A CASE OF SYSTEMIC LUPUS ERYTHEMATOSUS COMPLICATED BY LIBMAN-SACKS ENDOCARDITIS AND SECONDARY CHRONIC MYELOID LEUKEMIA

 

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A CASE OF SYSTEMIC LUPUS ERYTHEMATOSUS COMPLICATED BY LIBMAN-SACKS ENDOCARDITIS AND SECONDARY CHRONIC MYELOID LEUKEMIA

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Zhilian
Li
Fu Lei rayauden@139.com Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China -
Anning Xu 18815505392@163.com Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China -
Linlin Yuan linlinyuan000@163.com Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China -
Li Zhang zhanglichangde@163.com Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China -
LiXia Xu 15820264379@139.com Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China -
Zhiming Ye yezhiming@gdph.org.cn Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China -
Yiming Tao taoyiming@gdph.org.cn Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China -
Zhilian Li lizhilian@gdph.org.cn Guangdong Provincial People's Hospital Department of Nephrology Guangzhou China *
 
 
 
 
 
 
 

Systemic lupus erythematosus (SLE) rarely complicated by Libman-Sacks endocarditis (LSE), and even more exceptionally by therapy- or immune-inflammatory-driven chronic myeloid leukemia (CML).

We report a 19-year-old woman admitted with malar rash, intermittent fever, bilateral leg edema, alopecia and arthralgia. Laboratory tests showed nephrotic-range proteinuria, hypoalbuminaemia, autoimmune haemolytic anaemia, thrombocytopenia, high-titre ANA and anti-dsDNA, hypocomplementaemia, and positive lupus anticoagulant. Transthoracic and transoesophageal echocardiography revealed a 10.5 × 4.2 mm sterile vegetation on the left ventricular aspect of the aortic valve with mild regurgitation, consistent with LSE. Renal biopsy was withheld because of high bleeding risk. Intravenous methylprednisolone pulse (500 mg × 3 days) followed by oral prednisone 40 mg/day, combined with mycophenolate mofetil, hydroxychloroquine, IVIG and warfarin, was initiated (SLEDAI 20).

The valvular vegetation resolved completely within 20 weeks, and proteinuria entered complete remission by month 9. After 21 months of treatment the patient developed asymptomatic leukocytosis (peak 109 × 10⁹/L). Bone-marrow morphology and BCR::ABL1 RT-PCR confirmed chronic-phase CML (p210 transcript, low-risk Sokal/ELTS). Imatinib was started; a deep molecular response (BCR::ABL1 IS 0.003%) was achieved and sustained. The drug was discontinued after 32 months, and treatment-free remission persists to date. SLE has remained inactive on low-dose mycophenolate mofetil and hydroxychloroquine.

This case illustrates that aggressive immunosuppression can induce complete resolution of LSE and underscores the need for lifelong hematologic surveillance in SLE patients, even during remission, to detect rare secondary neoplasms such as CML.

Kewords