Case Report of International Journal of Case Reports
A Rare Reason of Ileus: Atypical Hemolytic Uremic Syndrome
Eray Eroglu1, Aysenur Cirak2, Ismail Kocyiğit1, Kemal Deniz3, Murat Hayri Sipahioglu1, Bulent Tokgoz1, Oktay Oymak1
1Erciyes University School of Medicine,Department of Nephrology, Kayseri,Turkey
2Erciyes University School of Medicine, Department of Internal Medicine, Kayseri,Turkey
3Erciyes University School of Medicine,Department of Pathology, Kayseri,Turkey
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy that is characterized by intravascular hemolytic anemia, thrombocytopenia and acute renal failure. Typical form occurred with the shiga-like toxin produced by Escherichia coli bacterium or Shigella dysenteriae bacterium while atypical HUS is sporadic or familial form of the disease which develops due to uncontrolled activation in alternative complement pathway. It may present with recurrent attacks and it has a potential of poor prognosis. In this case report we presented a 36-year-old woman in her 22nd week pregnancy without any history of chronic disease or medication whom admitted to hospital with abdominal pain, bloody diarrhea, oliguria and diagnosed with atypical HUS. She was diagnosed with ileus at the first month of Eculizumab treatment and a polyploid lesion obstructing the lumen of the colon was detected. Pathologic examination of the mass revealed a chronic active inflammatory lesion.
Keywords: Atypical HUS, Pregnancy, Eculizumab, Ileus
How to cite this article:
Eray Eroglu, Aysenur Cirak, Ismail Kocyiğit, Kemal Deniz, Murat Hayri Sipahioglu, Bulent Tokgoz, Oktay Oymak. A Rare Reason of Ileus: Atypical Hemolytic Uremic Syndrome. International Journal of Case Reports, 2018 3:47. DOI:10.28933/ijcr-2018-11-1501
1. Moake J. Thrombotic microangiopathies. N Engl J Med 2002;347:589-600
2. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med 2009;361: 1676-87.
3. Jokiranta TS. HUS and atypical HUS. Blood. 2017 Apr 17.
4. Ohanian M, Cable C, Halka K. Eculizumab safely reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome. Clin Pharmacol 2011;3:5-12.
5. Formeck C, Swiatecka-Urban A. Extra-renal manifestations of atypical hemolytic uremic syndrome. Pediatr Nephrol. 2018 Aug 14.
6. Moschcowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc N Y Pathol Soc 1924;24:21-4.
7. Warwicker P, Goodship TH, Donne RL, Pirson Y, Nicholls A, Ward RM, et al. Genetic studies into inherited and sporadic hemolytic uremic syndrome. Kidney Int 1998;53(4):836-44.
8. Dashe JS, Ramin SM, Cunningham FG. The long-term consequences of thrombotic microangiopathy (thrombotic thrombocytopenic purpura and hemolytic uremic syndrome) in pregnancy. Obstet Gynecol. 1998 May;91(5 Pt 1):662-8.
9. Tuncay M, Dervişoğlu E. Pregnancy and Thrombotic Microangiopathies (HELLP, TTP and AHUS Turkiye Klinikleri J Nephrol-Special Topics 2016;9(1):23-30
10. Fakhouri F, Roumenina L, Provot F, Sallée M, Caillard S, Couzi L, Essig M, Ribes D, Dragon-Durey MA, Bridoux F, Rondeau E, Frémeaux-Bacchi V. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010 May;21(5):859-67.
11. Besbas N, Gulhan B, Soylemezoglu O, Ozcakar ZB, Korkmaz E, Hayran M, Ozaltin F. Turkish pediatric atypical hemolytic uremic syndrome registry: initial analysis of 146 patients. BMC Nephrol. 2017 Jan 5;18(1):6.
12. Johnson S, Stojanovic J, Ariceta G, Bitzan M, Besbas N, Frieling M, Karpman D, Landau D, Langman C, Licht C, Pecoraro C, Riedl M, Siomou E, van de Kar N, Walle JV, Loirat C, Taylor CM. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol. 2014 Oct;29(10):1967-78.
13. De Yao J, Kaplan R, Magro C. An Atypical Case of Atypical Hemolytic Uremic Syndrome: Predominant Gastrointestinal Involvement, Intact Renal Function, and C5b-9 Deposition in colon and skin. J Hematol 2015, 4:193–195
14. Webb TN, Griffiths H, Miyashita Y, Bhatt R, Jaffe R, Moritz M, Hofer J, Swiatecka-Urban A. Atypical Hemolytic Uremic Syndrome and Chronic Ulcerative Colitis Treated with eculizumab. Int J Med Pharm Case Reports.2015, 4:105–112