| 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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MedLine Citation:
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PMID: 19713050 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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Debby Rampisela; Ludvik R Donner |
Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2009-08-26 |
Journal Detail:
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Title: Pathology, research and practice Volume: 206 ISSN: 1618-0631 ISO Abbreviation: Pathol. Res. Pract. Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-07-15 Completed Date: 2010-11-10 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7806109 Medline TA: Pathol Res Pract Country: Germany |
Other Details:
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Languages: eng Pagination: 467-71 Citation Subset: IM |
Copyright Information:
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Copyright 2009 Elsevier GmbH. All rights reserved. |
Affiliation:
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Department of Pathology, Scott and White Memorial Hospital and Clinic, 2401 South 31st Street, Temple, TX 76508, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Burkitt Lymphoma / complications, pathology Cell Proliferation Cell Separation Flow Cytometry Humans Immunohistochemistry Lymphoproliferative Disorders / complications*, pathology*, physiopathology Male Neoplasm Regression, Spontaneous* Neurofibromatosis 1 / complications* Wiskott-Aldrich Syndrome / complications* |
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