Vega F, Medeiros L J, Bueso-Ramos C, Jones D, Lai R, Luthra R, Abruzzo L V
Dept of Hematopathology, Box 72, University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA.
Am J Clin Pathol. 2001 Sep;116(3):410-9. doi: 10.1309/BM40-YM6J-9T3X-MH8H.
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.
我们报告了8例累及骨髓的肝脾T细胞淋巴瘤（HSTCL），并将组织学发现与疾病进展相关联。免疫表型分析显示为成熟的、异常的γ/δ T细胞免疫表型。4例患者检测到7号染色体长臂等臂染色体；1例显示t(7;14)(q34;q13)。7例检测病例中有7例存在单克隆TCRγ基因重排。最初诊断时的骨髓活检标本细胞增多，常见不明显的、主要为窦状隙浸润的非典型中小淋巴细胞。所有病例在诊断时及后续标本的涂片中有类似原始细胞的恶性细胞，部分细胞有细小的胞质颗粒。随着疾病进展，骨髓活检标本中HSTCL的模式逐渐变为间质型，肿瘤细胞变大。涂片检查中，原始细胞比例增加。7例患者死亡；1例失访。对1例患者进行的尸检显示，所有取样器官的血管腔内均有恶性细胞，肿瘤形成相对较少，这些部位类似血管内淋巴瘤。HSTCL在骨髓中常可通过其独特的组合表现被识别，即活检标本中的窦状隙模式和涂片检查中的原始细胞形态。
