Auto Immune Mixed Haemolytic anaemia: A rare presentation of SLE
DOI:
https://doi.org/10.18203/2349-3933.ijam20192269Keywords:
Autoimmune haemolytic anaemia, Direct anti-globulin test, Immunoglobulin (Ig), Intravenous immunoglobulin, Systemic lupus erythematosusAbstract
Auto Immune Mixed Haemolytic Anaemia (AIHA) is defined as presence of both warm and cold antibodies against patient’s own red blood cells which is diagnosed by monospecific Direct anti-globulin test. Hereby we report a middle-aged women old women who was a known case of hypothyroidism on regular medication, presented with history of fatigability, exercise intolerance and exertional breathlessness of 1 month duration. The patient was subjected for preliminary investigations, which revealed severe anaemia with hemoglobin of 3.6 g% and an increased reticulocyte count of 12% with normal total leukocyte and platelet counts. Peripheral smear revealed anisopoikilocytosis, nucleated RBCs and schistocytes. Biochemical tests for haemolysis was evaluated which showed and elevated LDH levels (780U/L), and a reduced serum haptoglobulin levels. Liver Function test revealed a total bilirubin of 6.8mg/dl with indirect bilirubin of 5.4 mg/dl with normal liver enzymes. Baseline evaluation of Auto immune haemolytic anaemia with coombs test turned out to be positive. Patient was subjected for Mono specific DAT, Indirect Anti-globulin test (IAT) and antibody screening. Mono specific DAT showed both Anti IgG and anti C3 antibodies. IAT test was positive at 4⁰C and negative at 22⁰C and 39⁰C which confirmed that the AIHA was of mixed warm and cold type. On evaluation for connective tissue diseases, patient serum was reactive for ANA and ds-DNA and found to have Systemic Lupus Erythematosus which is a rarity and was responded to corticosteroids.
References
Packman CH. The Clinical Pictures of Autoimmune Hemolytic Anemia. Transfus Med Hemotherapy. 2015;42(5):317-24.
Sudha Reddy VR, Samayam P, Ravichander B, Bai U. autoimmune hemolytic anemia: mixed type-a case report. Indian J Hematol Blood Transfus. 2011;27(2):107-10.
Nossent JC, Swaak AJ. Prevalence and significance of haematological abnormalities in patients with systemic lupus erythematosus. QJM: An Inter J Med. 1991 Jul 1;80(1):605-12.
Hirano Y, Itonaga T, Yasudo H, Isojima T, Miura K, Harita Y, et al. Systemic lupus erythematosus presenting with mixed-type fulminant autoimmune hemolytic anemia: SLE and mixed-type fulminant AIHA. Pediatr Int. 2016;58(6):527-30.
Kokori SI, Ioannidis JP, Voulgarelis M, Tzioufas AG, Moutsopoulos HM. Autoimmune hemolytic anemia in patients with systemic lupus erythematosus. Am J Med. 2000;108(3):198-204.
Hepburn AL, Narat S, Mason JC. The management of peripheral blood cytopenias in systemic lupus erythematosus. Rheumatol. 2010;49(12):2243-54.
Gomard-Mennesson E, Ruivard M, Koenig M, Woods A, Magy N, Ninet J, et al. Treatment of isolated severe immune hemolytic anaemia associated with systemic lupus erythematosus: 26 cases. Lupus. 2006;15(4):223-31.
Kaushansky K, Lichtman MA, Prchal JT, Levi MM, Burns LJ, Caligiuri M. Williams Hematology. 9th Ed. 2016:5094.
Fayyaz A, Igoe A, Kurien BT, Danda D, James JA, Stafford HA, et al. Haematological manifestations of lupus. Lupus Sci Med. 2015;2(1):e000078-e000078.
Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol. 2002;69(4):258-71.
Haller W, Hind J, Height S, Mitry R, Dhawan A. Successful treatment of mixed-type autoimmune hemolytic anemia with rituximab in a child following liver transplantation. Pediatr Transplant. 2010;14(3):E20-5.
Lilleyman J. De Gruchy’s Clinical Haematology in Medical Practice. J Clin Pathol. 1990;43(4):352.
Singh A, Mandal A, Patel A, Mishra S. Autoimmune Haemolytic Anaemia-A Spectrum of Presentation in Children. JCDR. 2017 Sep;11(9):SR01.
