Polypoid tongue lesions arising after
bone marrow transplantation have been described. Their etiopathogenesis has been unclear, as has their relationship to similar lesions arising in other settings of chronic immunodeficiency. We identified 12 polypoid lesions (from 8 immunosuppressed patients aged 6 months to 13 years) among all tongue lesions biopsied over the course of 13 years at our institution. Clinical history, histologic and ultrastructural features, special stains (Gram, Grocott
methenamine silver,
acid-fast bacilli, CD34, actin,
desmin, human herpesvirus-8), in situ hybridization for Epstein-Barr virus, and cytogenetic features were studied. Immunocompromise was from
bone marrow transplantation for
severe combined immunodeficiency (n = 1) and
acute lymphoblastic leukemia (n = 3),
hypogammaglobulinemia (n = 2),
22q11 deletion syndrome (n = 1), and
asthma therapy (n = 1). Histologic examination revealed fibrous stromal cores with squamous epithelial covering and various degrees of ulceration and accompanying
inflammation and granulation tissue. In 2 patients lesions were multiple in number. Fibroblasts were variably positive for smooth muscle actin and
desmin and negative for CD34. Special stains, immunohistochemistry, in situ hybridization, and ultrastructural examination identified no organisms except occasional surface bacteria. The tongue lesion from 1 patient with
Down's syndrome showed t(2;9)(p11;q34)+21 (translocation not seen in peripheral blood). Another patient had constitutional del 22q11. All transplant patients had
Philadelphia chromosome-positive
acute lymphoblastic leukemia (ALL) (translocations involving 9q34 and 22q11). Patients with congenital immunosuppression had
polyps arise at significantly younger ages than did patients with acquired immunosuppression. Immunosuppression-related lingual
polyps are a fibroproliferative process occurring in patients with
bone marrow transplantation and other immune-deficient conditions. Our findings indicate that these
polyps are driven by both immunosuppression and chromosomal rearrangement.