Autoanti-C in a patient with primary sclerosing cholangitis and autoimmune hemolytic anemia: a rare presentation

Publications

Share / Export Citation / Email / Print / Text size:

Immunohematology

American National Red Cross

Subject: Medical Laboratory Technology

GET ALERTS

ISSN: 0894-203X
eISSN: 1930-3955

DESCRIPTION

9
Reader(s)
31
Visit(s)
0
Comment(s)
0
Share(s)

SEARCH WITHIN CONTENT

FIND ARTICLE

Volume / Issue / page

Related articles

VOLUME 32 , ISSUE 3 (September 2016) > List of articles

Autoanti-C in a patient with primary sclerosing cholangitis and autoimmune hemolytic anemia: a rare presentation

Meenu Bajpai * / Ashish Maheshwari / Shruti Gupta / Chhagan Bihari

Keywords : autoimmune hemolytic anemia, primary sclerosing cholangitis, autoantibody

Citation Information : Immunohematology. Volume 32, Issue 3, Pages 104-107, DOI: https://doi.org/10.21307/immunohematology-2019-054

License : (Transfer of Copyright)

Published Online: 09-October-2019

ARTICLE

ABSTRACT

Primary sclerosing cholangitis (PSC) is rarely associated with autoimmune hemolytic anemia (AIHA), and the presence of
 specific autoantibodies has not been reported previously. We present a unique case report of PSC associated with AIHA implicating autoanti-C. A 17-year-old girl was admitted to our hospital with PSC along with AIHA. Her blood sample demonstrated a positive direct antiglobulin test and a positive autocontrol in the antihuman globulin phase, confirming the patient had warm-reactive AIHA. Further testing showed the possibility of anti-C. The patient’s Rh phenotype was C+D+E–c– e+. Further testing with select cells, serial alloadsorption, and an elution confirmed anti-C specificity. The patient was transfused with two C–, crossmatch-compatible packed red blood cell units. The patient’s hemoglobin level and general condition showed improvement. This unique case report shows PSC associated with AIHA caused by autoanti-C. Usually, warm AIHA presents with a panreactive pattern, and it is difficult to find compatible blood. In this rare case, we could determine the specific antibody; efforts should always be made in cases of AIHA to identify the specificity of autoantibody.

Content not available PDF Share

FIGURES & TABLES

REFERENCES

1. Eaton WW, Rose NR, Kalaydjian A, Pedersen MG, Mortensen PB. Epidemiology of autoimmune diseases in Denmark. J Autoimmun 2007;29:1–9.

2. Leger RM. The positive direct antiglobulin test and immune mediated hemolysis. In: Fung MK, Grossman BJ, Hillyer CD, Westhoff CM, eds. AABB technical manual. 18th ed. Bethseda, MD: AABB, 2014;439.

3. Moeller DD. Sclerosing cholangitis associated with autoimmune hemolytic anemia and hyperthyroidism. Am J Gastroenterol 1985;80:122–5.

4. Baker SS, Compton CC. A 17-year-old boy with autoimmune hemolytic anemia and abnormal liver function. N Engl J Med 1991;324:180–8.

5. Wiesner RH, LaRusso NF. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology 1980;79:200–6.

6. Chapman RW, Arborgh BA, Rhodes JM, et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut 1980;21:870–7.

7. Hirschfield GM, Karlsen TH, Lindor KD, Adams DH. Primary sclerosing cholangitis. Lancet 2013;382:1587–99.

8. Mendes FD, Jorgensen R, Keach J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006;101:2070–5.

9. Moeller DD. Sclerosing cholangitis associated with autoimmune hemolytic anemia and hyperthyroidism. Am J Gastroenterol 1985;80:122–5.

10. Salagre KD, Sahay RN, Patil A, Joshi A, Shukla A. Leukemoid reaction, a rare manifestation of autoimmune hemolytic anemia in a case of small duct primary sclerosing cholangitis. J Assoc Physicians India 2013;61:494–5.

11. Vaglio S, Arista MC, Perrone MP, et al. Autoimmune hemolytic anemia in childhood: serologic features in 100 cases. Transfusion 2007;47:50–4.

12. Issitt PD, Pavone BG. Critical re-examination of the specificity of auto-anti-Rh antibodies in patients with a positive direct antiglobulin test. Br J Haematol 1978;38:63–74.

13. Weber J, Caceres VW, Pavone BG, Issitt PD. Allo-anti-C in a patient who had previously made an autoantibody mimicking anti-C. Transfusion 1979;19:216–8.

14. Petz LD. A physician’s guide to transfusion in autoimmune haemolytic anaemia. Br J Haematol 2004;124:712–6.

EXTRA FILES

COMMENTS