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  • Title: Regressing atypical histiocytosis: report of two cases with progression to high grade T-cell non-Hodgkin's lymphoma.
    Author: Turner ML, Gilmour HM, McLaren KM, Langlands K, Craig JI, Parker AC.
    Journal: Hematol Pathol; 1993; 7(1):33-47. PubMed ID: 8385660.
    Abstract:
    Two cases of regressing atypical histiocytosis (RAH) are presented. Both patients followed a typical regressing/relapsing course for several years before progression to high-grade neoplasia. In both cases these high-grade tumors were diagnosed as T-cell non-Hodgkin's lymphoma on histopathologic and immunophenotypic grounds, and demonstrated T-cell receptor beta chain (TCR beta) gene rearrangement on Southern blotting. The original cases of RAH were considered to be indolent neoplasms of histiocytic lineage. A single case of a patient with RAH demonstrating TCR beta and gamma gene rearrangements has been described. Our cases lend further weight to the proposition that RAH is a neoplasm of T-cell lineage, and ultimately of aggressive potential. This description accords with current thinking that many of the conditions previously classified as malignant histiocytosis would be better classified as T-cell non-Hodgkin's lymphoma.
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