Regressing atypical histiocytosis: report of two cases with progression to high grade T-cell non-Hodgkin's lymphoma.
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.
Turner
M L
ML
Department of Haematology, Royal Infirmary of Edinburgh, U.K.
Gilmour
H M
HM
McLaren
K M
KM
Langlands
K
K
Craig
J I
JI
Parker
A C
AC
D000293:Adolescent; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D015620:Histiocytic Disorders, Malignant / Q000145:classification / Q000175:diagnosis / Q000473:pathology*; D006689:Hodgkin Disease / Q000175:diagnosis; D006801:Humans; D016130:Immunophenotyping; D008198:Lymph Nodes / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000473:pathology*; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology*; D013270:Stomach / Q000473:pathology