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Systemic Lupus Erythematosus, Evans Syndrome, and Neurofibromatosis

An Unusual Combination in Pediatric Patient

Moskop, Amy MD*; Dalrymple, Austin DO; Dolatshahi, Leili MD

Journal of Pediatric Hematology/Oncology: April 24, 2019 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001490
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Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organ systems. Patients can have hematologic manifestations, including Evans syndrome (ES), which is characterized by immune-mediated thrombocytopenia and anemia. The association of neurofibromatosis 1 (NF1) with autoimmune disorders is rarely reported. We will review the literature for this combination of disorders and describe a case of a 16-year-old girl who presents with immune-mediated cytopenias and is diagnosed with SLE, ES, and NF1. There are 7 reported cases of SLE and NF1 and only 2 are pediatric cases. There are no reports of the combination of SLE, ES, and NF1.

*Department of Pediatrics

Division of Adult & Pediatric Rheumatology

Division of Pediatric Hematology-Oncology, Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children’s Hospital, St Louis, MO

Present address: Amy Moskop, MD, Department of Pediatrics, Medical College of Wisconsin, Children’s Hospital of Wisconsin, 8915 W Connell Ct, Milwaukee, WI 53226.

The authors declare no conflict of interest.

Reprints: Amy Moskop, MD, Department of Pediatrics, Medical College of Wisconsin, Children’s Hospital of Wisconsin, 8915 W Connell Ct, Milwaukee, WI 53226 (e-mail: amoskop@mcw.edu).

Received October 16, 2018

Accepted March 21, 2019

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