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Histiocytic Diseases   475


           Chronic Treatment                   •  Three-view thoracic radiographs and physical   diagnosing DC-origin disorders and ruling
           •  Reactive  histiocytoses  are  typically  treated   exam, including lymph node palpation and   out lymphoma. Polymerase chain reaction
  VetBooks.ir  prednisone 2 mg/kg PO q 24h is used until   3 months for the first year after diagnosis     (PARR) may not adequately distinguish   Diseases and   Disorders
             with immunosuppressive therapy. Initially,
                                                                                      (PCR) for antigen receptor rearrangement
                                                exam of the site, should be performed every
                                                                                      HS from lymphoma because of infiltrating
             clinical response is noted; then a slow taper
                                                for localized HS and every 4-6 months
             of 1 mg/kg PO q 24h for 1 month, 0.5 mg/
                                                                                      clonal T cells in HS.
             kg PO q 24h for 1 month, 0.5 mg/kg PO q   thereafter.                  ○   Newer markers include ionized calcium
             48h for 3 months, and then stop or maintain    PROGNOSIS & OUTCOME       binding adaptor molecule 1 (IBA-1) with
             at lowest effective dose.                                                good sensitivity and specificity and CD204
           •  If no response is noted within 1 month of   •  CH:  good;  may  regress  spontaneously  or   with conflicting reports of utility.
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             prednisone therapy or lesions relapse and no   respond to immunosuppressive doses of   ○   Dendritic cells should be CD1a  or CD1c ,
                                                                                                     +
                                                                                           +
             longer respond to prednisone, azathioprine   prednisone                  CD11c , and MHC II  (Langerhans DCs
             2 mg/kg PO q 24h, with the same taper   •  SH  and  disseminated  HS:  poor.  SH  may   are associated with E-cadherin positivity
             schedule as prednisone or cyclosporine may   wax and wane but is ultimately progres-  as in most histiocytomas, and interstitial
                                                                                                +
             be added.                          sive, whereas disseminated HS is rapidly   DCs are CD90 )
           •  Evidence suggests efficacy of tetracycline and   progressive, with both leading to death.   ○   Macrophages (hemophagocytic HS) should
                                                                                                     −
                                                                                                               +
             niacinamide (dogs < 10 kg: 250 mg of each   Disseminated HS is considered uniformly   be CD 1 low/− , CD11c , and CD11d  as
             drug PO q 8h, and dogs > 10 kg: 500 mg   fatal. Survival of dogs with SH ranges from   well as lysozyme positive.
             of each drug PO q 8h).             months to years. Survival for dogs with dis-  ○   Malignant histiocytic diseases should be
                                                                                         −
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           •  All treatments can be used with or without   seminated HS averages 4 months, although   CD4 , and reactive syndromes, CD4 .
             antibiotics, depending on appearance of skin   chemotherapy in the adjuvant setting with
             lesions and at the discretion of the treating   CCNU may prolong survival to greater than   Prevention
             clinician.                         1 year for some dogs (see Treatment above).   Because histiocytic diseases are often inherited,
           •  Systemic malignant histiocytic diseases are   Prognosis to date for cats with malignant   careful documentation of pedigrees for affected
             poorly responsive to therapy, and remissions,   histiocytosis is similarly grave, and response   animals may reduce disease through selective
             if achieved, are typically short lived.  to therapy has been poor.   breeding. Inheritance of histiocytosis in BMDs
                             2
             ○   CCNU 60-90 mg/m  (p. 609); 29%-46%   •  Focal histiocytic sarcoma: good if low grade   has been calculated at a moderate 0.298, sug-
               response with macroscopic disease. Dogs   and local control is achieved with surgery   gesting that careful breeding could be effective
               with metastasis limited to lymph nodes   ± radiation therapy. Periarticular HS has a   at eliminating this disease. This means that
               responded favorably (median survival time,   better prognosis than that at other sites,   29.8% of the risk of developing histiocytosis
               219 days; 38% 1-year survival). CCNU   even with suspected metastasis at the time   in the population of BMDs is attributable
               has been used in combination with other   of diagnosis. CNS HS has a grave prognosis   to genetic differences among individuals.
               drugs with similar results.      (3 days).                         The remainder of the risk is environmental,
             ○   Other chemotherapy drugs that have   •  More than 1 of 7 BMDs and FCRs die from   although specific factors have not been
               been used include doxorubicin 30 mg/  HS.                          identified.
                2
                                        2
               m  IV (p. 609), epirubicin 30 mg/m  IV
               (p. 609), and dacarbazine 900-1000 mg/   PEARLS & CONSIDERATIONS   Technician Tips
                2
               m  (p. 609) diluted in saline over 5                               Some tumor-bearing dogs have coagulopathies.
               hours, although none provided durable     Comments                 Although peripheral veins may be preferred for
               responses.                      The following clinical and pathologic differences   routine blood draws, they may be needed to
             ○   Liposome-encapsulated clodronate, a novel   can help differentiate histiocytic syndromes:  deliver intravenous chemotherapy.
               formulation of a bisphosphonate capable   •  Clinical findings/behavior
               of  eliminating  macrophage/dendritic   ○   Disseminated HS is a multiorgan disease   Client Education
               cells, is not widely available but has been   typically arising from internal sites, includ-  •  Owners should contact the breeder or source
               reported and may be useful in treatment.  ing viscera, whereas SH involves skin and   of affected animals.
             ○   TALL104 (cytotoxic  T lymphocytes)   lymph nodes with occasional internal sites.  •  An overtly (phenotypically) normal breeding
               therapy has been described but is not   ○   CH is limited to the skin and does not   pair of BMDs with one affected offspring
               commercially available.            lead to HS.                       will produce on average one in seven puppies
           •  Focal malignant histiocytic diseases respond   ○   Hemophagocytic HS may be confused   that will ultimately succumb to histiocytosis.
             well to surgical resection (amputation if joint   with Evans syndrome (anemia and
             or bone affected or complete resection with   thrombocytopenia), but HS is Coombs   SUGGESTED READING
             limb salvage impossible) ± irradiation.  negative.                   Moore PF: A review of histiocytic diseases of dogs
                                               •  Histopathology and immunohistochemistry   and cats. Vet Pathol 51(1):167-184, 2014.
           Recommended Monitoring               for dogs and cats
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           •  The  involved  site(s)  should  be  monitored   ○   CD18  (leukocyte marker), CD3 , and   AUTHOR: Kim A. Selting, DVM, MS, DACVIM, DACVR
                                                                                  EDITOR: Kenneth M. Rassnick, DVM, DACVIM
                                                       −
             closely by regular observation and diagnostic   CD79a  are three stains and the immu-
             imaging as appropriate.              nohistochemical pattern most useful for






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