Talks
Presenting Author Academic/Professional Position
Internal Medicine Resident, PGY-2
Academic Level (Author 1)
Resident
Discipline/Specialty (Author 1)
Internal Medicine
Academic Level (Author 2)
Faculty
Discipline/Specialty (Author 2)
Internal Medicine
Discipline Track
Patient Care
Abstract Type
Case Report
Abstract
Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem involvement, including hematologic manifestations. Autoimmune Hemolytic Anemia (AIHA), though an established feature, is relatively uncommon and may occur early or in isolation. When AIHA presents without overt systemic symptoms, the underlying autoimmune etiology may be overlooked, resulting in delays in diagnosis and treatment. Prompt recognition is essential to prevent unnecessary interventions and prolonged morbidity.
Case Presentation: A 39-year-old woman residing in Texas presented to the Rheumatology office with persistent synovitis and severe transfusion-dependent anemia, requiring red blood cell transfusions every 48 hours for over three months. She had previously undergone an extensive invasive workup, including a bone marrow biopsy, which revealed normal cellularity and no evidence of malignancy. Upper endoscopy showed antral ectasia, and colonoscopy revealed a benign polyp, but no source of bleeding was identified.
Initial rheumatologic laboratory evaluation revealed hemoglobin 6.1 g/dL (normal: 12.0–16.0), hematocrit 18.3% (normal: 36–46), white blood cell count 4.8 x10³/µL (normal: 4.5–11.0), and platelets 182 x10³/µL (normal: 150–450). ANA titer was 1:1280 with a speckled pattern (positive if ≥1:80). Lactate dehydrogenase (LDH) was elevated at 243 IU/L (normal: 119–226), haptoglobin was undetectable at < 10 mg/dL (normal: 33–278), ESR was 36 mm/hr (normal: 0–30), and CRP was 5 mg/L (normal: 0–10). Complement C4 was low at 4.98 mg/dL (normal: 10–40), and anti-dsDNA was markedly elevated at 616 IU/mL (positive if ≥302). Autoantibodies including anti-SSA/Ro52 and Ro60 (>240 U/mL), anti-SSB/La (43 U/mL), and T-cell IgG autoantibodies were positive. The direct Coombs test was positive, and antiphospholipid markers showed weakly positive IgM anticardiolipin (11 MPL) and equivocal anti-β2 glycoprotein IgG (7 U/mL).
The patient met the SLICC criteria for SLE, with manifestations including synovitis, hemolytic anemia, leukopenia, thrombocytopenia, positive ANA, elevated anti-dsDNA, and low complement. Based on this, she was started on treatment with hydroxychloroquine, intramuscular dexamethasone, and oral prednisone 20 mg daily. Once AIHA was confirmed, pulse-dose IV methylprednisolone 250 mg daily for three days was administered, followed by high-dose oral prednisone at 1 mg/kg. She later received rituximab.
Her clinical and laboratory response was pronounced, with hemoglobin increasing to 13.8 g/dL, hematocrit to 44.9%, white blood cell count to 8.2 × 10³/µL, and platelets to 143 × 10³/µL. Complement levels also improved, with C4 rising to 12 mg/dL. At one-month follow-up, disease stability was successfully maintained, demonstrated by hemoglobin of 15.7 g/dL and platelet count of 219 × 10³/µL, without further need for transfusions or evidence of SLE flares.
Conclusion: This case illustrates the diagnostic challenges that arise when autoimmune cytopenias precede classic manifestations of SLE. Despite an extensive workup, a lack of consideration for autoimmune disease delayed effective treatment. The patient's rapid improvement following immunosuppressive therapy underscores the need for clinicians to recognize SLE as a potential cause of isolated hemolytic anemia and cytopenias. Raising awareness of such atypical presentations can lead to earlier diagnosis, timely specialist referral, and avoidance of unnecessary procedures and prolonged morbidity.
