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Chapter 28  Haematological changes in systemic disease  /  387



                      (a heavily glycosylated derivative) or monthly (a   Liver  d isease
                      pegolated derivative). Complications of therapy
                                                                 The haematological abnormalities in liver disease

                      have been initial transient flu - like  symptoms,

                                                                are listed in  Table  28.4 . Chronic liver disease is
                      hypertension, clotting of the dialysis lines and,
                                                                associated with anaemia that is mildly macrocytic

                      rarely, fits. A poor response to erythropoietin sug-
                                                                and often accompanied by target cells, mainly as a

                      gests iron or folate deficiency, infection, aluminium
                                                                result of increased cholesterol in the membrane
                      toxicity or hyperparathyroidism. Intravenous iron is
                                                                (Fig.  28.5 a). Contributing factors to the anaemia
                      often needed to correct iron deficiency shown by

                                                                may include blood loss (e.g. bleeding varices) with
                      serum ferritin, percentage saturation of total iron -


                                                                iron deficiency, dietary folate deficiency and direct
                        binding capacity or percentage hypochromic red
                                                                suppression of haemopoiesis by alcohol.
                      cells in the blood.
                                                                    Haemolytic anaemia may occur in patients with
                                                                alcohol intoxication (Zieve  s syndrome) (Fig.  28.5 b)
                                                                                     ’
                                                                and in Wilson  s disease (caused by copper oxidation
                                                                           ’
                          Platelet and  c oagulation  a bnormalities   of red cell membranes); autoimmune haemolytic
                                                                anaemia is found in some patients with chronic
                        A bleeding tendency with purpura, gastrointestinal
                                                                immune hepatitis. Haemolysis may also occur in
                      or uterine bleeding occurs in 30 – 50% of patients
                                                                end - stage liver disease because of abnormal red cell
                      with chronic renal failure and is marked in patients
                                                                membranes resulting from lipid changes. Viral hep-

                      with acute renal failure. The bleeding is out of pro-
                      portion to the degree of thrombocytopenia and has
                      been associated with abnormal platelet or vascular

                      function, which can be reversed by dialysis.       Table 28.4   Haematological abnormalities in
                      Correction of the anaemia with erythropoietin also   liver disease.
                      improves the bleeding tendency. Immune complex -
                                                                        Liver failure  ±  obstructive jaundice  ±  portal
                        mediated thrombocytopenia occurs in some patients
                                                                  hypertension
                      with acute nephritis, SLE and polyarteritis nodosa
                                                                        Refractory anaemia –  usually mildly macrocytic,

                      and also following renal allografts. Renal allografts
                                                                  often with target cells; may be associated with:
                      may also lead to polycythaemia in 10 – 15% of
                                                                      Blood loss and iron defi ciency
                      patients.
                                                                      Alcohol ( ±  ring sideroblastic change)

                          The haemolytic uraemic syndrome and throm-      Folate defi ciency
                      botic thrombocytopenic purpura are discussed on       Haemolysis (e.g. Zieve ’ s syndrome, Wilson ’ s

                      p. 337 . Patients with the nephrotic syndrome have   disease, immune hypersplenism from portal
                      an increased risk of venous thrombosis.       hypertension)
                                                                        Bleeding tendency
                                                                      Defi ciency of vitamin K - dependent factors; also
                          Congestive  h eart  f ailure            of factor V and fi brinogen
                                                                      Thrombocytopenia hypersplenism, immune
                        Anaemia is common in congestive heart failure due   platelet function defects

                      to a variety of causes. These include haemodilution,       Functional abnormalities of fi brinogen
                      chronic kidney disease, release of cytokines increas-      Increased fi brinolysis
                      ing hepcidin synthesis and so reducing iron absorp-      Portal hypertension  –  haemorrhage from varices
                      tion and recycling of iron from macrophages, and
                                                                        Viral hepatitis
                      reducing erythropoetin secretion and erythropoie-
                                                                      Aplastic anaemia
                      tin responsiveness of erythroblasts. Iron defi ciency
                      may develop. Treatment with oral or intravenous         Tumours
                      iron may reduce anaemia, fatigue and increase       Polycythaemia
                      cardiac function, exercise capacity and quality of       Neutrophil leucocytosis and leukaemoid
                                                                  reactions
                      life.
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