title: Hepatosplenic gamma/delta T-cell lymphoma in bone marrow. A sinusoidal neoplasm with blastic cytologic features.
abstract: We report 8 cases of hepatosplenic T-cell lymphoma (HSTCL) involving bone marrow and correlate histologic findings with disease progression. Immunophenotypic analysis demonstrated mature, aberrant gamma/delta T-cell immunophenotypes. Isochromosome 7q was identified in 4 cases; 1 case showed the t(7;14)(q34;q13). Seven of 7 cases tested had monoclonal TCR gamma gene rearrangements. The initial diagnostic bone marrow biopsy specimens were hypercellular with a frequently subtle, predominantly sinusoidal infiltrate of atypical small to medium-sized lymphoid cells. In all cases, aspirate smears at diagnosis and in subsequent specimens contained malignant cells that resembled blasts, some with fine cytoplasmic granules. With progression, the pattern of HSTCL in bone marrow biopsy specimens became increasingly interstitial, and the neoplastic cells became larger. In aspirate smears, the proportion of blasts increased. Seven patients died; 1 was lost to follow-up. Autopsy performed on 1 patient demonstrated malignant cells within vascular channels in all organs sampled, with relatively little tumor formation, resembling intravascular lymphoma at these sites. HSTCL often can be recognized in bone marrow by its unique combination of a sinusoidal pattern in core biopsy specimens and blastic cytology in aspirate smears.
authors.affiliation: Dept of Hematopathology, Box 72, University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA.
authors.forename: F
authors.initials: F
authors.lastname: Vega
authors.forename: L J
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: C
authors.initials: C
authors.lastname: Bueso-Ramos
authors.forename: D
authors.initials: D
authors.lastname: Jones
authors.forename: R
authors.initials: R
authors.lastname: Lai
authors.forename: R
authors.initials: R
authors.lastname: Luthra
authors.forename: L V
authors.initials: LV
authors.lastname: Abruzzo
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology*; D002648:Child; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D002897:Chromosomes, Human, Pair 7 / Q000235:genetics; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte / Q000235:genetics; D006529:Hepatomegaly / Q000209:etiology / Q000473:pathology; D006801:Humans; D007124:Immunoenzyme Techniques; D008113:Liver Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics / Q000276:immunology; D013160:Splenic Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D013163:Splenomegaly / Q000209:etiology / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D004273:DNA, Neoplasm; D016692:Receptors, Antigen, T-Cell, gamma-delta