Development of Diffuse Large Cell Lymphoma from Follicular Lymphoma with Multiple Immunoglobulin Heavy Chain Gene Rearrangement Occurring in a Patient with Wiskott-Aldrich Syndrome

Patients with Wiskott-Aldrich syndrome (WAS), a congenital immunodeficiency, show a markedly increased risk of developing non-Hodgkin’s lymphoma compared with the general population [1]. Malignant lymphoma in WAS commonly presents as extranodal disease, and its histology almost always shows diffuse lymphoma, especially of large cell or immunoblastic cell type, as in other congenital or acquired immunodeficiencies [2]. We report a case of B-cell lymphoma occurring in a patient with WAS. At presentation the disease was follicular lymphoma with different histological, immunohistochemical, and molecular biological findings node by node, suggesting the presence of more than a single neoplastic clone. Eventually, the patient was killed by the systemic invasion of monoclonal diffuse large cell lymphoma, which appeared to have existed since the onset of the disease. A 21-year-old Japanese man was admitted because of general lymphadenopathy. Because the patient had been suffering since early infancy from recurrent respiratory infections, atopic eczema, and a tendency to bleed due to thrombocytopenia, his condition was diagnosed as WAS. Physical examination and computed tomographic scans revealed generalized lymphadenopathy. The patient showed a marked Development of Diffuse Large Cell Lymphoma from Follicular Lymphoma with Multiple Immunoglobulin Heavy Chain Gene Rearrangement Occurring in a Patient with Wiskott-Aldrich Syndrome Keiki Kawakami,a Motoko Yamaguchi,b Yasuyuki Watanabe,a Tetsuya Muratac