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1368   PART XIII   Hematology



                          CHAPTER                               84
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                                        Erythrocytosis

















            DEFINITION AND CLASSIFICATION                        concentrations. Recently, JAK2 mutations were identified
                                                                 in a dog with PRV (Beurlet et al., 2011); this mutation is
            Erythrocytosis is defined as an increase in the circulating red   common in humans with PRV. In a recent study, the clinical
            blood cell (RBC) mass and is manifested hematologically as   and hematologic features of 18 cats with primary erythrocy-
            an increase in the packed cell volume (PCV) or hematocrit   tosis were reviewed (Darcy et al., 2018).
            (HCT) above the reference interval (RI). Because determina-  Secondary erythrocytosis results from increased ortho-
            tion of the RBC mass in a clinical setting is cumbersome and   topic (produced by the kidneys) or heterotopic (produced
            impractical, a diagnosis of erythrocytosis is typically made   in sites other than the kidneys) Epo production. Ortho-
            on the basis of the high HCT, not an increased RBC mass.   topic (physiologically appropriate) Epo production occurs
            Certain dog breeds, such as most sighthounds, have HCT   in response to tissue hypoxia, such as that occurring at a
            values above the RI for the species; this also occurs in dogs   high altitude and in the settings of chronic cardiopulmonary
            that live at high altitudes. For example, normal actively   disease, right-to-left cardiovascular shunts, and carboxyhe-
            racing or retired racing Greyhounds can have an HCT as   moglobinemia. Tumor-associated erythrocytosis (hetero-
            high as 70%. An increase in the RBC numbers may lead to   topic or orthotopic Epo production) has been observed in
            severe hemorheologic alterations, resulting in clinical signs   humans with a wide variety of neoplasms, in dogs with renal
            secondary to hyperviscosity. Although the term polycythe-  masses, and in dogs with spindle cell sarcomas (e.g., nasal
            mia is commonly used to refer to this hematologic abnor-  fibrosarcoma, schwannoma, cecal gastrointestinal stromal
            mality, it is incorrect because the term actually means an   tumor). In a recent retrospective study we conducted,
            increase in the numbers of all circulating cells (“poly” means   15/29 (51%) of dogs with renal lymphoma had erythrocy-
            multiple).                                           tosis. Hormonal stimuli may also trigger erythrocytosis in
              On the basis of its pathogenesis, erythrocytosis can be   animals with normal tissue oxygenation, such as in dogs with
            classified as relative or absolute (Box 84.1). The term relative   hyperadrenocorticism and cats with hyperthyroidism. At the
            erythrocytosis refers to hemoconcentration (i.e., dehydra-  author’s clinic, secondary erythrocytosis is more common in
            tion) and is characterized by an increased PCV, usually in   dogs, and PRV is more common in cats. However, erythrocy-
            association with an increased serum or plasma protein con-  tosis is rare in both species. Interestingly, although infiltrative
            centration; in dogs and cats with relative erythrocytosis, the   renal diseases (e.g., lymphoma, feline infectious peritonitis)
            RBC mass is normal. Dogs with hemorrhagic gastroenteritis   are common in cats, they rarely result in secondary eryth-
            (HGE) frequently have relative erythrocytosis associated   rocytosis. Recently a cat with a renal adenocarcinoma and
            with  normal  serum  or  plasma  protein  concentration;  the   secondary erythrocytosis was reported.
            reason for the lack of increase in the protein concentration
            is unknown, but the erythrocytosis resolves with appropriate   Clinical and Clinicopathologic Findings
            fluid therapy. In absolute or true erythrocytosis, the RBC   The clinical signs may occur acutely and consist primarily of
            mass is increased; it can be classified as primary or secondary   functional abnormalities of the central nervous system (e.g.,
            depending on the pathogenesis and serum erythropoietin   behavioral, motor, or sensory changes; seizures). In cats,
            (Epo) concentration or activity.                     signs of a transverse myelopathy are common; in the retro-
              Primary erythrocytosis (polycythemia rubra vera [PRV])   spective study of 18 cats with erythrocytosis, the most
            results from an autonomous, Epo-independent proliferation   common clinical signs were seizures and mentation changes
            of RBC precursors in the bone marrow and is considered   (Darcy et al., ePub ahead of print). A common manifestation
            a myeloproliferative disease. As a consequence, most dogs   of erythrocytosis in dogs is paroxysmal sneezing, likely
            and cats with PRV have low to nondetectable serum Epo   caused by increased blood viscosity in the nasal mucosa.

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