Presentation Type
Talk
Recommended Citation
Acosta Pozo, Diana and Álvarez Pérez, Luis, "Delayed Diagnosis of Systemic Lupus Erythematosus Presenting as Autoimmune Hemolytic Anemia: A Case for Heightened Clinical Suspicion" (2025). Research Colloquium. 2.
https://scholarworks.utrgv.edu/colloquium/2025/talks/2
Included in
Hemic and Lymphatic Diseases Commons, Immune System Diseases Commons, Internal Medicine Commons, Medical Immunology Commons, Medical Pathology Commons, Pathological Conditions, Signs and Symptoms Commons, Patient Safety Commons, Rheumatology Commons, Skin and Connective Tissue Diseases Commons
Delayed Diagnosis of Systemic Lupus Erythematosus Presenting as Autoimmune Hemolytic Anemia: A Case for Heightened Clinical Suspicion
Background: Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem involvement, including hematologic manifestations. Autoimmune Hemolytic Anemia (AIHA), though an established feature, is relatively uncommon and may occur early or in isolation. When AIHA presents without overt systemic symptoms, the underlying autoimmune etiology may be overlooked, resulting in delays in diagnosis and treatment. Prompt recognition is essential to prevent unnecessary interventions and prolonged morbidity.
Case Presentation: A 39-year-old woman residing in Texas presented to the Rheumatology office with persistent synovitis and severe transfusion-dependent anemia, requiring red blood cell transfusions every 48 hours for over three months. She had previously undergone an extensive invasive workup, including a bone marrow biopsy, which revealed normal cellularity and no evidence of malignancy. Upper endoscopy showed antral ectasia, and colonoscopy revealed a benign polyp, but no source of bleeding was identified.
Initial rheumatologic laboratory evaluation revealed hemoglobin 6.1 g/dL (normal: 12.0–16.0), hematocrit 18.3% (normal: 36–46), white blood cell count 4.8 x10³/µL (normal: 4.5–11.0), and platelets 182 x10³/µL (normal: 150–450). ANA titer was 1:1280 with a speckled pattern (positive if ≥1:80). Lactate dehydrogenase (LDH) was elevated at 243 IU/L (normal: 119–226), haptoglobin was undetectable at < 10 mg/dL (normal: 33–278), ESR was 36 mm/hr (normal: 0–30), and CRP was 5 mg/L (normal: 0–10). Complement C4 was low at 4.98 mg/dL (normal: 10–40), and anti-dsDNA was markedly elevated at 616 IU/mL (positive if ≥302). Autoantibodies including anti-SSA/Ro52 and Ro60 (>240 U/mL), anti-SSB/La (43 U/mL), and T-cell IgG autoantibodies were positive. The direct Coombs test was positive, and antiphospholipid markers showed weakly positive IgM anticardiolipin (11 MPL) and equivocal anti-β2 glycoprotein IgG (7 U/mL).
The patient met the SLICC criteria for SLE, with manifestations including synovitis, hemolytic anemia, leukopenia, thrombocytopenia, positive ANA, elevated anti-dsDNA, and low complement. Based on this, she was started on treatment with hydroxychloroquine, intramuscular dexamethasone, and oral prednisone 20 mg daily. Once AIHA was confirmed, pulse-dose IV methylprednisolone 250 mg daily for three days was administered, followed by high-dose oral prednisone at 1 mg/kg. She later received rituximab.
Her clinical and laboratory response was pronounced, with hemoglobin increasing to 13.8 g/dL, hematocrit to 44.9%, white blood cell count to 8.2 × 10³/µL, and platelets to 143 × 10³/µL. Complement levels also improved, with C4 rising to 12 mg/dL. At one-month follow-up, disease stability was successfully maintained, demonstrated by hemoglobin of 15.7 g/dL and platelet count of 219 × 10³/µL, without further need for transfusions or evidence of SLE flares.
Conclusion: This case illustrates the diagnostic challenges that arise when autoimmune cytopenias precede classic manifestations of SLE. Despite an extensive workup, a lack of consideration for autoimmune disease delayed effective treatment. The patient's rapid improvement following immunosuppressive therapy underscores the need for clinicians to recognize SLE as a potential cause of isolated hemolytic anemia and cytopenias. Raising awareness of such atypical presentations can lead to earlier diagnosis, timely specialist referral, and avoidance of unnecessary procedures and prolonged morbidity.
