Transfusion and Apheresis Science 51 (2014) 81–82
Contents lists available at ScienceDirect
Transfusion and Apheresis Science j o u r n a l h o m e p a g e : w w w. e l s e v i e r. c o m / l o c a t e / t r a n s c i
Letter to the Editor Treatment of steroid resistant ulcerative colitis with severe autoimmune haemolytic anaemia
Dear Editor, Autoimmune haemolytic anaemia (AIHA) accompanied by ulcerative colitis (UC) is a rare and highly severe complication in ulcerative colitis. The frequency is defined as rare due to representing 0.2–1.7% of all UC patients [1,2]. We report a case of steroid combined with cyclophosphamide resistant autoimmune haemolytic anemia accompanied with active ulcerative colitis. The patient, a 42 year old male, diagnosed with ulcerative colitis in February 2010, was admitted to our clinic with acute exacerbation of ulcerative colitis. Although meselazine and prednisolone had been prescribed, he had given up medications without a doctor’s recommendation 2 months before admission. He complained of severe bloody diarrhoea, weakness and fatigue. His hgb concentration was 4.1 gr/dl, RBC count: 0.99 × 10∧6/μl, MCV:148.5 fL, Hct: 12.9% and reticulocyte count 49.3% with a haptoglobulin level of 2.00 (30–200) mg/dl on admission. The peripheral smear revealed many spherocytes. Due to hypotension and tachycardia, an emergency blood transfusion was planned; however neither compatible blood nor the patient’s blood group could be determined. Direct and indirect Coombs tests were highly positive. The positive direct Coombs showed IgG specifity and the indirect Coombs titer was 1/128. Two units of uncrossmatched type B Rh-negative (Rh-) blood transfused based on the blood group given by the patient. High dose pulse steroids (1 g/day) were administered for 3 days. Two milligram per kilogram methylprednisolone was continued, five more units of uncrossmatched blood transfusion were required considering hypotension and the haemodynamics of the patient. Plasma exchange sessions with fresh frozen plasma and albumin combination were performed for two consecutive days. Meanwhile, a colonoscopy was performed and left type ulcerative colitis with
http://dx.doi.org/10.1016/j.transci.2014.07.002 1473-0502/© 2014 Elsevier Ltd. All rights reserved.
an activity index of 8 was reported. Due to inadequate response meselazine 3 g/day and cyclophosphamide 50 mg/ day were added to the therapy and continued for 7 months with decreasing doses of methylpredsiolone. Although the ulcerative colitis was in remission (colonospic activity index 4–6 nearly 1 year after the first admission), the haemolytic anaemia was unresponsive so cyclophosphamide was replaced with azathiopurine 50 mg twice daily. Both UC and AIHA benefited from treatment (Fig. 1). Since both conditions are autoimmune and have connections, we represent this case for management for ulcerative colitis and autoimmune haemolytic anaemia. Nowadays, a conservative approach is preferred by choosing pharmacologic management such as 5-aminosalicylates, corticosteroids, and immunosuppressants, including purine antimetabolites and cyclosporine. Moreover colectomy due to failure of medical treatment within the first 5 years after diagnosis varies from 9% to 35% in patients according to involvement of the colitis. Alternative therapies like plasma exchange may also provide benefits when complications are fatal and resistant. Surgical intervention such as splenectomy and colectomy is preserved for resistant cases and a conservative approach is preferred [1,3,4]. References [1] Oliff IA, Compton CC. Case 7 – 2000. N Engl J Med 2000;342:722–8. [2] Podolsky DK. Inflammatory bowel disease. N Engl J Med 2002;347:417–29. [3] Giannadaki E, Potamianos S, Roussomoustakaki M, Kyriakou D, Fragkiadakis N, Manousos ON. Autoimmune hemolytic anemia and positive Coombs test associated with ulcerative colitis. Am J Gastroenterol 1997;92(10):1872–4. [4] Rutgeerts P, Sandborn WJ, Feagan BG, et al. Infliximab for induction and maintenance therapy for ulcerative colitis. N Engl J Med 2005;353:2462–76.
Taktakoglu Onur, Sumbul Hilmi Erdem, Kocabas Firat, Guven Birol Cukurova University Faculty of Medicine, Adana 01330, Turkey E-mail address: [email protected]
82
Letter to the Editor/Transfusion and Apheresis Science 51 (2014) 81–82
60
3000
50
2500
40
2000
1500
30 Cyclophosphamide Admission
Azathiopurine Rbc Tx
Rbc Tx
20
1000
10
500
0 Rtc%
28.6.11 30.6.11 2.7.11 7.7.11 9.7.11 19.7.11 3.8.11 22.8.11 11.1.12 7.2.12 19.7.12 25.7.13 49.3
37.5
37.5
37.5
14.1
5.4
5.4
11.3
9.4
2.85
1.4
1.4
Hgb
4.1
6.9
5.8
4.8
7.8
8.1
8.4
9.2
7.1
13.9
14.8
13.9
LDH
1785
2411
1576
1716
1269
687
564
388
422
129
132
141
Fig. 1. Course of LDH, Hgb and Rtc during treatment.
