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Chapter 28  Haematological changes in systemic disease  /  391


                      hypocellular or fibrotic. Dysplastic features are   cular haemolysis is marked with haemoglobinuria.


                      common, with ineffective thrombopoiesis or granu-  This may be associated with quinine therapy ( ‘ black-


                      locyte formation accounting at least in part for the   water  fever ’ ).  Thrombocytopenia is commonly
                      cytopenia. The myelodysplasia does not show the   found in acute malaria. Patients with chronic

                      chromosome abnormalities found in classic myelo-  malaria have an anaemia of chronic disorders;
                      dysplasia and does not appear to be pre - leukaemic.   hypersplenism may contribute to the anaemia and
                      Thrombocytopenia is treated if necessary by corti-  result in moderate thrombocytopenia and neutro-

                      costeroids, high - dose gammaglobulin infusions or   penia. Tropical splenomegaly (see p. 144 ) is prob-

                      by other therapies for immune thrombocytopenia   ably a chronic immune reaction to malaria.

                      (see p. 336 ), or by antiretroviral therapy.   Dyserythropoiesis in the marrow, folate defi ciency
                          Anaemia is also common, like the other cytope-  and protein - calorie malnutrition may contribute to
                      nias, and more severe as the disease progresses.   anaemia.
                      It is usually multifactorial in origin  –  the anaemia
                      of chronic disorders, marrow dysplasia and drug
                                                                    Toxoplasmosis
                      therapy, especially zidovudine. Serum vitamin
                      B  12   is often low, most likely because of intestinal    Toxoplasmosis in children and adults is associated
                      malabsorption, but the anaemia does not respond   with lymphadenopathy and large numbers of atypi-
                      to vitamin B  12   therapy. Blood transfusions   cal lymphocytes in the blood. Congenital disease
                      or recombinant erythropoietin injections are   may be confused with hydrops fetalis in a severely
                      needed.                                   anaemic hydropic infant with gross hepatosplenom-
                          Increased plasma cells in the marrow and poly-  egaly, thrombocytopenia or a leucoerythroblastic
                      clonal increase in immunoglobulins are frequent. A   blood fi lm.
                      paraprotein is present in 5 – 10% but appears benign.
                      Non - Hodgkin lymphoma, in over 90% high grade,

                                                                    Kala - a zar ( v isceral  l eishmaniasis)
                      both systemically and in the central nervous system,
                      occurs in HIV infected individuals with over 100    The visceral form of leishmaniasis is associated with

                      times the frequency expected in the general popula-  pancytopenia, hepatosplenomegaly and lymphade-

                      tion. Diffuse large B - cell lymphomas are most   nopathy. Bone marrow or splenic aspirates may

                      common, with 20% confined to the central nervous   show large numbers of parasitized macrophages
                      system; a substantial minority are Burkitt lym-  (Fig.  28.9 ).
                      phoma. Hodgkin lymphoma, usually of poor prog-
                      nosis type, is also increased in frequency. EBV
                                                                    Other  p arasitic  d iseases
                      infection appears to usually underlie this as well as
                      Burkitt lymphoma.                          In the acute phase of both African and South
                           Treatment of lymphomas in the setting of HIV   American trypanosomiasis, organisms are found in
                      is with standard regimens. Continuation of antiret-  the peripheral blood (Fig.  28.10 ). Microfi lariae of

                      roviral therapy exaggerates the tendency to cytope-  bancroftian filariasis and loiasis are also detected

                      nia induced by chemotherapy so prophylaxis against   during blood film examination (Fig.  28.11 ). In
                      opportunistic infections is important. Th e  bone   chronic schistosomiasis, hypersplenism follows the
                      marrow may indeed reveal the presence of oppor-  splenic enlargement associated with portal hyper-
                      tunistic infection (Fig.  28.7 a,b).      tension. In many parasitic diseases there is eosi-
                                                                nophilia (Table  28.5 ).
                          Malaria
                                                                    Osteopetrosis
                        Some degree of haemolysis is seen in all types of

                      malarial infection. The most severe abnormalities     Osteopetrosis is a rare genetic disorder in which there
                      are found in  Plasmodium falciparum  infections (Fig.   is an increase in bone mass with skeletal abnormality
                        28.8 ). In the worst cases, DIC occurs and intravas-  and bone marrow failure. It is caused by defects in a
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