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
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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.
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