Védrine L, Boucher E, Samson T, Souleau B, De Revel T, Nedellec G
Service d'hématologie clinique, Hôpital d'instruction des armées Percy, Clamart, France.
Presse Med. 2002 Nov 23;31(39 Pt 1):1846-8.
Lymphoproliferative syndromes are rarely complicated by medullar fibrosis simulating myeloid splenomegaly.
We report an unusual case of an IgD myeloma revealed in a context of myeloid splenomegaly in a 37 year-old man, initially admitted for severe anaemia associated with a voluminous splenomegaly.
The occurrence of myeloid splenomegaly during myeloma is extremely rare and only 14 cases have been reported. In this context, myelofibrosis is secondary to plasmocyte invasion of the bone marrow and regresses, or disappears, following specific treatment of the myeloma. The hypothesis evoked to explain the appearance of a secondary myelofibrosis is the "inappropriate" secretion, by the malignant plasmocyte clone, of functional analogs of pro-fibrosis cytokines, usually secreted by the mega-caryocyte precursors implied in myeloid splenomegaly.
淋巴增生性综合征很少并发类似髓样脾肿大的骨髓纤维化。
我们报告了一例不寻常的IgD骨髓瘤病例,该病例发生在一名37岁男性的髓样脾肿大背景下,最初因严重贫血伴巨大脾肿大入院。
骨髓瘤期间发生髓样脾肿大极为罕见,仅报告过14例。在此情况下,骨髓纤维化继发于浆细胞对骨髓的浸润,在骨髓瘤接受特异性治疗后会消退或消失。为解释继发性骨髓纤维化的出现而提出的假说是,恶性浆细胞克隆“不适当”地分泌促纤维化细胞因子的功能类似物,这些细胞因子通常由参与髓样脾肿大的巨核细胞前体分泌。