Autoanti-C in a patient with primary sclerosing cholangitis and autoimmune hemolytic anemia: a rare presentation.
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.
Department of Transfusion Medicine, Institute of Liver and Biliary Sciences, Sector D-1, Vasant Kunj, New Delhi 110070, India.
Meenu
M
Bajpai
Senior Resident, Department of Transfusion Medicine.
Ashish
A
Maheshwari
Senior Resident, Department of Transfusion Medicine.
Shruti
S
Gupta
Associate Professor, Department of Pathology, Institute of Liver and Biliary Sciences, New Delhi, India.
Chhagan
C
Bihari
D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000276:immunology* / Q000628:therapy; D000918:Antibody Specificity; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D001788:Blood Grouping and Crossmatching; D027682:Cation Transport Proteins / Q000276:immunology*; D015209:Cholangitis, Sclerosing / Q000276:immunology*; D003298:Coombs Test; D017707:Erythrocyte Transfusion; D005260:Female; D006801:Humans; D008562:Membrane Glycoproteins / Q000276:immunology*
D001323:Autoantibodies; D001324:Autoantigens; D027682:Cation Transport Proteins; D008562:Membrane Glycoproteins; C413213:RHCG protein, human