Autoimmune Hemolytic Anemia in Systemic Lupus Erythematosus: Clinical Presentation, Diagnostic Challenges, and Management Strategies
Ramesh T V1, Gopi Ayyaswamy2, Zioni Sangeetha3 and Vinod Kumar P 4*
Journal of Angiotherapy 6 (1) 1-5 https://doi.org/10.25163/angiotherapy.6162187290707122
Submitted: 29 November 2021 Revised: 14 December 2021 Published: 07 January 2022
Background: Cold agglutinin-induced hemolytic anemia is an extremely rare presentation in systemic lupus erythematosus (SLE). This case report discusses a 32-year-old male who presented with severe anemia and was later diagnosed with SLE. The patient exhibited symptoms of fatigue, weight loss, and shortness of breath, and laboratory findings revealed a hemoglobin level of 7 g/dL, positive cold agglutinins, and autoimmune markers consistent with SLE. Methods: A detailed clinical and laboratory investigation was performed, including blood tests, autoimmune profiles, bone marrow aspiration, and positron emission tomography-computed tomography (PET-CT) to assess the extent of systemic involvement. Nerve conduction studies were conducted to evaluate the patient's neurological symptoms. Results: The patient was diagnosed with cold agglutinin-induced hemolytic anemia secondary to SLE. Treatment included corticosteroids, hydroxychloroquine, and antibiotics for an accompanying infection. The patient responded well to treatment, with stabilization of hemoglobin levels and improvement in neurological symptoms. Conclusion: Cold agglutinin-induced hemolytic anemia is a rare initial manifestation of SLE. Early diagnosis and treatment are critical to preventing complications. This case underscores the importance of considering autoimmune hemolytic anemia in patients presenting with unexplained anemia, especially in the context of potential autoimmune diseases like SLE.
Keywords: Autoimmune hemolytic anemia, systemic lupus erythematosus, cold agglutinin disease, corticosteroids.
Autoimmune haemolytic anemia (AIHA) is a group of rare but severe blood disorders. They occur when the body destroys red blood cells. Autoimmune haemolytic anemia (AIHA) may be the first manifestation of systemic lupus erythematosus (SLE) and can appear several years before the diagnosis of SLE is made of cells more rapidly than it produces them (Bijaya Mohanty et al., 2019).
32-year-old gentleman with no comorbidities presented with complaints of low back pain radiating to bilateral lower limbs for the past 1 month, complaints of Di?culty in walking, and shortness of breath on exertion. History of weight loss (17 kg in one month), decreased appetite, and fatigue present. He has no history of diabetes, hypertension, asthma or heart disease. No surgical history and no relevant family history. On examination, Pallor is present with Bilateral Axillary lymph nodes. Vitals stable. Central nervous system examination reveals Bilateral lower limb power of 4/5. Other systems examination ?ndings are unremarkable. Investigation revealed Hemoglobin of 7gm/dl, Elevated ESR with A/G reversal. LFT, RFT were normal. Anemia workup revealed Normal B 12, folic acid levels, and normal transferrin saturation levels. DAT and cold agglutinins were positive.
Elevated LDH OF 1042, SERUM TSH was elevated (17). Anti-thyroglobulin was found to be positive. Autoimmune pro?le revealed ANA, dsDNA, Sm, Sm/RNP positive, and C3, C4levels were low. Bone marrow aspiration was nil signi?cant. PET CT showed metabolically active lymph nodes in bilateral cervical, axillary, mediastinal, retroperitoneal, and iliac region- suggestive of infective granulomatous ethology. Bone marrow biopsy showed mild Erythroid hyperplasia reduced myeloid precursors, and increased iron stores. Investigations were shown in table 1. During the hospital stay, he developed fever spikes with a further drop in haemoglobin. 2 units of PRBC were transfusions were done. Urine culture and sensitivity reveal growth of ESBL E. Coli . Right Axillary Lymph node biopsy revealed Non-speci?c reactive and angio follicular change with interfollicular vascular proliferation and plasmacytosis. Because of lower limb weakness, a Nerve conduction study was done, which reveals sensorimotor radiculoneuropathy of lower limbs and mildly of upper limbs, showing predominantly demyelinating and some axonal changes. The patient was started on thyroid supplements, Steroids, Antibiotics, Hydroxychloroquine and other supportive measures. Patient improved in clinical and laboratory parameters with above mentioned treatment and on follow up.
In Systemic lupus erythematosus patients, about 5 to 10 % present with Autoimmune haemolytic anaemia. AIHA is a rare disease comprising of warm, cold, and mixed type antibodies directed against an antigen on red blood cells (Michel, 2011; Velo-García et al., 2016; Gormezano et al., 2017). They are classified into warm and cold type which are differentiated by IgM autoantibodies called cold agglutinins. Treatment for AIHA includes corticosteroids, Rituximab, IVIg or splenectomy. Although cold agglutinin disease induced haemolytic anaemia is a rarer presentation, patients with anemia symptoms should raise suspicion for cold agglutinin disease and Direct coombs test should be managed early.
Ramesh T V, Gopi Ayyaswamy, Zioni Sangeetha, and Vinod Kumar P encouraged and supervised the findings of this work. All authors discussed the results and contributed to the final manuscript.
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