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伴有“热区”的再生障碍性贫血

Aplastic anaemia with 'hot pockets'.

作者信息

Kansu E, Erslev A J

出版信息

Scand J Haematol. 1976 Nov;17(5):326-34.

PMID:1034334
Abstract

4 patients with chronic severe aplastic anaemia and with persistent foci of intense haematopoietic activity, so-called 'Hot Pockets', in their bone marrow were studied. In all patients the remainder of the bone marrow was morphologically and erythrokinetically hypoplastic. The cellular morphology in these 'Hot Pockets' displayed megaloblastic features and definite maturation abnormalities in erythroblasts, but no consistent changes in the myeloid or megakaryocytic series. The presence of persistent 'Hot Pockets' presents a conceptual challenge since these pockets contain multipotential stem cells capable of differentiation and self-renewal, but obviously incapable of repopulation of the bone marrow. In view of additional evidence for dyserythropoiesis in aplastic anaemia such as changes in erythroblast morphology and the production of macrocytes, fetal haemoglobin and complement sensitive cells, it seems likely that the bone marrow of aplastic anaemia patients is totally dyserythropoietic rather than hypoactive and that bone marrow transplantation in many cases may be both justified and necessary.

摘要

对4例慢性重型再生障碍性贫血患者进行了研究,这些患者骨髓中存在所谓的“热点区”,即持续存在的强烈造血活动灶。所有患者骨髓的其余部分在形态学和红细胞动力学方面均发育不全。这些“热点区”的细胞形态表现为巨幼样特征,成红细胞存在明确的成熟异常,但髓系或巨核细胞系未见一致变化。持续存在的“热点区”的出现带来了一个概念上的挑战,因为这些区域含有能够分化和自我更新的多能干细胞,但显然无法重新填充骨髓。鉴于再生障碍性贫血中红细胞生成异常的其他证据,如成红细胞形态的改变以及大红细胞、胎儿血红蛋白和补体敏感细胞的产生,再生障碍性贫血患者的骨髓似乎完全存在红细胞生成异常而非活性低下,并且在许多情况下骨髓移植可能是合理且必要的。

相似文献

1
Aplastic anaemia with 'hot pockets'.伴有“热区”的再生障碍性贫血
Scand J Haematol. 1976 Nov;17(5):326-34.
2
Physician Education: Myelodysplastic Syndrome.医师教育:骨髓增生异常综合征
Oncologist. 1996;1(4):284-287.
3
Special Education: Aplastic Anemia.特殊教育:再生障碍性贫血。
Oncologist. 1996;1(3):187-189.
4
[Refractory anemia with hypercellular bone marrow and preleukemia (author's transl)].
Med Klin. 1976 Nov 26;71(48):2127-35.
5
Transplantation haemopoiesis. Morphological bone marrow studies after allogeneic marrow transplantation in man for severe aplastic anaemia and acute leukaemia.造血移植。人类严重再生障碍性贫血和急性白血病异基因骨髓移植后的形态学骨髓研究。
Nouv Rev Fr Hematol (1978). 1979;21(2):133-48.
6
[Histopathologic characteristics of bone marrow in patients with aplastic anemia].再生障碍性贫血患者骨髓的组织病理学特征
Srp Arh Celok Lek. 2000 May-Jun;128(5-6):200-4.
7
[Bone marrow biopsy investigation: an important prognostic factor in aplastic anaemia (author's transl)].骨髓活检研究:再生障碍性贫血的一个重要预后因素(作者译)
Med Klin. 1977 Jun 10;72(23):1038-42.
8
[Proliferative activity of differentiated bone marrow cells in children with aplastic anemia].再生障碍性贫血患儿分化骨髓细胞的增殖活性
Probl Gematol Pereliv Krovi. 1981 Sep;26(9):36-9.
9
Purification of aplastic anaemia (AA) marrow haemopoietic progenitors combined with long-term marrow cultures (LTMC) to assess haemopoiesis in AA.再生障碍性贫血(AA)骨髓造血祖细胞的纯化与长期骨髓培养(LTMC)相结合,以评估AA中的造血功能。
Bone Marrow Transplant. 1991;7 Suppl 2:98.
10
[Progress in biology and therapy of aplastic anemia and other conditions of bone marrow failure].再生障碍性贫血及其他骨髓衰竭病症的生物学与治疗进展
Postepy Hig Med Dosw. 1992;46(1):21-51.

引用本文的文献

1
Aplastic anaemia: an analysis of 174 patients.再生障碍性贫血:174例患者的分析
Postgrad Med J. 1980 May;56(655):322-9. doi: 10.1136/pgmj.56.655.322.
2
Marrow hypoplasia with splenomegaly in adolescence.青少年期骨髓发育不全伴脾肿大。
J Clin Pathol. 1980 Aug;33(8):730-4. doi: 10.1136/jcp.33.8.730.
3
Aplastic anaemia and the hypocellular myelodysplastic syndrome: histomorphological, diagnostic, and prognostic features.再生障碍性贫血和低细胞性骨髓增生异常综合征:组织形态学、诊断及预后特征
J Clin Pathol. 1985 Nov;38(11):1218-24. doi: 10.1136/jcp.38.11.1218.