A patient with WT syndrome and Castleman disease.
WT syndrome, an autosomal dominant condition, combines hematological abnormalities with mild lib defects. Anemia, pancytopenia, leukemia and lymphoma can occur at varying ages from childhood to middle age. Limb defects include ulnar and radial defects, bifid or hypoplastic thumbs and cutaneous syndactyly. Castleman disease is characterized by tumorous masses of lymphoid tissue showing plasma cell or hyaline vascular type changes in histological specimens. A 13 year old boy, diagnosed as WT syndrome with ulnar and radial deviation and 5th finger clinodactyly also had neutropenia, cervical and mediastinal lymphadenopathy. Histology of the cervical lymph node showed angiofollicular hyperplasia of the hyaline-vascular type (Castleman disease). This interesting patient is reported because Castleman disease, together with WT syndrome has not been previously described.
Vergin
C
C
Faculty of Medicine, Department of Pediatrics, Ege University, Izmir, Turkey.
Cetingül
N
N
Kavakli
K
K
Oztop
S
S
Ozkinay
F
F
Coker
M
M
Nişli
G
G
Soydan
S
S
Balik
E
E
Akar
O
O
D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications*; D005871:Castleman Disease / Q000150:complications* / Q000151:congenital; D006228:Hand Deformities, Congenital; D006801:Humans; D008297:Male; D011884:Radius / Q000002:abnormalities*; D013577:Syndrome; D014457:Ulna / Q000002:abnormalities*