Mixed Autoimmune Hemolytic Anemia as the Initial Manifestation of Systemic Lupus Erythematosus: A Case Report
- 1Acute Medicine, The Princess Alexandra Hospital NHS Trust, Harlow, GBR
Abstract
Mixed autoimmune hemolytic anemia (mAIHA) is a rare subtype of autoimmune hemolytic anemia (AIHA) characterized by the coexistence of warm (immunoglobulin G (IgG)) and cold (immunoglobulin M (IgM)) autoantibodies. It may occur as a primary disorder or secondary to underlying conditions, including systemic lupus erythematosus (SLE). We report the case of a 16-year-old female who presented with severe hemolytic anemia as the initial manifestation of SLE. On admission, her hemoglobin was 34 g/L with marked reticulocytosis and a positive direct antiglobulin test (DAT) demonstrating both IgG and IgM antibodies, confirming mAIHA. Immunological investigations revealed positive antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA) antibody, and anti-Smith antibody, supporting a diagnosis of SLE. At the time of the first presentation, she had no other clinical features suggestive of SLE. She was treated with blood transfusions and corticosteroids, resulting in significant improvement, and was discharged on a tapering prednisolone regimen. Shortly after, she was readmitted with a malar rash, arthralgia, joint swelling, nephrotic-range proteinuria, and worsening renal function. She was subsequently diagnosed with SLE complicated by lupus nephritis and recurrent AIHA. Following the rheumatology and nephrology review, she received intravenous methylprednisolone and was transferred to a tertiary care center for renal biopsy and further management. Renal biopsy demonstrated focal segmental glomerulosclerosis (FSGS). Further treatment with prednisolone, mycophenolate mofetil, intravenous belimumab, and hydroxychloroquine resulted in an excellent clinical response, with follow-up confirming remission. This case highlights mAIHA as a rare initial presentation of SLE and underscores the importance of early recognition, close monitoring, and multidisciplinary management, as patients presenting with AIHA may be at increased risk of developing lupus nephritis and other severe SLE-related complications.
Related
- When the Heart Reveals Lupus: Severe Dilated Cardiomyopathy as the Initial Manifestation of Systemic Lupus Erythematosus
- Bilateral Earlobe Purpura as an Unusual Initial Manifestation of Systemic Lupus Erythematosus: A Case Report.
- ACQUIRED HEMOPHILIA A AS THE INITIAL HEMATOLOGIC MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS IN AN ELDERLY PATIENT: A RARE CASE REPORT
- Recurrent Aphasic Seizures as the Initial Manifestation of LGI1 Antibody-Associated Autoimmune Limbic Encephalitis Mimicking a Transient Ischaemic Attack: A Case Report
- Breaking the Storm: Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome as the First Manifestation of Systemic Lupus Erythematosus/Mixed Connective Tissue Disease in an Adolescent Successfully Treated With Anakinra.
BibTeX
@article{iqbal2026mixed,
title = {{Mixed Autoimmune Hemolytic Anemia as the Initial Manifestation of Systemic Lupus Erythematosus: A Case Report}},
author = {Tariq Iqbal and Cleopatra Chivero and Sharmin Farhana},
journal = {Cureus},
year = {2026},
month = sep,
doi = {10.7759/cureus.116935},
url = {https://doi.org/10.7759/cureus.116935},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Mixed Autoimmune Hemolytic Anemia as the Initial Manifestation of Systemic Lupus Erythematosus: A Case Report
AU - Tariq Iqbal
AU - Cleopatra Chivero
AU - Sharmin Farhana
PY - 2026
DA - 2026/09/25
JO - Cureus
DO - 10.7759/cureus.116935
UR - https://doi.org/10.7759/cureus.116935
AB - Mixed autoimmune hemolytic anemia (mAIHA) is a rare subtype of autoimmune hemolytic anemia (AIHA) characterized by the coexistence of warm (immunoglobulin G (IgG)) and cold (immunoglobulin M (IgM)) autoantibodies. It may occur as a primary disorder or secondary to underlying conditions, including systemic lupus erythematosus (SLE). We report the case of a 16-year-old female who presented with severe hemolytic anemia as the initial manifestation of SLE. On admission, her hemoglobin was 34 g/L with marked reticulocytosis and a positive direct antiglobulin test (DAT) demonstrating both IgG and IgM antibodies, confirming mAIHA. Immunological investigations revealed positive antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA) antibody, and anti-Smith antibody, supporting a diagnosis of SLE. At the time of the first presentation, she had no other clinical features suggestive of SLE. She was treated with blood transfusions and corticosteroids, resulting in significant improvement, and was discharged on a tapering prednisolone regimen. Shortly after, she was readmitted with a malar rash, arthralgia, joint swelling, nephrotic-range proteinuria, and worsening renal function. She was subsequently diagnosed with SLE complicated by lupus nephritis and recurrent AIHA. Following the rheumatology and nephrology review, she received intravenous methylprednisolone and was transferred to a tertiary care center for renal biopsy and further management. Renal biopsy demonstrated focal segmental glomerulosclerosis (FSGS). Further treatment with prednisolone, mycophenolate mofetil, intravenous belimumab, and hydroxychloroquine resulted in an excellent clinical response, with follow-up confirming remission. This case highlights mAIHA as a rare initial presentation of SLE and underscores the importance of early recognition, close monitoring, and multidisciplinary management, as patients presenting with AIHA may be at increased risk of developing lupus nephritis and other severe SLE-related complications.
SN - 2168-8184
ER -