Affiliation:
1. Service “Jacques-Delarue” d'Anatomie et de Cytologie Pathologiques, Hôtel Dieu, Paris, France; Service “Jacques-Delarue” d'Anatomie et de Cytologie Pathologiques, Hôtel Dieu, 1 place du Parvis Notre Dame, 75181 Paris, Cedex 04, France
2. Service “Jacques-Delarue” d'Anatomie et de Cytologie Pathologiques, Hôtel Dieu, Paris, France
Abstract
Extramedullary accumulation of myeloblasts or immature myeloid cells form tumors called myeloid sarcoma in the WHO classification. Such tumors develop in lymphoid organs, bone (skull, orbit, etc.), skin, soft tissue, various mucosae and organs, and the CNS. They may precede or occur concurrently with acute myeloid leukemia, or reveal blastic transformation of chronic myeloproliferative disorders or myelodysplastic syndromes. They may also reveal relapses in treated patients. They are constituted by a diffuse infiltrate made up of medium-to-large cells. The cells are difficult to identify. Imprints are very useful. Immunohistochemistry can help diagnose and distinguish four variants: granulocytic myeloperoxidase (MPO+, CD 68+ [KP1, PGM1-] lysozyme+, CD 34), monoblastic (MPO-, CD 68+, [KPI+, PGMI+] lysozyme+, CD 34-), myelomonoblastic (MPO-, CD 68+, [KPI+, PGM1+] lysozyme+, CD 34-), or megakaryoblastic (positivity for factor VIII, CD 61, CD 31). Immunohistochemistry sometimes demonstrates expression of CD 43, CD 7, CD 79a, and CD 56 (particularly the monoblastic variant with t[8;2 1]). Recently the demonstration of CD 99 and CD 1 7, which can now be done on paraffin sections, may be useful to identify blasts of granulocytic origin. The diagnosis is missed in about 50% of cases when immunohistochemistry is not used. Patients with myeloid sarcomas should be treated in the same way as patients with acute myeloblastic leukemia. Disease progression and prognosis are similar for the two conditions.
Subject
Pathology and Forensic Medicine,Surgery,Anatomy
Cited by
106 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献