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自发性软组织血肿——慢性粒细胞白血病(CML)的一种罕见表现

Spontaneous Soft Tissue Haematomas- A Rare Presentation of Chronic Myeloid Leukemic (CML).

作者信息

Lakhotia Manoj, Pahadiya Hans Raj, Prajapati Gopal Raj, Choudhary Akanksha, Gandhi Ronak

机构信息

Senior Professor, Department of Medicine, Dr. SNMC , Jodhpur, Rajasthan, India .

Senior Resident, Department of Medicine, Dr. SNMC , Jodhpur, Rajasthan, India .

出版信息

J Clin Diagn Res. 2015 Jul;9(7):OD03-5. doi: 10.7860/JCDR/2015/14318.6172. Epub 2015 Jul 1.

Abstract

Spontaneous soft tissue haematomas are rarely found in haematological malignancies. Chronic myeloid leukemia (CML) is a myeloproliferative disorder which rarely present with thrombo-haemorrhagic phenomenon. It is a malignant clonal disorder of pleuripotent stem cells that results in increase in myeloid, erythroid and platelets cells in peripheral blood and marked myeloid hyperplasia in bone marrow. It is characterized by the presence of Philadelphia chromosome (Ph) with BCR - ABL 1 fusion gene. This gene is responsible for the formation of 210 KDa chimeric proteins with enhanced tyrosine kinase activity which leads to the abnormal bone marrow cell proliferation and to the clinical and morphologic manifestations of leukemia. Cutaneous and mucous membrane bleeding is common in CML whereas bleeding in deep soft tissue is rarely found because of qualitative and quantitative platelet abnormalities. Here, we report a case of CML (BCR-ABL rearrangement positive) who presented with large haematoma in the anterior as well as posterior compartment of left thigh and treated successfully with hydroxyurea and imatinib.

摘要

自发性软组织血肿在血液系统恶性肿瘤中很少见。慢性粒细胞白血病(CML)是一种骨髓增殖性疾病,很少出现血栓出血现象。它是一种多能干细胞的恶性克隆性疾病,导致外周血中髓系、红系和血小板细胞增多,骨髓中明显的髓系增生。其特征是存在带有BCR - ABL 1融合基因的费城染色体(Ph)。该基因负责形成具有增强酪氨酸激酶活性的210 kDa嵌合蛋白,这导致骨髓细胞异常增殖以及白血病的临床和形态学表现。皮肤和黏膜出血在CML中很常见,而深部软组织出血由于血小板的质和量异常很少见。在此,我们报告一例CML(BCR - ABL重排阳性)患者,其左大腿前侧和后侧间隙出现巨大血肿,并成功接受羟基脲和伊马替尼治疗。

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