title: An unusual self-limited clonal Mott cell proliferation with lymphoplasmacytic lymphoma-like features in a child with the Wiskott-Aldrich syndrome and Von Recklinghausen's neurofibromatosis.
abstract: Patients with the Wiskott-Aldrich syndrome are at high risk for development of lymphomas, which are predominantly extranodal and of the immunoblastic type. We present a case of a self-limited lymphoproliferation with features of lymphoplasmacytic lymphoma arising in a patient with the Wiskott-Aldrich syndrome. The patient also had stigmata of von Recklinghausen's neurofibromatosis. The tumor was composed of CD138+, IgGkappa+, CD20-, PAX-5- Mott cells and CD5-, CD10-, CD19+, CD20+, CD43- small lymphoid B-cells that partially expressed CD23. The lymphadenopathy spontaneously resolved after a period of less than a year, and the patient had remained free of detectable lymphoproliferation for almost 4 years. He then developed Burkitt's lymphoma of the left parapharyngeal space. It is remarkable that both known lymphoproliferations with features of lymphoplasmatic lymphoma arising in patients with the Wiskott-Aldrich syndrome, this one and the previously described one, have spontaneously resolved. This observation is truly intriguing and requires further clinico-pathologic studies.
authors.affiliation: Department of Pathology, Scott and White Memorial Hospital and Clinic, 2401 South 31st Street, Temple, TX 76508, USA.
authors.forename: Debby
authors.initials: D
authors.lastname: Rampisela
authors.forename: Ludvik R
authors.initials: LR
authors.lastname: Donner
mesh_terms: D000293:Adolescent; D002051:Burkitt Lymphoma / Q000150:complications / Q000473:pathology; D049109:Cell Proliferation; D002469:Cell Separation; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D009365:Neoplasm Regression, Spontaneous; D009456:Neurofibromatosis 1 / Q000150:complications*; D014923:Wiskott-Aldrich Syndrome / Q000150:complications*