Valbuena Jose R, Herling Marco, Admirand Joan H, Padula Anthony, Jones Dan, Medeiros L Jeffrey
Department of Hematopathology, The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
Am J Clin Pathol. 2005 Mar;123(3):456-64. doi: 10.1309/93P4-2RNG-5XBG-3KBE.
T-cell prolymphocytic leukemia (T-PLL) can involve extramedullary sites, but the diagnosis is usually established by examination of blood and bone marrow. As a result, the histologic findings at extramedullary sites are poorly documented in the literature. We describe 19 extramedullary biopsy specimens from 14 patients with T-PLL. Skin (n = 10) was the most common site biopsied. T-PLL surrounded dermal blood vessels and appendages (n = 6), diffusely replaced dermis (n = 3), or formed a subcutaneous mass (n = 1). Other extramedullary sites included liver and lymph nodes (3 each) and spleen, lung, and cecum (1 each). In liver and lymph nodes, the neoplasm predominantly involved portal tracts and paracortex, respectively. Cytologically, the T-PLL cells were round (n = 16) or Sezary cell-like (n = 3). Nucleoli were observed in a subset of cells in 8 specimens and were prominent in 3 specimens. Immunostaining for T-cell leukemia-1 (TCL-1) was positive in specimens from 9 (64%) of 14 patients. We conclude that the prolymphocytoid features of T-PLL cells can be difficult to detect in routinely stained sections of extramedullary biopsy specimens. TCL-1 expression can aid in diagnosis at extramedullary sites.
T 细胞幼淋巴细胞白血病（T-PLL）可累及髓外部位，但通常通过血液和骨髓检查来确诊。因此，文献中关于髓外部位的组织学发现记录较少。我们描述了 14 例 T-PLL 患者的 19 份髓外活检标本。皮肤（n = 10）是最常进行活检的部位。T-PLL 围绕真皮血管和附属器（n = 6），弥漫性取代真皮（n = 3），或形成皮下肿块（n = 1）。其他髓外部位包括肝脏和淋巴结（各 3 例）以及脾脏、肺和盲肠（各 1 例）。在肝脏和淋巴结中，肿瘤分别主要累及门管区和副皮质区。细胞学上，T-PLL 细胞呈圆形（n = 16）或 Sezary 细胞样（n = 3）。在 8 份标本的部分细胞中观察到核仁，其中 3 份标本中的核仁明显。14 例患者中有 9 例（64%）的标本中 T 细胞白血病-1（TCL-1）免疫染色呈阳性。我们得出结论，在髓外活检标本的常规染色切片中，T-PLL 细胞的幼淋巴细胞样特征可能难以检测到。TCL-1 表达有助于髓外部位的诊断。
