Cytotoxic T‐cell lymphoma presenting as secondary myelofibrosis with high levels of PDGF and TGF‐β
Cytotoxic T‐cell lymphoma presenting as secondary myelofibrosis with high levels of PDGF and TGF‐β
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细胞毒性 T 细胞淋巴瘤表现为继发性骨髓纤维化，PDGF 和 TGF-β 水平高
DOI：
10.1034/j.1600-0609.2001.00302.x
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发表时间：
2001
影响因子：
3.1
通讯作者：
K. Muta
中科院分区：
文献类型：
--
作者：
Y. Abe;K. Ohshima;M. Shiratsuchi;K. Honda;J. Nishimura;H. Nawata;K. Muta
致编者：髓系骨化常见于多种血液系统恶性肿瘤，包括慢性髓系白血病(1)、急性巨核细胞白血病(2)和毛细胞白血病(3)。然而，恶性淋巴瘤伴弥漫性骨髓破裂是罕见的。我们报告一例细胞毒性t细胞淋巴瘤，表现为骨髓弥漫性网状损伤引起的全血细胞减少症。此外，我们还讨论了®brosis的发病机制，特别是细胞因子在®brosis中的作用。一名19岁女性于1998年8月入院接受全血细胞减少症评估。外周血分析显示血红蛋白51 g/L，白细胞计数2.7r10/L，无异常细胞，血小板计数35r10/L。右侧颈部淋巴结肿大，肝脏和脾脏均可触及。全身计算机断层扫描（CT）显示双侧颈椎、锁骨上、主动脉旁和肠系膜淋巴结多发肿胀，并伴有明显的脾肿大（图1）。右侧颈部淋巴结活检显示小的非典型淋巴细胞弥漫性增生，伴多核巨细胞。免疫表型分析显示，两种细胞CD3、CD8、TCRbF1和TIA-1均阳性，CD4、CD20、CD30和CD56均阴性。骨髓穿刺导致干龙头，活检显示淋巴样细胞弥漫性网状纤维化（图2A）。正常的造血功能受到严重抑制。我们诊断为外周细胞毒性t细胞淋巴瘤伴髓细胞坏死，患者随后接受化疗（环磷酰胺、阿霉素、长春新碱和强的松龙）。我们进行了六个疗程的化疗。化疗三个疗程后，骨髓活检显示造血功能恢复，骨化消失（图2B）。然而，即使在所有6个疗程的化疗后，颈部和腹部仍有直径1±2cm的小淋巴结。该患者于1999年4月接受了同种异体外周血干细胞移植，到2000年8月还活着，疾病没有进展。我们在诊断时和三个疗程后测定了几种血清参数的水平（表1）。血清可溶性白介素-2受体和LDH浓度分别从14800降至502 U/mL和972降至313 U/ L。此外，两种®brosis血清标志物，包括前胶原末端肽III(4)，在type合成过程中释放
To the Editor: Myelo®brosis is often observed in a variety of hematological malignancies including chronic myelogenous leukemia (1), acute megakaryoblastic leukemia (2) and hairy cell leukemia (3). However, malignant lymphoma with diffuse myelo®brosis is rare. We present here a case of cytotoxic T-cell lymphoma presenting as pancytopenia due to diffuse reticulo®brosis of bone marrow. Furthermore, we also discuss the pathogenesis of the ®brosis, and in particular the role of cytokines relative to the ®brosis. A 19-yr-old female was admitted to our hospital to undergo evaluation of pancytopenia in August 1998. Peripheral blood analysis showed a hemoglobin level of 51 g/L, a white blood cell count of 2.7r10/L without abnormal cells, and a platelet count of 35r10/L. Her right cervical lymph nodes were enlarged, and both the liver and spleen were palpable. A systemic computed tomography (CT) scan demonstrated multiple swellings of the bilateral cervical, supraclavicular, para-aortic and mesenteric lymph nodes and marked splenomegaly (Fig. 1). Biopsy of the right cervical lymph node revealed diffuse proliferation of small atypical lymphocytes with some multi-nuclear giant cells. Immunophenotyping revealed that both cells were positive for CD3, CD8, TCRbF1 and TIA-1 and negative for CD4, CD20, CD30 and CD56. Bone marrow aspiration resulted in a dry tap, and its biopsy revealed diffuse reticulo®brosis with lymphoid cells (Fig. 2A). Normal hematopoiesis was severely suppressed. We diagnosed peripheral cytotoxic T-cell lymphoma with myelo®brosis, and the patient subsequently underwent chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisolone). We performed six courses of chemotherapy. After three courses of chemotherapy, a bone marrow biopsy demonstrated recovery of hematopoiesis and a disappearance of ®brosis (Fig. 2B). However, small lymph nodes 1±2 cm in diameter remained in the neck and abdomen even after all six courses of chemotherapy. The patient underwent alloperipheral blood stem cell transplantation in April 1999, and is alive as of August 2000 with no progression of the disease. We determined the levels of several serum parameters at diagnosis and after three courses of treatment (Table 1). The serum concentration of soluble interleukin-2 receptor and LDH decreased from 14,800 to 502 U/mL, and from 972 to 313 U/ L, respectively. In addition, two serum markers of ®brosis, including procollagen terminal peptide III (4), which was released during the synthesis of type