Composite Tlymphoblastic leukemia/lymphoma and diffuse large B-cell lymphoma: case report.
Composite Tlymphoblastic leukemia/lymphoma and diffuse large B-cell lymphoma: case report.
复制标题
复合 T 淋巴细胞白血病/淋巴瘤和弥漫性大 B 细胞淋巴瘤：病例报告。
DOI：
10.1111/j.1440-1827.2011.02687.x
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发表时间：
2010
期刊：
影响因子：
--
通讯作者：
OhshimaK
中科院分区：
文献类型：
--
作者：
Niino D;Sugita Y;OhshimaK
真性红细胞增多症（PV）是一种造血干细胞克隆性骨髓增生性肿瘤（MPN）。尽管MPN患者的管理通常侧重于预防高凝状态引起的血栓栓塞事件，但血液恶性肿瘤患者经常患有非典型放射学模式的肺部疾病。我们在此报告一位56岁女性肺静脉携带JAK2V617F突变，胸部X线片显示弥漫性网状结节样改变，最初怀疑为粟粒性肺结核。视频辅助胸腔镜手术肺活检的病理学评估显示病变实际上是机化性肺炎（OP）。有趣的是，在肉芽组织周围也观察到肺髓外造血，肺泡毛细血管被许多非典型巨核细胞弥漫性堵塞。我们的不寻常的OP病例的组织学结果表明，局部活化的肿瘤性巨核细胞和血小板在纤维化病变的发展中发挥了重要作用。JAK2突变或MPN的白血病前期可加速巨核细胞的活化，导致纤维化的增殖过程。
Polycythemia vera (PV) is a clonal myeloproliferative neoplasm (MPN) of hematopoietic stem cells. Although the management of MPN patients generally focuses on the prevention of thromboembolic events caused by hypercoagulability, it is true that the patients with hematological malignancy often suffer from pulmonary diseases with atypical radiological patterns. We present here a 56‐year‐old woman with PV harboring a JAK2V617Fmutation that had a diffuse reticulonodular pattern on chest radiography and was initially suspected of having military tuberculosis. Pathological assessment of a video‐assisted thoracoscopic surgery lung biopsy revealed that the lesions were in fact organizing pneumonia (OP). Interestingly, pulmonary extramedullary hematopoiesis with a diffuse plugging of the alveolar blood capillaries by numerous atypical megakaryocytes was also observed around the granulation components. The histological findings of our case of unusual OP suggest that local activated neoplastic megakaryocytes and platelets played an important role in the development of spreading fibrotic lesions. JAK2 mutation or the preleukemic phase of MPN may accelerate the activation of megakaryocytes and result in the proliferative process of fibrosis.