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A patient with WT syndrome and Castleman disease
Author(s) -
VERGIN CANAN,
ÇETINGÜL NAZAN,
KAVAKLI KAAN,
ÖZTOP SENAY,
ÖZKINAY FERDA,
ÇOKER MAHMUT,
NIŞLI GÜNGÖR,
SOYDAN SALIHA,
BALIK EROL,
AKAR ORHAN
Publication year - 1995
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1995.tb03700.x
Subject(s) - medicine , pancytopenia , pathology , cervical lymphadenopathy , hyaline , castleman disease , disease , bone marrow
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.