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伴有游离轻链过度排泄的IgD-λ多发性骨髓瘤:单克隆丙种球蛋白病诊断中的一个陷阱。

IgD-λ multiple myeloma with excessive excretion of free light chains: a diagnostic trap in the identification of monoclonal gammopathies.

作者信息

Zahir Hanane, Tali Abdelali, Rachidi Meryem, Mouhib Hanane, Daif Hayat, Ait Ouzdi Zohair, Haouach Khalil, Chabaa Laila

机构信息

Service de biochimie clinique, Hôpital Arrazi, Centre hospitalier universitaire Mohammed VI, Marrakech, Maroc.

Service d'hématologie, Hôpital Arrazi, Centre hospitalier universitaire Mohammed VI, Marrakech, Maroc.

出版信息

Ann Biol Clin (Paris). 2019 Feb 1;77(1):107-111. doi: 10.1684/abc.2018.1403.

DOI:10.1684/abc.2018.1403
PMID:30799291
Abstract

Immunoglobulin D multiple myeloma (IgD MM) is a rare entity of monoclonal gammopathies. We report the case of a IgD MM, associated with excessive excretion of lambda free light chains (FLL λ) diagnosed and managed at the University Hospital Mohammed VI of Marrakech among an adult hospitalized in the hematology department for bone pain and alteration of the general condition. Indeed, IgD MM is characterized by its clinical severity and poor prognosis. The discretion or absence of a monoclonal peak in the electrophoresis of serum proteins makes detection difficult. The present case demonstrates that IgD MM may be associated with excessive production of CLL and may therefore be erroneously diagnosed as CLL MM. Knowledge of this rare subtype of MM and its epidemiological, clinical and especially biological characteristics is crucial for establishing the correct diagnosis.

摘要

免疫球蛋白D型多发性骨髓瘤(IgD MM)是单克隆丙种球蛋白病中的一种罕见类型。我们报告了一例在马拉喀什穆罕默德六世大学医院诊断和治疗的IgD MM病例,该病例伴有λ游离轻链(FLL λ)过度排泄,患者为一名因骨痛和全身状况改变而入住血液科的成年患者。事实上,IgD MM的特点是临床症状严重且预后不良。血清蛋白电泳中出现单克隆峰的情况不明显或不存在,这使得检测变得困难。本病例表明,IgD MM可能与慢性淋巴细胞白血病(CLL)的过量产生有关,因此可能被误诊为CLL MM。了解这种罕见的MM亚型及其流行病学、临床尤其是生物学特征对于做出正确诊断至关重要。

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