Wen Jin-Quan, Feng Hai-Lin, Wang Xu-Qing, Pang Ju-Ping
Author Affiliations: Department of Hematology, Xi'an Children's Hospital, Xi'an 710003, China.
World J Pediatr. 2008 Feb;4(1):36-40. doi: 10.1007/s12519-008-0007-9.
Aplastic anemia (AA) and myelodysplastic syndrome (MDS) are both acquired disorders in which bone marrow fails to produce or release sufficient blood cells. Anemia, infections and thrombocytopenia are common signs of such diseases. Clinically, it is difficult to distinguish chronic aplastic anemia (CAA) from MDS, especially from MDS without splenomegaly. As prognosis and treatment of AA and MDS are different, it is extremely important to make a differential diagnosis for the two diseases.
The medical records of 31 patients with CAA and 17 patients with MDS were retrospectively reviewed. Hemogram, bone marrow smear and biopsy for those patients were analyzed.
The mean counts of monocytes and platelets in the peripheral blood of the CAA patients were significantly lower than those of the MDS patients. Bone marrow smear showed a reduction of cellularity in CAA patients. The mean counts of myeloblasts+promyelocytes, myeloblasts+proerythroblasts, and megakaryocytes in the bone marrow of CAA patients were markedly lower than those in MDS patients. But the mean lymphocyte count was reversed. Bone marrow cells showed morphological abnormalities in MDS. Hematopoietic tissue in the bone marrow biopsy decreased obviously in more than 96% of the patients with CAA. Adipose tissue in the bone marrow of CAA patients increased obviously. A reduction or deficiency (<2 cell/piece) of megakaryocytes was noted in 28 patients with CAA. Fibrous tissue in the bone marrow was detected in 5 patients with CAA. Bone marrow biopsy results showed hypercellular changes in 12 MDS patients. Ten patients showed aggregated erythroblasts which were in the same stage of development, and 15 patients had abnormal localization of immature precursors (ALIP).
Blood cell counts can be decreased in addition to the reduction of cellularity in the bone marrow without dyshematopoiesis in CAA patients. Peripheral blood monocytes, fibrous tissue and cellularity in bone marrow are increased in MDS. Dyshematopoiesis and ALIP may appear characteristically in the children with MDS. Histology of bone marrow is important in the differential diagnosis of MDS and CAA.
再生障碍性贫血（AA）和骨髓增生异常综合征（MDS）均为获得性疾病，其骨髓无法产生或释放足够的血细胞。贫血、感染和血小板减少是此类疾病的常见症状。临床上，慢性再生障碍性贫血（CAA）与MDS难以区分，尤其是与无脾肿大的MDS。由于AA和MDS的预后及治疗不同，对这两种疾病进行鉴别诊断极为重要。
回顾性分析31例CAA患者和17例MDS患者的病历。对这些患者的血常规、骨髓涂片及活检进行分析。
CAA患者外周血单核细胞及血小板平均计数显著低于MDS患者。骨髓涂片显示CAA患者骨髓细胞减少。CAA患者骨髓中原始粒细胞+早幼粒细胞、原始粒细胞+早幼红细胞及巨核细胞的平均计数明显低于MDS患者。但淋巴细胞平均计数情况相反。MDS患者骨髓细胞显示形态异常。超过96%的CAA患者骨髓活检造血组织明显减少。CAA患者骨髓脂肪组织明显增加。28例CAA患者巨核细胞减少或缺乏（<2个/片）。5例CAA患者骨髓中检测到纤维组织。12例MDS患者骨髓活检结果显示细胞增多。10例患者出现处于同一发育阶段的聚集幼红细胞，15例患者有未成熟前体细胞异常定位（ALIP）。
CAA患者除骨髓细胞减少外血细胞计数也可降低，且无造血异常。MDS患者外周血单核细胞、纤维组织及骨髓细胞增多。造血异常和ALIP可能是MDS患儿的特征性表现。骨髓组织学检查对MDS和CAA的鉴别诊断很重要。
