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