A CHILLING COMPLICATION: COLD AGGLUTININ HEMOLYTIC ANEMIA IN ADULT-ONSET STILL'S DISEASE

Document Type

Conference Proceeding

Publication Date

7-17-2025

Publication Title

J Gen Intern Med

Keywords

colchicine, cold agglutinin, corticosteroid, ferritin, fibrinogen, glucocorticoid, haptoglobin, hemoglobin, interleukin 1, lactate dehydrogenase, methotrexate, prednisone, rituximab, steroid, triacylglycerol, adult, adult onset Still disease, anemia, antigen antibody reaction, arthralgia, autoimmune hemolytic anemia, blood smear, blood transfusion, case report, clinical article, clinical assessment, complication, conference abstract, Coombs test, drug therapy, erythema, erythrocyte transfusion, fever, follow up, hemolysis, hemolytic anemia, hemophagocytic syndrome, hospitalization, human, human cell, human tissue, hypertransaminasemia, hypotension, induced hypothermia, jaundice, leukocytosis, macrophage activation syndrome, male, oral drug administration, partial thromboplastin time, pericarditis, prothrombin time, reticulocyte, special situation for pharmacovigilance, therapy

Abstract

CASE: We present the case of a 39-year-old male with Adult-Onset Still's Disease (AOSD) who presented with recurrent hospitalizations for hemolysis and anemia. He was first diagnosed with AOSD based on migrating arthralgias, fevers, inflammatory knee effusions, erythematous rash, pericarditis, leukocytosis, transaminitis, and markedly elevated ferritin. He was treated with prednisone, methotrexate, and colchicine. After a period of medication nonadherence, he presented to the hospital with severe anemia, jaundice, and hypotension requiring vasopressor support. Laboratory evaluation showed hemoglobin 5.5 g/dL requiring multiple red blood cell transfusions, platelets 93 K/uL, LDH 3500 IU/L, haptoglobin < 30 mg/dL, reticulocyte 3.8%, fibrinogen 200 mg/ dL, PT 18 sec, PTT 40 sec, normal C3 and C4, triglycerides 293 mg/ dL, ferritin 973 ng/mL, positive Direct Antiglobulin Test (anti-complement positive, anti-IgG negative), peripheral blood smear with agglutination, suspicious for cold agglutinin hemolytic anemia (CAHA). The patient initially improved with pulse-dose steroids but later required Rituximab for worsening hemolysis. He was discharged on maintenance prednisone and followed outpatient with hematology and rheumatology with stable disease activity and hematologic parameters. Subsequent admissions demonstrated similar ongoing hemolytic episodes. Management included corticosteroids and supportive blood transfusions. Despite treatment, hemolysis persisted, prompting reinitiation of Rituximab. He was discharged with plans to treat underlying AOSD with an Interleukin-1 (IL-1) inhibitor, however, he was lost to follow-up. IMPACT/DISCUSSION: Common hematologic complications of AOSD include macrophage activation syndrome or hemophagocytic lymphohistiocytosis. This case highlights the rare and sparsely documented occurrence of CAHA as a manifestation of AOSD. Key teaching points include recognizing and managing atypical hematologic complications in AOSD. Traditional treatments involve systemic glucocorticoids, methotrexate, or biologics. However, limited data exists on the use of Rituximab in AOSD. This case demonstrates the successful application of Rituximab in treating AOSD complicated by recurrent CAHA. This case emphasizes the importance of a multidisciplinary approach involving hematology and rheumatology for addressing atypical complications. By documenting one of the few cases of recurrent CAHA in AOSD, this case provides a valuable reference for clinicians managing similar complex presentations. CONCLUSION: Clinical assessment of hemolysis in patients with autoimmune disorders should include a thorough evaluation for atypical secondary causes, such as cold agglutinin disease for optimal treatment. Multidisciplinary collaboration between rheumatology and hematology is essential to optimize outcomes in patients with complex autoimmune and hematologic conditions like CAHA and AOSD.

Volume

40

First Page

S103

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