0
Contents lists available at ScienceDirect
Transfusion and Apheresis Science j o u r n a l h o m e p a g e : w w w. e l s e v i e r. c o m / l o c a t e / t r a n s c i
Letter to the Editor Treatment of steroid resistant ulcerative colitis with severe autoimmune haemolytic anaemia
Dear Editor, Autoimmune haemolytic anaemia (AIHA) accompanied by ulcerative colitis (UC) is a rare and highly severe complication in ulcerative colitis. The frequency is defined as rare due to representing 0.2–1.7% of all UC patients [1,2]. We report a case of steroid combined with cyclophosphamide resistant autoimmune haemolytic anemia accompanied with active ulcerative colitis. The patient, a 42 year old male, diagnosed with ulcerative colitis in February 2010, was admitted to our clinic with acute exacerbation of ulcerative colitis. Although meselazine and prednisolone had been prescribed, he had given up medications without a doctor’s recommendation 2 months before admission. He complained of severe bloody diarrhoea, weakness and fatigue. His hgb concentration was 4.1 gr/dl, RBC count: 0.99 × 10∧6/μl, MCV:148.5 fL, Hct: 12.9% and reticulocyte count 49.3% with a haptoglobulin level of 2.00 (30–200) mg/dl on admission. The peripheral smear revealed many spherocytes. Due to hypotension and tachycardia, an emergency blood transfusion was planned; however neither compatible blood nor the patient’s blood group could be determined. Direct and indirect Coombs tests were highly positive. The positive direct Coombs showed IgG specifity and the indirect Coombs titer was 1/128. Two units of uncrossmatched type B Rh-negative (Rh-) blood transfused based on the blood group given by the patient. High dose pulse steroids (1 g/day) were administered for 3 days. Two milligram per kilogram methylprednisolone was continued, five more units of uncrossmatched blood transfusion were required considering hypotension and the haemodynamics of the patient. Plasma exchange sessions with fresh frozen plasma and albumin combination were performed for two consecutive days. Meanwhile, a colonoscopy was performed and left type ulcerative colitis with
http://dx.doi.org/10.1016/j.transci.2014.07.002 1473-0502/© 2014 Elsevier Ltd. All rights reserved.
an activity index of 8 was reported. Due to inadequate response meselazine 3 g/day and cyclophosphamide 50 mg/ day were added to the therapy and continued for 7 months with decreasing doses of methylpredsiolone. Although the ulcerative colitis was in remission (colonospic activity index 4–6 nearly 1 year after the first admission), the haemolytic anaemia was unresponsive so cyclophosphamide was replaced with azathiopurine 50 mg twice daily. Both UC and AIHA benefited from treatment (Fig. 1). Since both conditions are autoimmune and have connections, we represent this case for management for ulcerative colitis and autoimmune haemolytic anaemia. Nowadays, a conservative approach is preferred by choosing pharmacologic management such as 5-aminosalicylates, corticosteroids, and immunosuppressants, including purine antimetabolites and cyclosporine. Moreover colectomy due to failure of medical treatment within the first 5 years after diagnosis varies from 9% to 35% in patients according to involvement of the colitis. Alternative therapies like plasma exchange may also provide benefits when complications are fatal and resistant. Surgical intervention such as splenectomy and colectomy is preserved for resistant cases and a conservative approach is preferred [1,3,4]. References [1] Oliff IA, Compton CC. Case 7 – 2000. N Engl J Med 2000;342:722–8. [2] Podolsky DK. Inflammatory bowel disease. N Engl J Med 2002;347:417–29. [3] Giannadaki E, Potamianos S, Roussomoustakaki M, Kyriakou D, Fragkiadakis N, Manousos ON. Autoimmune hemolytic anemia and positive Coombs test associated with ulcerative colitis. Am J Gastroenterol 1997;92(10):1872–4. [4] Rutgeerts P, Sandborn WJ, Feagan BG, et al. Infliximab for induction and maintenance therapy for ulcerative colitis. N Engl J Med 2005;353:2462–76.
Taktakoglu Onur, Sumbul Hilmi Erdem, Kocabas Firat, Guven Birol Cukurova University Faculty of Medicine, Adana 01330, Turkey E-mail address: [email protected]
82
Letter to the Editor/Transfusion and Apheresis Science 51 (2014) 81–82
60
3000
50
2500
40
2000
1500
30 Cyclophosphamide Admission
Azathiopurine Rbc Tx
Rbc Tx
20
1000
10
500
0 Rtc%
28.6.11 30.6.11 2.7.11 7.7.11 9.7.11 19.7.11 3.8.11 22.8.11 11.1.12 7.2.12 19.7.12 25.7.13 49.3
37.5
37.5
37.5
14.1
5.4
5.4
11.3
9.4
2.85
1.4
1.4
Hgb
4.1
6.9
5.8
4.8
7.8
8.1
8.4
9.2
7.1
13.9
14.8
13.9
LDH
1785
2411
1576
1716
1269
687
564
388
422
129
132
141
Fig. 1. Course of LDH, Hgb and Rtc during treatment.
0
