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首页> 外文期刊>Hematology/Oncology and Stem Cell Therapy >The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis
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The effects of hematopoietic stem cell transplant on splenic extramedullary hematopoiesis in patients with myeloproliferative neoplasm-associated myelofibrosis

机译：造血干细胞移植对骨髓增生性肿瘤相关性骨髓纤维化患者脾髓外造血的影响

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Background/objective: Hematopoietic stem cell transplant (HSCT) is the only curative treatment for myeloproliferative neoplasm-associated myelofibrosis (MPN-MF). The main clinical manifestation of MPN-MF is splenomegaly secondary to extramedullary hematopoiesis (EMH). The effects of HSCT on splenic EMH and associated vascular and stromal changes are unknown. This study compares the findings seen in spleens following HSCT with those of nontransplanted patients, normal controls, and matched bone marrow (BM) samples. Methods: This study included three transplanted MPN-MF spleens, three nontransplanted MPN-MF spleens, and three normal controls. Spleens were assessed for: (a) presence/extent of EMH; (b) presence of Gamna-Gandy bodies; (c) splenic fibrosis; (d) CD34-positive microvessel density; (e) CD8-positive sinusoids; (f) frequency of smooth muscle actin-positive myoid cells; and (g) nerve growth factor receptor-positive adventitial reticulum cells. In two cases, matched BM samples were assessed for cellularity, presence of atypical megakaryocytes, and fibrosis. Results: Compared with normal controls, all MPN-MF spleens were larger in size, had EMH, red pulp fibrosis, higher CD34-positive microvessel density, and decreased CD8-positive sinusoids. Compared with nontransplanted cases, post-HSCT spleens showed disappearance or reduction of EMH. Gamna-Gandy bodies were increased; no differences in the remaining parameters were found. A reduction of splenic EMH was associated with normalization of BM cellularity and megakaryopoiesis. Conclusion: HSCT reduces/abrogates splenic EMH and is associated with an increased number of Gamna-Gandy bodies, which may suggest vascular damage. The lack of stromal changes in spleens removed shortly after transplant is in line with similar observations in the BM, where a longer interval is often necessary for resolution of fibrosis.
机译：背景/目的：造血干细胞移植（HSCT）是骨髓增生性肿瘤相关性骨髓纤维化（MPN-MF）的唯一治疗方法。 MPN-MF的主要临床表现是继发于髓外造血（EMH）的脾肿大。 HSCT对脾EMH以及相关血管和基质变化的影响尚不清楚。这项研究将HSCT后脾脏中的发现与未移植患者，正常对照和匹配的骨髓（BM）样本中的发现进行了比较。方法：本研究包括三个移植的MPN-MF脾脏，三个未移植的MPN-MF脾脏和三个正常对照。评估脾脏：（a）EMH的存在/程度; （b）甘纳纳·甘迪尸体的存在; （c）脾纤维化; （d）CD34阳性微血管密度; （e）CD8阳性正弦曲线; （f）平滑肌肌动蛋白阳性肌样细胞的频率; （g）神经生长因子受体阳性的外膜网状细胞。在两种情况下，评估相匹配的BM样品的细胞性，非典型巨核细胞的存在和纤维化。结果：与正常对照组相比，所有MPN-MF脾脏均较大，具有EMH，红髓纤维化，较高的CD34阳性微血管密度和减少的CD8阳性正弦曲线。与未移植的病例相比，HSCT后的脾脏显示EMH消失或减少。 Gamna-Gandy尸体增加;其余参数没有发现差异。脾EMH的减少与BM细胞的正常化和巨核细胞生成有关。结论：HSCT减少/消除了脾脏EMH，并且与Gamna-Gandy体数目增加有关，这可能表明血管受损。移植后不久切除的脾脏缺乏基质变化，这与BM中的类似观察结果相符，在BM中，通常需要更长的间隔才能解决纤维化。

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