
- 22.3.10 Thrombocytosis
- 22.3.11 Aplastic anaemia and pure red cell aplasia
- 22.3.12 Paroxysmal nocturnal haemoglobinuria
- 22.4 The white cells and lymphoproliferative disorders
- 22.5 The red cell
- 22.6 Haemostasis and thrombosis
- 22.7 The blood in systemic disease
- 22.8 Blood replacement
- 23 Disorders of the skin
- 24 Neurological disorders
- 25 The eye
- 26 Psychiatry and drug related problems
- 27 Forensic medicine
- 28 Sports medicine
- 29 Geratology
- 30 Pain
- 31 Palliative medicine
- 32 Biochemistry in medicine
- 33 Acute medicine
(p. 4288) (p. 4289) (p. 4290) (p. 4291) (p. 4292) (p. 4293) (p. 4294) (p. 4295) (p. 4296) (p. 4297) Aplastic anaemia and pure red cell aplasia
- Chapter:
- (p. 4288) (p. 4289) (p. 4290) (p. 4291) (p. 4292) (p. 4293) (p. 4294) (p. 4295) (p. 4296) (p. 4297) Aplastic anaemia and pure red cell aplasia
- Author(s):
Judith C.W. Marsh
, Austin Kulasekararaj
, and Ghulam J. Mufti
- DOI:
- 10.1093/med/9780199204854.003.220311_update_001
August 28, 2014: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.
Update:
This chapter has been completely rewritten (Nov 2012), to reflect in particular an increased awareness of inherited forms of AA/bone marrow failure (BMF) disorders, better understanding of the immunological changes that occur in acquired, immune-mediated AA showing the important role of CD4+ T-cells in the pathogenesis, improved clinical outcomes following haemopoietic stem cell transplantation (HSCT) for severe AA, updated information on the clinical investigation of pure red cell aplasia (PRCA), and recent changes in treatment approach to acquired PRCA, and recent advances in detection of molecular and genetic changes that are found in inherited BMF disorders.
Aplastic anaemia (AA) is a rare bone marrow failure (BMF) disorder characterized by pancytopenia and a hypocellular bone marrow. AA is commonly acquired, immune-mediated and idiopathic in nature.
Activate auto-reactive, cytotxic CD8+ T-cells are present but recent work has shown that CD4+ T-cells appear to be more important in the pathogenesis of acquired AA. The immune nature of acquired AA provides the rationale for one of the treatment options, namely immunosuppressive therapy....
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- 1 On being a patient
- 2 Modern medicine: foundations, achievements, and limitations
- 3 Global patterns of disease and medical practice
- 4 Cell biology
- 5 Immunological mechanisms
- 6 Principles of clinical oncology
- 7 Infection
- 8 Sexually transmitted diseases and sexual health
- 9 Chemical and physical injuries and environmental factors and disease
- 10 Clinical pharmacology
- 11 Nutrition
- 12 Metabolic disorders
- 13 Endocrine disorders
- 14 Medical disorders in pregnancy
- 15 Gastroenterological disorders
- 16 Cardiovascular disorders
- 17 Critical care medicine
- 18 Respiratory disorders
- 19 Rheumatological disorders
- 20 Disorders of the skeleton
- 21 Disorders of the kidney and urinary tract
- 22 Disorders of the blood
- 22.1 Introduction
- 22.2 Haemopoietic stem cells
- 22.3 The leukaemias and other bone marrow disorders
- 22.3.1 Cell and molecular biology of human leukaemias
- 22.3.2 The classification of leukaemia
- 22.3.3 Acute lymphoblastic leukaemia
- 22.3.4 Acute myeloid leukaemia
- 22.3.5 Chronic lymphocytic leukaemia
- 22.3.6 Chronic myeloid leukaemia
- 22.3.7 Myelodysplasia
- 22.3.8 The polycythaemias
- 22.3.9 Primary myelofibrosis
- 22.3.10 Thrombocytosis
- 22.3.11 Aplastic anaemia and pure red cell aplasia
- 22.3.12 Paroxysmal nocturnal haemoglobinuria
- 22.4 The white cells and lymphoproliferative disorders
- 22.5 The red cell
- 22.6 Haemostasis and thrombosis
- 22.7 The blood in systemic disease
- 22.8 Blood replacement
- 23 Disorders of the skin
- 24 Neurological disorders
- 25 The eye
- 26 Psychiatry and drug related problems
- 27 Forensic medicine
- 28 Sports medicine
- 29 Geratology
- 30 Pain
- 31 Palliative medicine
- 32 Biochemistry in medicine
- 33 Acute medicine