UNCOVERING SYSTEMIC LUPUS ERYTHEMATOSUS: A RARE PRESENTATION WITH SEVERE PANCYTOPENIA, IDIOPATHIC THROMBOCYTOPENIC PURPURA AND PERICARDIAL EFFUSION

Document Type

Conference Proceeding

Publication Date

7-17-2025

Publication Title

J Gen Intern Med

Keywords

antinuclear antibody, cardiolipin, cobalamin, cyanocobalamin, immunoglobulin G, rituximab, Sm antibody, steroid, troponin, adult, anemia, antibody titer, bleeding, blood clotting, blood transfusion, bone marrow biopsy, case report, clinical article, complication, computer assisted tomography, conference abstract, contusion, diagnosis, drug therapy, dyspnea, electrocardiogram, electrocardiography, Epstein Barr virus, female, first-line treatment, follow up, hematemesis, hematopoiesis, hemoglobin blood level, hepatitis B, human, hyperplasia, idiopathic thrombocytopenic purpura, immunosuppressive treatment, intravenous drug administration, laboratory test, megakaryocyte, melena, menorrhagia, oral drug administration, overall survival, pancytopenia, pericardial effusion, pericardiotomy, platelet count, Raynaud phenomenon, second-line treatment, sinus tachycardia, splenomegaly, systemic lupus erythematosus

Abstract

CASE: A 33-year-old female with Raynaud's phenomenon presented with four days of chest heaviness, dyspnea, coffee-ground emesis and melena, along with three weeks of heavy menstrual bleeding. Examination revealed subconjunctival hemorrhages, bruises and purpura along with sinus tachycardia on Electrocardiogram. Laboratory tests showed severe pancytopenia with a hemoglobin of 4.1 g/dL, white blood cells of 2.8 Thous/mcL and platelets of 2 Thous/mcL, and elevated troponins (0.43 and 0.48 ng/mL). Bedside ultrasound revealed pericardial effusion with right ventricular collapse during diastole as an early sign of tamponade. A pericardial window was promptly done, evacuating 500 mL of blood-tinged fluid, which was negative for malignant cells. Viral testing for Human immunodeficiency, hepatitis B and C and Epstein-Barr viruses was only positive hepatitis B surface and total core antibodies. Computed tomography revealed no splenomegaly. Bone marrow biopsy demonstrated hyperplastic marrow with trilineage hematopoiesis and increased megakaryocytes. Studies showed antinuclear antibodies titer of 1:1280, with elevated cardiolipin immunoglobulin G, histone, ribonucleoprotein and Smith antibodies. Other than low vitamin B12 (196 pg/mL), further anemia, coagulation and autoimmune work-up was unrevealing, warranting the diagnosis of Systemic Lupus Erythematosus (SLE) and associated Idiopathic thrombocytopenic purpura (ITP). Management included multiple blood transfusions, intravenous immunoglobulin (IVIG) and steroids. Platelet count remained critically low at 3 Thous/mcL despite three days of treatment prompting the initiation of Rituximab. With subsequent improvement of platelet count to 37 Thous/mcL and stabilization of her hemoglobin level, she was discharged on a steroid taper with hematology and rheumatology follow ups for immunosuppressive therapy and long-term SLE management. IMPACT/DISCUSSION: This case highlights a rare initial presentation of SLE manifesting as severe pancytopenia, ITP, profound bleeding and pericardial effusion with early signs of tamponade. SLE is a multisystem autoimmune disease with a wide range of manifestations. Life-threatening pericardial effusion is an unusual but reported presenting sign. Rapid recognition and treatment, including pericardial window placement, are essential to prevent fatal outcomes. Steroids and IVIG are first-line therapy for ITP. If these fail to achieve a safe and stable platelet count, Rituximab, other immunosuppressive agents and thrombopoietin receptor agonists are second-line treatments. Tailored therapy and a multidisciplinary approach are crucial for favorable outcomes and long-term management. CONCLUSION: This case underscores the importance of maintaining a high index of suspicion for autoimmune diseases as SLE in patients with multisystem involvement, including hematologic and cardiac complications. Early diagnosis and intervention in a multidisciplinary approach are vital for patient survival and optimizing long-term outcomes.

Volume

40

First Page

S362

Last Page

S363

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