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Evans Syndrome: Breaking Down IMHA and ITP THE NEW ZEALAND VETERINARY NURSE SEPTEMBER 2015 Evans Syndrome: Breaking down IMHA and ITP Amy Breton CVT, VTS (ECC), BluePearl-Massachusetts, USA NZVNJ Issue 75 Volume 21 Pages 24-28 Introduction There is an increased rate of extra vascular platelet-associated antibody levels. In ITP Immune-mediated haemolytic anaemia haemolysis within the spleen and liver patients, platelet life span is reduced from (IMHA) is the most common cause of because of the removal of the antibody- the normal four to eight days to usually less haemolytic anaemia in dogs and immune- coated RBCs by the macrophages.3 While than one day.5 In patients with high antibody mediated thrombocytopenia (ITP) is the generally the antibodies attack the antigens levels platelets may survive less than one most common cause of severe thrombocy- of mature RBCs, in some patients, they may hour.5 Platelets that survive usually function topenia in dogs.1, 2 Both can occur in cats, attack the precursors of RBCs in bone mar- normally. 1 4 but are less common. IMHA and ITP may row. In most IMHA patients regeneration Immune-mediated haemolytic occur simultaneously or separate from each of RBCs is apparent, but if the antibodies anaemia other. When they occur together they are attack the bone marrow the patient may 4 IMHA can affect both dogs and cats, but is known as Evans syndrome, an autoimmune develop a non-regenerative IMHA. 2 typically seen in middle aged dogs. Dogs disease in which an individual’s antibod- ITP, also written with the acronym of IMT, that have secondary IMHA due to disease ies attack their own red blood cells and 3 1 occurs also because of primary (idiopathic) are commonly spayed females. There is platelets. or secondary (drug induced, neoplasia) some evidence of genetic predisposition in Pathophysiology reasons.1 Similarly to IMHA, ITP occurs Cocker Spaniels and Miniature Schnau- IMHA can be primary (idiopathic), or because antibodies are produced against zers, but it is also prevalent in Poodles, Irish secondary (viral, bacterial, parasitic, drug or platelet antigens which ultimately destroy Setters, Dobermans, Lhasa Apsos, English 2 vaccine induced). Approximately 60-75% platelets.3 The major organ of immune- Springer Spaniels and Old English Sheep- of IMHA in dogs is primary IMHA rather mediated platelet destruction is the spleen, dogs. 1, 2, 3 Roughly 60% of dogs with IMHA 2 than secondary. Vaccines and some medica- which is also a major source of anti-platelet will also experience concurrent ITP (Evans tions (cephalosporin’s, trimethoprim-sulfa) antibodies.5 Platelet life span is correlated to Syndrome).6 have been reported as a secondary causes 1 of IMHA. In both primary and second- B cell ary IMHA, the destruction of red blood cells (RBCs) occurs because antibodies are produced against the red blood cell secretion membrane antigens.2 An antibody is a spe- cialized immune protein which is produced IgG IgM in response to an antigen in the body and works to destroy the antigen. It is unknown Splenic macrophage what triggers this inappropriate antibody production in primary IMHA. Antibody Fcy receptor attachment to cell membranes triggers RBC destruction by a number of different C3b C3b mechanisms. With high levels of antibody C3b attachment the RBC membranes may C3b C3b become damaged so that water leaks into C3 receptor complement fixation agglutination to C3b the cytoplasm.4 This causes swelling and 4 phagocytosis of immunoglobin- rupture of the RBC thus causing hemolysis. and complement (C3b)-coated RBCs Antibodies may bind to two different RBCs, which, in turn, causes the RBCs to clump complement fixation 4 to membrane attack complex together, also known as agglutination. causing intravascular RBC lysis This clumping of RBC will slow down the Above: IMHA RBC destruction passage of other RBCs through the vessels. Image sourced from http://www.eclinpath.com/wp-content/uploads/IMHA-rbc-destruction.jpg 24 THE NEW ZEALAND VETERINARY NURSE SEPTEMBER 2015 Because the animal’s body is destroying its and anticoagulated blood, placing them on a positives can occur, but they are rare. False RBCs, it typically presents with signs of slide and rocking it back/forth for a minute.7 negatives can occur because of a low amount anaemia. Anaemic signs include weakness/ It is not possible to differentiate between of circulating antibodies.9 If the patient collapse, lethargy, dull/depressed mentation, rouleax and autoagglutination using this receives a blood transfusion or if there is pale/white mucous membranes, bounding method. 