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骨髓增生异常综合征的临床、血液学及组织形态学特征

Clinical, hematological and histomorphological profile of myelodysplastic syndrome.

作者信息

Nigam S, Rani S, Singh T, Gupta S, Rakheja D, Gaiha M

机构信息

Department of Pathology, MAM College, New Delhi.

出版信息

J Assoc Physicians India. 2001 Apr;49:430-4.

Abstract

AIM

The present study was designed to analyse clinical, haematological and histomorphogical features in 37 cases of myelodysplasic syndrome (MDS).

MATERIAL AND METHODS

FAB criteria was used to diagnose MDS in 37 patients. Clinical details, peripheral blood findings, bone marrow aspirate and biopsy were reviewed.

RESULTS

Of 37 cases, there were 25 females and 12 males in age range of 2 years to 75 years. There were three children. There were nine cases of refractory anaemia (RA), 18 of refractory anaemia with excess blasts (RAEB), nine of refractory anaemia with excess blasts in transformation (RAEB-t) and one of chronic myelomonocytic leukemia (CMML). All were denovo MDS. All patients had pallor. Hepato and/or splenomegaly was seen in 15 cases. Anemia, leukopenia and thrombocytopenia was seen in 37, 6 and 23 cases respectively. Bone marrow aspiration (BMA) was hyper to normo cellular in 35 cases. Dyshemopoiesis was seen in one or more cell lines in all cases. Bone marrow biopsy (BMB) was available in 16 cases. Abnormal localisation of immature precursors (ALIP) was seen in 11/12 biopsies of RAEB and 3/4 of RAEB-t. BMB was helpful in diagnosis of 3 doubtful cases (one RAEB and two RAEB-t), and upgrading of 2 cases of RAEB to RAEB-t. On follow up, 1 case of RA, 3 of RAEB, 1 of RAEB-t developed AML. One case of RAEB developed ALL and 2 progressed to RAEB-t.

CONCLUSION

Primary MDS is seen in all age groups. Pallor is commonest presentation. RAEB was commonest subgroup seen. BMB is helpful in diagnosis of doubtful cases. ALIP is a reliable diagnostic feature of MDS. BMB is a diagnostic as well as prognostic tool in MDS cases which should be taken as a mandatory investigation.

摘要

目的

本研究旨在分析37例骨髓增生异常综合征(MDS)患者的临床、血液学和组织形态学特征。

材料与方法

采用FAB标准对37例患者进行MDS诊断。回顾临床细节、外周血检查结果、骨髓穿刺及活检情况。

结果

37例患者中,年龄在2岁至75岁之间,女性25例,男性12例。有3名儿童。难治性贫血(RA)9例,难治性贫血伴原始细胞增多(RAEB)18例,难治性贫血伴原始细胞增多转变型(RAEB-t)9例,慢性粒-单核细胞白血病(CMML)1例。均为原发性MDS。所有患者均有面色苍白。15例患者出现肝和/或脾肿大。贫血、白细胞减少和血小板减少分别见于37例、6例和23例。35例骨髓穿刺(BMA)显示细胞增生高于正常。所有病例均可见一个或多个细胞系的造血异常。16例患者进行了骨髓活检(BMB)。在11/12例RAEB和3/4例RAEB-t的活检中可见未成熟前体细胞异常定位(ALIP)。BMB有助于诊断3例疑难病例(1例RAEB和2例RAEB-t),并将2例RAEB升级为RAEB-t。随访中,1例RA、3例RAEB、1例RAEB-t发展为急性髓系白血病(AML)。1例RAEB发展为急性淋巴细胞白血病(ALL),2例进展为RAEB-t。

结论

原发性MDS可见于所有年龄组。面色苍白是最常见的表现。RAEB是最常见的亚组。BMB有助于诊断疑难病例。ALIP是MDS的可靠诊断特征。BMB是MDS病例的诊断及预后工具,应作为一项必需的检查。

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