Lethal midline granuloma (peripheral T‐cell lymphoma) after lymphomatoid papulosis

• Published in: Cancer Vol. 70; no. 4; pp. 835 - 839
• Main Authors: Harabuchi, Yasuaki, Kataura, Akikatsu, Kobayashi, Kazutoyo, Yamamoto, Tetsuo, Yamanaka, Noboru, Hirao, Motoyasu, Onodera, Kazufumi, Kon, Shinichiro
• Format: Journal Article
• Language: English
• Published: New York Wiley Subscription Services, Inc., A Wiley Company 15.08.1992; Wiley-Liss
• Subjects: Adult; Biological and medical sciences; Biopsy; DNA, Viral - analysis; Female; Gene Rearrangement, T-Lymphocyte - genetics; genotypic analysis; Granuloma, Lethal Midline - genetics; Granuloma, Lethal Midline - microbiology; Granuloma, Lethal Midline - pathology; Hematologic and hematopoietic diseases; Herpesvirus 4, Human - genetics; Humans; immunophenotypic analysis; Immunophenotyping; lethal midline granuloma; Lymphoma, T-Cell, Peripheral - genetics; Lymphoma, T-Cell, Peripheral - microbiology; Lymphoma, T-Cell, Peripheral - pathology; lymphomatoid papulosis; Lymphoproliferative Disorders - genetics; Lymphoproliferative Disorders - microbiology; Lymphoproliferative Disorders - pathology; Medical sciences; peripheral T‐cell lymphoma; Skin Diseases - genetics; Skin Diseases - microbiology; Skin Diseases - pathology; Human; Case study; Lymphomatoid papulosis; Midline granuloma; Skin disease; Cutaneous hematologic disease; Lymphoproliferative syndrome; Etiopathogenesis; Adult; Female; Malignant hemopathy; Peripheral T cell lymphoma
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/1097-0142(19920815)70:4<835::AID-CNCR2820700419>3.0.CO;2-F

A Japanese woman with an 8‐year history of lymphomatoid papulosis (LP) had lethal midline granuloma (LMG) develop at the age of 51 years. There were histologic similarities between LP and LMG seen in this patient. Surface phenotypic studies on nasal and cutaneous lesions demonstrated a population of T‐cells expressing CD2, CD4, CD25, CD30, and histocompatibility antigen‐DR (HLA‐DR). Genotypic analyses of nasal and skin biopsy specimens disclosed a clonal rearrangement of the beta T‐cell receptor gene with the same rearrangement pattern. These data indicate that this patient had LMG characterized by clonal peripheral T‐cell lymphoma, which probably resulted from progression of the LP. Cancer 1992; 70:835–839.