7 Lastly there are other references a large amount of agglutination a direct 1 1, 9 pulses, heart murmur and tachycardia. Some that recommend one drop of anticoagulated Coombs’ test will not be accurate. patients may present with a fever due to the blood should be mixed with “several” drops Flow cytometry is a newer test for evaluat- immune and/or inflammatory response 10 of 0.9% NaCl on a slide and rocked back/ ing antibodies in dogs. It allows for the while others may be hypothermic due to the 8 forth. It is recommended to look at the detection of red cell surface bound immu- anemia.1, 4 The spleen and, less commonly, slide using a microscope to identify rouleax noglobulin G (IgG) and immunoglobulin the liver may be enlarged since they are 10 versus agglutination. No matter the method M (IgM) antibodies. Anaemic dogs are the major organs where RBC destruction used, a positive agglutination test does not more likely to be positive for antibod- takes place. As large quantities of RBCs are confirm or rule out IMHA, but it does mean ies (IgG, IgM, or both) than nonanaemic broken down, bilirubin is released into the 4 11 the condition is acute and severe. dogs. However, dogs with IMHA have blood stream which may overwhelm the liver significantly higher percentages of the causing some patients to become icteric.5 A CBC should always be submitted to an outside laboratory to check for reticulocytes. antibodies than other dogs that are anaemic Diagnosis of IMHA 11 Reticulocytes are immature red blood cells. for non-IMHA causes. Flow cytometry The diagnosis of IMHA relies heavily on The presence of them indicates a functioning for the detection of IgG on RBCs is highly blood work. On a complete blood count bone marrow and often a better prognosis sensitive and, therefore, very specific for the 11 (CBC) 95% of dogs will have spherocytes for the pet. Reticulocytes will circulate diagnosis of IMHA. (small, spherical RBCs).9 Agglutination, for about a day in the blood stream before In cats it is important to test for feline leu- also known as clumping, of the RBCs usually 1 developing into mature red blood cells. kaemia and Mycoplasma haemofelis as they are occurs. There are several reported methods 3 Reticulocytes are found in approximately often the cause of IMHA. The organism on how to perform a slide agglutination test, 60-70% of IMHA patients and the number will fall off red blood cells in samples that but all involve anticoagulated blood and 0.9% of reticulocytes is relative to the degree of are greater than 24 hours old. Therefore it NaCl. One reference suggests taking one 2 anemia. is best to make a blood smear with a fresh drop of anticoagulated blood (from a purple sample. 12 In an acute infection the organism or capillary tube) and mixing with 10 drops The Coombs’ test, also known as a direct is only visible 50% of the time. 12 In cats the of 0.9% NaCl on a slide.1 Rouleax, which antiglobulin test (DAT), offers a more con- 9 anaemia is often regenerative and therefore causes the RBC’s to look like ‘stacked coins’ clusive diagnosis for IMHA. The Coombs’ offers a better prognosis.3 under the microscope, will disperse while test detects antibodies that are attached to agglutination will clump.1 Another reference the RBCs 9, and consists of running a series Complications of IMHA suggests taking equal parts of 0.9% NaCl of dilutions until agglutination occurs. False The complications of IMHA are vast. These include thrombocytopenia, disseminated intravascular coagulation (DIC), thrombo- embolism, gastrointestinal (GI) ulceration, renal failure and refractory anemia.2 Throm- bocytopenia can occur because of ITP (Evans Syndrome) or because of platelet consumption.2 The exact physiology of why ITP occurs concurrently with IMHA is unknown. 13 DIC can be triggered for a variety of reasons including thromboplastic substances released from the RBC membranes or tissue ischemia from hypoxia, 2 Ultimately coagulation times should be checked and patients should be monitored for signs of excessive bleeding, petechiae and ecchymoses. Thromboemobolism, particularly pulmonary thromboembolism, can occur in patients with IMHA, but it is unknown how fre- quently this occurs. The exact pathogenesis remains unknown, but the release of throm- Above: Note the stacking appearance of the RBC’s boplastic substances from RBCs, high Image sourced from http://www.myelomapennstate.net/images/DrBayerl/Rouleaux50x.jpg. volume of circulating bilirubin, venipuncture, 25 THE NEW ZEALAND VETERINARY NURSE SEPTEMBER 2015 catheters, and immunosuppressive agents immunoglobulin IgG.3,16 Steroids are not as Lastly both cyclosporine (Atopica™, may be contributing factors. 2,14 Pulmonary beneficial when dealing with intravascular Novartis) and danazol (Danocrine, Win- thromboembolism should be suspected in haemolysis that is mediated by IgM, so throp Pharmaceuticals) have both been used patients that suddenly develop respiratory in those cases other immunosuppressive to treat IMHA as secondary line medica- distress.4 agents (cyclosporine, cyclophosphamide) tions.17 Cyclosporine has been widely used can be used.3 Unfortunately, the studies in people for IMHA as well as for trans- Gastrointestinal ulceration and bleeding involving other immunosuppressive agents plantation surgery to help decrease factors can occur secondary to DIC, the use of are limited and have shown they are not any that stimulate an inappropriate autoimmune glucorticosterioids, ischemic injury to the 16 17 2 more effective then corticosteroids.
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