Selected bibliography

Acquired haemolytic anaemia - general

Dacie JV (1992) The Haemolytic Anemias, Vol. 3. The Autoimmune Haemolytic Anaemias, 3rd edn. Churchill Livingstone, Edinburgh.

Dacie JV (1995) The Haemolytic Anaemias, Vol. 4. Secondary or Symptomatic Haemolytic Anaemias, 3rd edn. Churchill Livingstone, Edinburgh.

Autoimmune haemolytic anaemia

Ekvall H (2003) Malaria and anemia. Current Opinion in Hemato-logy 10:108-14.

Gharib M, Poynton C (2002) Complete, long-term remission of refractoryidiopathic cold haemagglutinin disease after Mabthera. British Journal of Haematology 117:247-8.

Hall GW (2001) Kassabach-Merritt syndrome: pathogenesis and management. British Journal of Haematology 112: 851-62.

Iannitto E, Ammatuna E, Marino C et al. (2002) Sustained response of refractory chronic lymphocytic leukaemia complicated by acute haemolytic anaemia to anti-CD20 monoclonal antibody. Blood 99:1096-7.

Jeffries LC (1994) Transfusion therapy in autoimmune hemolytic anemia. Hematology-Oncology Clinics of North America S: 1087104.

Marani TM, Trich MB, Armstrong PM (1996) Carboplatin induced immune hemolytic anaemia. Transfusion 36:1016-18.

Myint H, Copplestone JA, Orchard J etal. (1995) Fludarabine-related autoimmune haemolytic anaemia in patients with chronic lymphocytic leukaemia. British Journal of Haematology 91:341-4.

Petz LD (1993) Drug-induced autoimmune hemolytic anemia. Transfusion Medicine Review 7:242-54.

Salama A, Kroll H, Wittmann G etal. (1996) Diclof enac-induced immune haemolytic anaemia: simultaneous occurence of red blood cell autoantibodies and drug dependent antibodies. British Journal of Haematology 95: 640-4.

Treon SP and Anderson KC (2000) The use of rituximab in the treatment of malignant and non-malignant plasma cell disorders. Seminars in Oncology 27: 79-85.

Mechanical haemolytic anaemias

Aqui NA, Stein SH, Konkle BA et al. (2003) Role of splenectomy in patients with refractory or relapsed thrombotic thrombocytopenic purpura. Journal of Clinical Apheresis 18: 51-4.

Davidson RJL (1969) March or exertional haemoglobinuria. Seminars in Hematology 6:150.

Fontana S, Kremer Hovinga JA, Studt J-D etal. (2004) Plasma therapy in thrombotic thrombocytopenic purpura: review of the literature and the Bern experience in a subgroup of patients with severe acquired ADAMTS-13 deficiency. Seminars in Hematology 41:48-59.

Levy GC, Nichols WC, Lian EC etal. (2001) Mutations in a member of the ADAMTS gene family cause thrombotic thrombocyto-penic purpura. Nature 413:488-94.

Rock GA, Shumack, Buskard NA etal. (1991) Comparison of plasma exchange with plasma infusion in the treatment of throm-botic thrombocytopenic purpura. The Canadian Apheresis Study Group. New England Journal of Medicine 325: 393-7.

Yarranton H, Machin SJ (2003) An update on the pathogenesis and management of acquired thrombotic thrombocytopenic purpura. Current Opinion in Neurology 16: 367-73.

Introduction, 169 Clinical features, 169 Laboratory findings and diagnosis, 170 Pathogenesis and pathophysiology, 171

Haemolytic anaemia, 171 Molecular analysis, 172

Thrombosis, 173

Bone marrow failure and relationship between paroxysmal nocturnal haemoglobinuria and acquired aplastic anaemia (AAA), 173 Management of paroxysmal nocturnal haemoglobinuria, 174

Supportive treatment, 174 Bone marrow transplantation and immunosuppressive treatment, 174 A new approach to controlling haemolysis, 175 Selected bibliography, 175

Introduction, 169 Clinical features, 169 Laboratory findings and diagnosis, 170 Pathogenesis and pathophysiology, 171

Haemolytic anaemia, 171 Molecular analysis, 172

Thrombosis, 173

Bone marrow failure and relationship between paroxysmal nocturnal haemoglobinuria and acquired aplastic anaemia (AAA), 173 Management of paroxysmal nocturnal haemoglobinuria, 174

Supportive treatment, 174 Bone marrow transplantation and immunosuppressive treatment, 174 A new approach to controlling haemolysis, 175 Selected bibliography, 175

Figure 11.1 Consecutive urine samples from a patient with PNH. The patient had stated that she had had haemoglobinuria in the past, but not at the moment. The figure illustrates the marked variability in haemoglobinuria over a short time interval and that subjective self-assessment of haemoglobinuria may be deceptive. After this test, the same patient's assessment of her haemoglobinuria became more reliable.

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired chronic haemolytic anaemia in which haemolysis is largely intravascular. In addition to haemolytic anaemia, the patient may have thrombosis and pancytopenia. This triad, when present, is highly characteristic of PNH.

PNH is a rare disorder (estimated prevalence of less than 1 in 100 000) that can occur anywhere in the world. There is no evidence of family clustering and PNH has been observed at all ages from 1 to 72 years; however, it is most common in young adults.

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