A T-cell neoplasia showing clinicopathologic features of malignant histiocytosis with novel chromosomal abnormalities and N-ras mutation
✍ Scribed by Takahiro Itoyama; Naoki Sadamoriy; Ippei Sasagawa; Hideo Nakamura; Seiji Tokunaga; Yasuaki Yamada; Michito Ichimaru; Takahisa Yoshida; Masahiro Kikuchi; Fuminao Takeshima; Keisuke Iwasaki; Sadahiro Asai; Shunji Yamamori; Shoichi Shimizu
- Publisher
- John Wiley and Sons
- Year
- 1991
- Tongue
- English
- Weight
- 930 KB
- Volume
- 67
- Category
- Article
- ISSN
- 0008-543X
No coin nor oath required. For personal study only.
✦ Synopsis
Malignant histiocytosis (MH) is a distinct disease entity defined clinically and morphologically. However, the neoplastic origin of MH is not well established. The authors report a 26-year-old woman who showed the typical clinicopathologic features of so-called MH. Cytogenetic and molecular genetic examinations were performed in addition to the morphologic and immunologic approach. The expression of CD2 and T-cell receptor gene rearrangements indicated the T-cell origin of this case. CD30, which is positive for anaplastic large cell lymphoma (Ki-1 lymphoma), was not expressed. The cytogenetic study revealed a clonal chromosome abnormality involving 3q25,6p21,11p15, and llq21. An N-ras point mutation within codon 12 (GGT-GCT) was also detected. These findings indicate that MH defined clinically and morphologically is not a tumor of true histiocytic origin and that it should be reclassified on the basis of immunologic, cytogenetic, and molecular genetic data. Cancer 672103-2110,1991. INCE FOUR CASES of "histiocytic medullary reticu-S losis" (HMR) were reported by Scott and Robb-Smith,' the distinct clinical entity characterized by fever, pancytopenia, lymphadenopathy, and hepatosplenomegaly due to widespread tissue infiltration of cells similar to the histiocytes frequently accompanying erythrophagocytosis has been regarded as a histiocytic neoplasia. Later, Rappaport and B ~r n e ~. ~ introduced the term, "malignant histiocytosis" (MH), which includes HMR, and defined it as "a systemic, progressive, invasive infiltration of mor-