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表现为腭部肿物的骨外浆母细胞性骨髓瘤——一种罕见的实体

Extra-skeletal plasmablastic myeloma presenting as palatal growth - An unusual entity.

作者信息

Kumar Sanjay, Bhutani Namita, Bhargawa Shilpi, Kataria Sant Parkash, Sen Rajeev

机构信息

Deptt. of Pathology, PGIMS Rohtak, Haryana, India.

出版信息

Int J Surg Case Rep. 2017 Nov 20;41:423-426. doi: 10.1016/j.ijscr.2017.11.023. eCollection 2017.

Abstract

INTRODUCTION

Multiple myeloma (MM) is characterized by malignant proliferation of plasma cells, monoclonal bone marrow plasmacytosis, the presence of M-protein in serum and/or in urine and osteolytic bone lesions.

PRESENTATION OF CASE

We report a case of a 28-years old female, who was diagnosed to have relapsing extra-skeletal and extra-nodal plasmablastic myeloma, an atypical variant of MM with a poor prognosis. In addition to bone marrow plasmacytosis and the presence of M protein in the serum, the patient had an extramedullary lesion affecting the hard palate.

DISCUSSION

There is a strong correlation with age. The peak incidence is seen in 6th-7th decade. The clinical course in adolescents and young individuals is generally indolent and the survival is longer. Extramedullary myelomas are rare tumors accounting for 0.4% of all head and neck malignancies.

CONCLUSION

A case of extraskeletal plasmablastic myeloma presenting as a hard palate growth and that too in young female patient is an extremely rare and requires a multidisciplinary approach for management.

摘要

引言

多发性骨髓瘤(MM)的特征是浆细胞恶性增殖、单克隆骨髓浆细胞增多、血清和/或尿液中存在M蛋白以及溶骨性骨病变。

病例介绍

我们报告一例28岁女性病例,该患者被诊断为复发性骨外和结外浆母细胞性骨髓瘤,这是MM的一种非典型变体,预后较差。除了骨髓浆细胞增多和血清中存在M蛋白外,患者还有一个累及硬腭的髓外病变。

讨论

与年龄有很强的相关性。发病率高峰出现在60至70岁。青少年和年轻人的临床病程通常较为惰性,生存期较长。髓外骨髓瘤是罕见肿瘤,占所有头颈部恶性肿瘤的0.4%。

结论

一例表现为硬腭肿物的骨外浆母细胞性骨髓瘤病例,且发生在年轻女性患者中极为罕见,需要多学科方法进行管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8c11/5702861/52ebcc606302/gr1.jpg

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