Corresponding author:
Olayinka Okeleji MD
Department of Pediatrics
The University of Texas MD Anderson Cancer Center
1515 Holcombe Blvd., Unit 87. Houston, TX 77030
Email: ookeleji@mdanderson.org
Word count:
Main text: 842
Figures: 2
Running title: Castleman disease and anemia
Keywords: Castleman disease, lymphoproliferative disorder, microcytic anemia, pediatrics
Abbreviations: CD Castleman disease, HVV Hyaline-vascular variant, PET/CT Positron emission tomography/computed tomography, LDH lactate dehydrogenase, CBC Complete blood count, TIBC total iron binding capacity, MCV mean corpuscular volume, EBV Epstein-Barr virus, CMV cytomegalovirus, ANA antinuclear antibodies, RF rheumatoid factor, dsDNA double-stranded DNA, tTG-IgA Tissue Transglutaminase IgA, CRP C-reactive protein, ESR erythrocyte sedimentation rate, DAT direct antiglobulin test, MRI magnetic resonance imaging
BackgroundCastleman disease (CD) is an uncommon lymphoproliferative disorder characterized by hypervascular lymphoid hyperplasia. CD can be classified into two subgroups that differ in their pathogenesis, prognosis, and presentation: unicentric and multicentric.1 Several histological variants are seen, including the hyaline vascular variant (HVV), plasma cell variant, and mixed variant1. Unicentric HVV, the most common variant, typically presents as a single enlarged lymph node that is most often found incidentally on radiographic studies or during surgical procedures and can be cured by surgery.2,3Approximately 5000 cases of unicentric CD occur in the United States annually, with few cases documented in pediatric patients.3 In children, similar to adults, the most common subtype of CD is unicentric.4 Here we discuss a delayed diagnosis of a case of unicentric HVV CD with an atypical presentation involving anemia.5