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Chapter 28  Haematological changes in systemic disease  /  385


                      bleeding, interruptions with  chemotherapy  and     In systemic lupus erythematosus (SLE) there
                      thrombocytopenia, anorexia or vomiting. Liver   may be anaemia of chronic disorders and 50% of
                      disease and drug interactions can cause further   patients are leucopenic with reduced neutrophil and
                      complications so daily low molecular weight heparin   lymphocyte counts often associated with circulating
                      injections may be preferable to oral anticoagulants.       immune complexes. Renal impairment and drug -
                                                                  induced gastrointestinal blood loss also contribute
                          Rheumatoid  a rthritis (and  o ther   to the anaemia. Autoimmune haemolytic anaemia
                        c onnective  t issue  d isorders)       (typically with immunoglobulin G (IgG) and the
                                                                C3 component of complement on the surface of the
                        In patients with rheumatoid arthritis, the anaemia
                                                                red cells) occurs in 5% of patients and may be the
                      of chronic disorders is proportional to the severity
                                                                presenting feature of the syndrome. There may be

                      of the disease. It is complicated in some patients
                                                                autoimmune thrombocytopenia in 5% of patients.

                      by iron deficiency caused by gastrointestinal bleed-
                                                                The lupus anticoagulant is described on p. 369  . Th is

                      ing related to therapy with salicylates, other non -
                                                                circulating anticardiolipin interferes with blood
                       steroidal anti - inflammatory agents or corticosteroids.

                                                                coagulation by altering the binding of coagulation

                      Bleeding into inflamed joints may also be a factor.
                                                                factors to platelet phospholipid and predisposes to
                      Marrow hypoplasia may follow therapy with gold.
                                                                both arterial and venous thrombosis and recurrent
                            ’
                      In Felty  s syndrome, splenomegaly is associated with
                                                                abortions. The antibody may be responsible for a

                      neutropenia (Fig.  28.3 ). Anaemia and thrombocy-
                                                                false positive Wassermann reaction. Tests for anti-
                      topenia may also be present.
                                                                nuclear factor and anti - DNA antibodies are usually
                                                                positive.
                                                                     Patients with temporal arteritis and polymyalgia
                                                                rheumatica have a markedly elevated ESR, pro-
                                                                nounced red cell rouleaux in the blood film and a

                                                                polyclonal immunoglobulin response. Th ese and
                                                                other collagen vascular disorders are associated with
                                                                anaemia of chronic disorders.
                                                                    Renal  f ailure
                                                                    Anaemia
                       (a)                                        A normochromic anaemia is present in most
                                                                patients with chronic renal failure. Generally, there

                                                                is a 2  g/dL fall in haemoglobin level for every


                                                                10  mmol/L rise in blood urea. There is impaired red
                                                                cell production as a result of defective erythropoi-
                                                                etin secretion (see Fig.  2.5 ). Uraemic serum has also


                                                                been shown to contain factors that inhibit prolifera-
                                                                tion of erythroid progenitors but, in view of
                                                                the excellent response to erythropoietin in most
                                                                patients, the clinical relevance of these is doubtful.
                                                                Variable shortening of red cell lifespan occurs and
                                                                in severe uraemia the red cells show abnormalities
                       (b)
                                                                including spicules (spurs) and  ‘ burr ’  cells (Fig.
                                Figure 28.3   Felty ’ s syndrome:  (a)  the typical     28.4 ). Increased red cell 2,3 - diphosphoglycerate


                      deformities of rheumatoid arthritis of the hand; and   (2,3 - DPG) levels in response to the anaemia and
                        (b)  splenomegaly.                      hyperphosphataemia result in decreased oxygen
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