Institute of Pathology, Hannover Medical School, Hannover, Germany.
Acta Haematol. 2013;129(3):187-96. doi: 10.1159/000342484. Epub 2012 Dec 21.
Concurrent manifestation of two chronic-stage myeloid and lymphoid/plasmacytoid neoplasms in one patient is rare and occurs in ≤1% of patients. There has been no systematic analysis of which combinations are frequent/infrequent and whether two concurrent diseases in one patient are clonally related or represent independent diseases. We therefore characterised a series of cases from our own archive (n = 65) and collected a large number of previously reported cases of patients in whom myeloid and lymphoid/plasmacytoid neoplasms co-occurred (n = 185). The most frequent combination was Philadelphia chromosome-negative myeloproliferative neoplasm with concurrent B cell chronic lymphocytic leukaemia, accounting for approximately 50% of double-disease patients. We compared the quantity of unsorted bone marrow cell-derived JAK2(V617F) and KIT(D816V) alleles with the quantity of the lymphoid/plasmacytoid compartment and analysed a subfraction of cases with fluorescence in situ hybridisation. Although a common aberrant progenitor has been reported in some cases in the literature, we found evidence of two independent chronic-stage myeloid and lymphoid/plasmacytoid neoplasms.
同一患者同时出现两种慢性期髓系和淋巴/浆细胞肿瘤的情况较为罕见，发生率≤1%。尚未对哪些组合更为常见/罕见以及同一患者的两种并存疾病是否具有克隆相关性或代表两种独立疾病进行系统分析。因此，我们对来自我们自己的档案中的一系列病例（n=65）进行了特征描述，并收集了大量先前报道的同时发生髓系和淋巴/浆细胞肿瘤的患者的病例（n=185）。最常见的组合是费城染色体阴性骨髓增殖性肿瘤伴发 B 细胞慢性淋巴细胞白血病，约占双疾病患者的 50%。我们比较了未分选骨髓细胞衍生的 JAK2(V617F)和 KIT(D816V)等位基因的数量与淋巴/浆细胞区室的数量，并对一些用荧光原位杂交进行分析的病例亚群进行了分析。尽管文献中报道了一些情况下存在共同的异常祖细胞，但我们发现了两种独立的慢性期髓系和淋巴/浆细胞肿瘤的证据。
