The study, “Cold agglutinin-induced hemolytic anemia as the primary presentation in SLE — A case report,” was published in the Journal of Family Medicine and Primary Care.
In autoimmune hemolytic anemia (AIHA), the immune system recognizes red blood cells as foreign, and produces autoantibodies that target them for destruction. Cold agglutinin disease (CAD) is a form of AIHA.
Although AIHA has rarely been reported in people diagnosed with SLE, an autoimmune disease that affects multiple organs, researchers now report the case of a 17-year-old girl who showed symptoms of AIHA and was later diagnosed with SLE.
The girl was admitted to the hospital with a high, intermittent fever with chills for up to five days, accompanied by tachycardia (fast or irregular heart rate) but normal blood pressure. She also had joint pain.
Laboratory tests ruled out the potential for bacterial infection with mycoplasma or viral infections caused by dengue, hepatitis, chikungunya, HIV, cytomegalovirus, and Epstein-Barr virus. She was also negative for markers of rheumatoid arthritis, namely rheumatoid factor and anti-cyclic citrullinated peptide (CCP), but levels of the complement system — a set of more than 50 blood proteins that form part of the body’s immune defenses — were low.
Blood tests revealed that the girl had antinuclear antibodies, had elevated bilirubin (2.51 mg/dl) — generated during breakdown of RBCs — but her urine was free of red blood cells or hemoglobin (the protein found in red blood cells). Liver function, as measured by the levels of certain enzymes, was normal.
Further blood analysis revealed the presence of low hemoglobin and red blood cells (anemia), which despite normal aspect were clumping together. This, researchers said, was associated with CAD.
The patient received a matched blood transfusion and broad-spectrum antibiotic including methylprednisolone, a steroid, administered intravenously (into the blood). The patient recovered and has been doing well at regular follow-up.
Overall, while “CA-induced AIHA as the primary clinical presentation of SLE is extremely rare,” this case highlights the need for “increased awareness among primary physicians about SLE presenting as hemolytic anemia especially in young ladies … for the early diagnosis and appropriate management,” the study concluded.