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1238   PART XI   Immune-Mediated Disorders


            immunosuppressive  drugs.  The  remaining  dogs  require   PURE RED CELL APLASIA
            long-term immunosuppressive therapy. Factors that clini-
  VetBooks.ir  cally appear to confer a good prognosis in dogs with IMHA   PRCA is a rare disorder characterized by severe, nonregen-
                                                                 erative anemia with marked depletion or absence of ery-
            include a rapid response to treatment with glucocorticoids,
            ability to maintain the packed cell volume above 30% with
                                                                 evidence of concurrent peripheral hemolysis is present on
            glucocorticoids alone, and identification of a treatable sec-  throid precursors in the bone marrow. In some cases,
            ondary cause.                                        the basis of the presence of spherocytes and a positive direct
              The prognosis is guarded in dogs that require multiple   Coombs test. Other cell lines are usually normal. The ery-
            drugs to control the disease and those with persistent auto-  throid aplasia in PRCA is in contrast to the nonregenerative
            agglutination, azotemia, an elevated bilirubin concentration,   form of IMHA, in which there is erythroid hyperplasia or
            marked thrombocytopenia, and severe leukocytosis. In a   sometimes maturation arrest of the erythroid maturation
            multicenter study, prognostic factors for canine IMHA were   sequence at the rubricyte or metarubricyte level. PRCA is
            investigated, and a model using the presence of SIRS, bili-  likely one end of the spectrum of IMHA, with acute periph-
            rubin, urea, and creatinine concentrations, and ASA score   eral hemolysis at the other end of this spectrum (Table 73.4).
            was predictive of outcome in 80% of cases. In this model,   The affinity of circulating antibody for different erythroid
            bilirubin concentration and azotemia were independently   precursors likely influences the level at which damage occurs
            associated with death. Reported mortality rates of dogs with   in the bone marrow. As with IMHA, both primary and sec-
            primary IMHA range from 26% to 70%; thromboembo-     ondary forms of PRCA are recognized. Secondary causes of
            lism  is  the  cause  of  death  in  30%  to 60%  of  cases.  Other   PRCA include treatment with recombinant human erythro-
            common causes of death include infection due to immu-  poietin and parvovirus infection in dogs. Infection with
            nosuppression, DIC, and failure to control anemia. Inter-  feline leukemia virus subtype C is a cause of PRCA in cats.
            estingly, patients that are hypercoagulable as assessed by   Dogs with PRCA have a similar signalment and present
            TEG have a better prognosis than those that have normal   with similar clinical signs as dogs with IMHA. As is the case
            coagulability; it has been proposed that, in these dogs, there   with primary IMHA, cats with primary PRCA are typically
            is an underlying consumptive coagulopathy. If a major TE   younger than dogs, with an age range of 8 months to 3 years.
            does occur in a dog with IMHA, particularly if the blood   Dogs and cats with PRCA have severe, nonregenerative
            supply to a major organ is disrupted, the long-term prog-  anemia, whereas the platelet count and leukogram are typi-
            nosis is poor. Contrary to popular opinion, the prognosis   cally normal. In contrast to IMHA, the biochemical panel
            in Cocker Spaniels with IMHA does not differ from that of    and  urinalysis  are  also  usually  unremarkable,  with  no
            other breeds.                                        evidence of peripheral hemolysis or inflammation. Low
                                                                 numbers of spherocytes are sometimes present in dogs with
                                                                 PRCA. The Coombs test is usually negative.
            FELINE IMMUNE-MEDIATED                                 Diagnosis of PRCA is made by evaluation of a bone
            HEMOLYTIC ANEMIA                                     marrow aspirate and bone marrow core biopsy. In PRCA,
                                                                 erythroid precursors are rare or absent and the M/E ratio is
            Cats with primary IMHA tend to be younger than dogs, with   high (>99 : 1). In contrast to nonregenerative IMHA, severe
            a median age of 2 years. Males are slightly overrepresented,   myelofibrosis is rare.
            with no influence of neuter status. Secondary causes for   Treatment of PRCA is similar to IMHA. In the authors’
            antierythrocyte antibody formation are identified more fre-  experience, most dogs with PRCA respond to prednisolone
            quently in cats, with feline leukemia virus and Mycoplasma   alone; however, other drugs are commonly added to the
            haemofelis being common infections associated with IMHA.   treatment protocol because of the length of time it takes to
            Primary IMHA in cats is more likely to present with a non-  see a response in some dogs. Adjunctive immunosuppressive
            regenerative anemia, accounting for about 50% of cases.  medications have been used with success in dogs that have
              Most cats with IMHA respond to prednisolone alone, and   an incomplete response to prednisolone alone. These adjunc-
            the adverse effects of glucocorticoids are less severe. In cats   tive medications include azathioprine, cyclophosphamide,
            that need an additional immunosuppressive drug to treat   cyclosporine, and mycophenolate. The time taken to achieve
            IMHA, treatment with chlorambucil, cyclosporine, or myco-  complete remission (2-6 months) is longer in dogs with
            phenolate should be considered. Not enough published   PRCA compared with IMHA, and it is sometimes difficult
            information exists to recommend one drug over another.   to judge whether a particular protocol is failing or whether
            Azathioprine is not recommended in cats because of the risk   inadequate time has been allowed for the bone marrow to
            of unacceptable adverse effects (see Chapter 72). The authors   respond to treatment and begin to produce and release RBCs
            typically use chlorambucil or cyclosporine in cats that need   into the circulation. Sequential bone marrow evaluations
            an additional immunosuppressive drug. There is an increased   should  ideally  be  used  to  determine  when  to  change  the
            risk of diabetes mellitus in cats treated with glucocorticoids;   treatment protocol. A repeat bone marrow aspirate should
            in these cats a second immunosuppressive drug should be   be considered after 2 months of treatment if no improvement
            added to allow tapering and ultimately discontinuation of   in the anemia is observed. Repeated transfusion of pRBCs
            glucocorticoids.                                     or whole blood is necessary while waiting for a response to
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