系统性肥大细胞增多症患者中集落形成祖细胞的数量：潜在诊断意义及与骨髓增殖性疾病的比较

Numbers of colony-forming progenitors in patients with systemic mastocytosis: potential diagnostic implications and comparison with myeloproliferative disorders.

作者信息

Jordan J-H, Jäger E, Sperr W R, Schwarzinger I, Födinger M, Fritsche-Polanz R, Ohler L, Geissler K, Valent P

机构信息

Department of Internal Medicine I, Division of Hematology and Hemostaseology, University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria.

出版信息

Eur J Clin Invest. 2003 Jul;33(7):611-8. doi: 10.1046/j.1365-2362.2003.01172.x.

DOI:10.1046/j.1365-2362.2003.01172.x

PMID:12814399

Abstract

BACKGROUND

An increase in colony-forming progenitor cells (CFU) is typically seen in myeloproliferative disorders (MPD). Systemic mastocytosis (SM) is a haemopoietic neoplasm involving myeloid progenitors similar to MPD. In the present study, we measured the levels of peripheral blood (pb) and bone marrow (bm) CFU in patients with different categories of SM, and compared them with those obtained in MPD patients and healthy controls.

MATERIALS AND METHODS

Numbers of CFU (CFU-GM, BFU-E, CFU-GEMM) were measured in a colony assay in 25 patients with SM [indolent SM (ISM), n = 15; smouldering SM (SSM), n = 3; SM with an associated haematologic clonal non-mast cell lineage disease (SM-AHNMD), n = 5; aggressive SM (ASM), n = 1; mast cell leukaemia (MCL), n = 1] and 37 with MPD [chronic myeloid leukaemia (CML), n = 10; polycythemia vera (PV), n = 8; essential thrombocytosis (ET), n = 9; idiopathic myelofibrosis (IMF), n = 10].

RESULTS

In the patients with MPD, elevated numbers of pb CFU were detected in all groups when compared with healthy controls (P < 0.05). In most of the patients with ISM, circulating CFU levels (CFU-GM, BFU-E, and CFU-GEMM) were within the normal range. In SSM, pb CFU-GM levels were normal in two patients, and elevated in a third patient. In the "SM-AHNMD-group", CFU levels were found to reflect the nature of the AHNMD: in SM with concomitant acute myeloid leukaemia (SM-AML, n = 2), the levels of CFU were low or undetectable, whereas in SM with chronic myelomonocytic leukaemia (SM-CMML, n = 2), elevated numbers of pb CFU-GM were found.

CONCLUSION

The numbers of CFU are normal in patients with ISM, but elevated in some patients with SSM and SM-CMML. An elevated CFU level in SM should raise the suspicion of an associated MPD (CMML) or smouldering SM, a novel SM-subtype that shares several features with MPD and sometimes progresses to an overt SM-MPD.

摘要

背景

在骨髓增殖性疾病（MPD）中通常可见集落形成祖细胞（CFU）增加。系统性肥大细胞增多症（SM）是一种造血肿瘤，涉及与MPD相似的髓系祖细胞。在本研究中，我们测量了不同类型SM患者外周血（pb）和骨髓（bm）CFU的水平，并将其与MPD患者和健康对照者的水平进行比较。

材料与方法

通过集落测定法测量了25例SM患者[惰性SM（ISM），n = 15；冒烟性SM（SSM），n = 3；伴有血液学克隆性非肥大细胞系疾病的SM（SM-AHNMD），n = 5；侵袭性SM（ASM），n = 1；肥大细胞白血病（MCL），n = 1]和37例MPD患者[慢性髓性白血病（CML），n = 10；真性红细胞增多症（PV），n = 8；原发性血小板增多症（ET），n = 9；原发性骨髓纤维化（IMF），n = 10]的CFU（CFU-GM、BFU-E、CFU-GEMM）数量。

结果

与健康对照者相比，MPD患者所有组的外周血CFU数量均升高（P < 0.05）。大多数ISM患者的循环CFU水平（CFU-GM、BFU-E和CFU-GEMM）在正常范围内。在SSM中，两名患者的外周血CFU-GM水平正常，第三名患者升高。在“SM-AHNMD组”中，CFU水平反映了AHNMD的性质：在伴有急性髓性白血病的SM（SM-AML，n = 2）中，CFU水平较低或检测不到，而在伴有慢性粒单核细胞白血病的SM（SM-CMML，n = 2）中，外周血CFU-GM数量升高。