Systemic Lupus Erythematosus, Evans Syndrome, and Neurofibromatosis: An Unusual Combination in Pediatric Patient

J Pediatr Hematol Oncol. 2020 May;42(4):e244-e247. doi: 10.1097/MPH.0000000000001490.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Anemia, Hemolytic, Autoimmune* / blood
  • Anemia, Hemolytic, Autoimmune* / complications
  • Anemia, Hemolytic, Autoimmune* / diagnosis
  • Female
  • Humans
  • Lupus Erythematosus, Systemic* / blood
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Neurofibromatosis 1* / blood
  • Neurofibromatosis 1* / complications
  • Neurofibromatosis 1* / diagnosis
  • Thrombocytopenia* / blood
  • Thrombocytopenia* / complications
  • Thrombocytopenia* / diagnosis

Supplementary concepts

  • Evans Syndrome