Case Description
A 12-year-old boy with recent poor weight gain and vague abdominal pain
was found to have microcytic anemia during the work-up of his short
stature. He denied fevers and night sweats. Blood work showed anemia
(6.3 gm/dL), low MCV (61 fL), leukopenia (2.5 ×
103/μL), neutropenia (0.5 × 103/μL),
thrombocytosis (401 × 103/μL), low serum iron (21
μg/dL), normal TIBC (298 μg/dL), normal ferritin (271 ng/mL), elevated
haptoglobin level (347 mg/dL) and a negative IgG DAT. Hemoglobin
electrophoresis was negative for hemoglobinopathy. Peripheral blood
smear revealed reactive lymphocytes. EBV IgG and IgM were positive.
Parvovirus, HIV, and CMV were negative. Chest x-ray and thyroid studies
were normal. Baseline autoimmune workup (RF, dsDNA, ANA as well as
tTG-IgA for celiac disease) was negative. Uric acid and LDH were normal.
CRP (118 mg/L) and ESR (143 mm/h) were elevated.
He was referred to oncology after an ultrasonography of the patient’s
abdomen demonstrated an ill-defined abdominal mass. A subsequent MRI of
the abdomen and pelvis revealed a solid, minimally enhancing 5 × 3.6 ×
4.3 cm retroperitoneal mass in the left para-aortic/mesenteric root
region, just below the inferior border of the pancreas (Figure
1) . Fine-needle aspiration of the mass showed reactive/benign lymphoid
proliferation with focal CD21-, CD35-, and D2-40+ dendritic meshwork, no
immunophenotypic support for B-cell or T-cell neoplasia and Castleman
disease could not be excluded. Positron emission tomography/computed
tomography (PET/CT) scan demonstrated a unifocal, 4.5-cm lesion with a
standardized uptake value of 8.5 (Figure 1 ). Given the concern
for Castleman disease, the patient underwent laparoscopic resection of
the mass. Pathology showed changes indicative of HVV CD (Figure
2) .
Within 2 months of the resection, the patient’s IL-6 level decreased
from 21 pg/mL to undetectable, hemoglobin level normalized, CRP level
declined from 118 mg/L to 0.6 mg/L, and sedimentation rate decreased
from 143 mm/h to 7 mm/h. PET/CT scan 1 month after excision showed no
recurrence or new lesions. The patient’s laboratory values remained
normal (HB 11.2g/dL, MCV 82fL) at last follow-up, 24 months after
resection.