AUTOIMMUNE MYELOFIBROSIS AS A RARE LUPUS COMPLICATION: A CASE REPORT

Document Type

Conference Proceeding

Publication Date

7-17-2025

Publication Title

J Gen Intern Med

Keywords

corticosteroid, hemoglobin, hydroxychloroquine, immunoglobulin G1, mycophenolic acid, prednisone, steroid, warfarin, acute leukemia, African American, anemia, anticoagulation, bleeding, blood smear, bone marrow biopsy, bone marrow depression, case report, clinical article, complication, conference abstract, Coombs test, cytopenia, diagnosis, drug dose reduction, drug megadose, drug therapy, erythrocyte, fatigue, female, first-line treatment, hemoglobin blood level, hemolysis, human, human tissue, immunosuppressive treatment, lupus erythematosus nephritis, muscle weakness, myelofibrosis, myeloid metaplasia, oral drug administration, platelet count, schistocyte, side effect, sigmoid sinus thrombosis, special situation for pharmacovigilance, splenomegaly, systemic lupus erythematosus, thrombocytopenia, vein thrombosis

Abstract

CASE: A 32-year-old African American female with SLE and lupus nephritis managed with mycophenolic acid and hydroxychloroquine presented with severe fatigue, generalized weakness, anemia, and thrombocytopenia. She was also taking warfarin due to her recent diagnosis of right sigmoid sinus thrombosis. Initial labs showed hemoglobin 7.5 g/dL and platelet count 104 x 109/ L, dropping to 67x 109/ L within 3 days. A bone marrow biopsy revealed autoimmune myelofibrosis, and her hemoglobin further dropped to 3.6 g/dL, prompting transfusion of five units of packed red blood cells. Peripheral blood smear showed very rare schistocytes. A Coombs test was positive 2+, anti-IgG 1+ and anti-C3d 1+, suggesting autoimmune hemolysis. High-dose prednisone (100 mg daily) was initiated, significantly improving hemoglobin levels from 3.6 g/dL to 8.2 g/dL. Warfarin was withheld in the setting of bleeding and a supratherapeutic INR of 5.5. Mycophenolic acid was increased to 720 mg twice daily and Hydroxychloroquine was maintained at 200 mg daily. Platelet recovery was less robust, improving modestly from 67 × 109/ L to 79 × 109/ L. The patient's condition stabilized, and she was discharged with plans for continued monitoring and management. IMPACT/DISCUSSION: AIMF is a rare but significant complication of SLE, involving immune-mediated bone marrow fibrosis and severe cytopenias. This case highlights acute anemia and thrombocytopenia in AIMF, particularly in the context of SLE. The dramatic hemoglobin drop post-biopsy, despite minimal bleeding, highlights the need to consider AIMF in SLE patients with unexplained cytopenias. It is also important to distinguish between primary myelofibrosis and AIMF. Autoimmune myelofibrosis is typically seen in younger individuals with a median age of 29 years whereas primary myelofibrosis patients have a median age of 66 years. Additionally, AIMF has no association with JAK2 mutations and splenomegaly is less common than primary myelofibrosis. This case is further distinguished by the patient's complex medication adjustments, which balanced management of SLE, lupus nephritis, and venous thrombosis alongside treatment of AIMF. The robust response of anemia to corticosteroids aligns with prior reports of AIMF being highly steroid-responsive. However, the modest platelet recovery underscores the variability in hematological responses and the potential for residual bone marrow dysfunction. Patients who do not receive an early diagnosis may experience a poor response to treatment. For patients who do not respond well to first-line treatment, transformation to acute leukemia may occur. CONCLUSION: Early diagnosis and treatment with corticosteroids are critical for reversing cytopenias and preventing further complications. The continuation of immunosuppressive therapy and anticoagulation alongside steroids reflects the importance of individualized treatment plans in AIMF cases with coexisting SLE complications.

Volume

40

First Page

S154

Find It @ Sladen

Share

COinS