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Ulcerative Colitis with Associated Autoimmune Hemolytic Anemia

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Dr. Deepak Govil    11 November 2017

Dr. Deepak Govil

Senior Consultant

Dept. of Gastrointestinal Surgery Indraprastha Apollo Hospital New Delhi

A 55 year old male presented with 4 5 loose stools day since 5 weeks with crampy abdominal pain. There was associated malaise anorexia and low grade fever. He also complained of yellowish discoloration of eyes since2 weeks. The patient was a nonsmoker and nonalcoholic. There was no history of any drug intake. The patient gave a history of recurrent diarrhea 2 years back. On examination blood pressure BP pulse and respiration were stable. General physical examination revealed pallor and jaundice. Hepatomegaly was present with a palpablespleen. There was no ascites lymphadenopathy or edema. Systemic examination did not reveal any abnormal findings. The lab results showed hemoglobin Hb 6.5 g dL total leukocyte count TLC 6 000 mm3 reticulocyte count 10 erythrocyte sedimentation rate ESR 20 mm hour C reactive protein CRP 10 mg L serum albumin 3 g dL serum sodium135 mEq L serum potassium 3.2 mEq L increased serum lactatedehydrogenase LDH and reduced serum haptoglobin. Liver function tests LFTs showed mildly elevated liver enzymes and unconjugated hyperbilirubinemia. Serological tests for hepatitis viruses were negative. The direct and indirect Coombs tests were positive. Routine stool examination was negative for ova and parasites. Three sequential stool cultures were negative for any infective pathology. A colonoscopy was done which showed continuous inflammation of the rectum with edematous mucosa and multiple superficialulcers. Biopsy confirmed nongranulomatous colitis suggestive of ulcerative colitis UC . The patient was hospitalized and put on oral sulfasalazine along with intravenous methylprednisolone for 3 days followed by oral prednisone. He was also transfused 2 units of fresh blood.The patient responded to this line of management and showed improvement in Hb and serum bilirubin levels. Diagnosis Ulcerative colitis with associated autoimmunehemolyticanemia. Discussion Autoimmune hemolyticanemia AIHA is characterized byan increased breakdown of red blood cells RBCs due to autoantibodies with or without complement activation. The diagnostic features of AIHA include the combination of clinical and laboratory signs of RBC hemolysis together with thedetection of autoantibodies and or complement deposition on RBC as mostly evidenced by a positive direct antiglobulin test DAT also known as direct Coomb s test.1 AIHA can range in severity from mildly symptomatic illness toa rapidly fatal syndrome. AIHA is mediated by antibodies andin the majority of cases immunoglobulin Ig G is the mediatingantibody. This type of AIHA is referred to as warm AIHA because IgG antibodies bind best at body temperature. Cold AIHA is mediated by IgM antibodies which bind maximally attemperatures below 37 C.2 In more than 50 of the patients the development of AIHA is associated with an underlying disease secondary AIHA but it can occur without any evidence of an under lying disorder idiopathic or primary AIHA .1 Primary or idiopathic AIHA is less frequent than secondary AIHA. Secondary cases are often challenging because not only AIHA but also the underlying disease s must be diagnosed and treated.3 The most common underlying diseases in which AIHA can occur are lymphoproliferative disorders and immune diseases. Theexclusion of a drug induced hemolyticanemia is particularly important because stopping the drug is the most effective therapeutic measure in this situation.3 Autoimmune hemolysis can antedate follow after a few months or occur simultaneously with the disease process.4 Patients with UC commonly present with a wide range ofsystemic and local problems that can add to the complexity of treatment. Anemia is a common hematologic complication in these patients. The anemia in patients with UC may be a result of acute or chronic gastrointestinal blood loss chronic disease folate deficiency from sulfasalazine therapy or autoimmune hemolysis.5 Furthermore the association of anemia withjaundice in UC may suggest AIHA which is uncommon but not an unknown disorder. 4In UC increased levels of factor V factor VIII and fibrinogen anddecreased levels of antithrombin III have been demonstrated. Coomb s positive hemolyticanemia has occasionally been reported in patients with UC.6The coexistence of UC with Coomb s positive hemolyticanemiashould be considered in every UC patient with persisting orsevere anemia. But other causes of hemolysis should be excludedsuch as hemolysis due to frequent transfusions hereditaryspherocytosis hereditary elliptocytosis glucose 6 phosphatedehydrogenase deficiency G6PD pyruvate 6 kinase deficiency infections microvasculitichemolytic anemia.6

References

  1. Zeerleder S. Autoimmune haemolytic anaemia a practical guide to copewith a diagnostic and therapeutic challenge. Neth J Med 2011 69 4 177 84.
  2. DeLoughery TG. Autoimmune hemolyticanemia. Hospital Physician2013 8 Pt 1 2 11.
  3. Lechner K Jager U. How I treat autoimmune hemolyticanemias in adults.Blood 2010 116 11 1831 8.
  4. Sharma SC Tonk RS Gadpayale AK et al. Autoimmune haemolytic anaemiaassociated with ulcerative colitis. JIACM 2002 3 2 198 201.
  5. Osterman ST Lichtenstein GR. Chapter 112. Ulcerative colitis. In Feldman Sleisenger and Fordtran s Gastrointestinal and Liver Disease. 9th edition Elsevier Saunders 2011 p.1975 2012.
  6. Katsanos KH Christodoulou DK Kitsanou M et al. Thrombophlebitismigrans including Mondors syndrome and autoimmune hemolytic anemia in ulcerative colitis case report and review of the literature. Ann Gastroenterol 2002 15 2 190 4.

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