Pangalis Gerassimos A, Kyrtsonis Marie-Christine, Kontopidou Flora N, Vassilakopoulos Theodoros P, Siakantaris Marina P, Dimopoulou Maria N, Kittas Christos, Angelopoulou Maria K
Haematology Section, First Department of Internal Medicine, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece.
Semin Oncol. 2003 Apr;30(2):201-5. doi: 10.1053/sonc.2003.50046.
Waldenstrom's macroglobulinemia (WM) is a rare lymphoproliferative disorder (LPD) characterized by lymphoplasmacytic infiltration of the bone marrow (BM) and/or occasionally other tissues and by the presence of a serum monoclonal IgM. The disease belongs to the lymphoplasmacytic lymphoma (LPL) subtype. Whether WM is indeed a separate entity or is merely an IgM-secreting subtype of low-grade B-cell lymphoma is still controversial. In our series of 67 patients, WM has a long median overall survival of 110 months, and the male/female ratio is 1.2/1. Clinical features include a wide spectrum of manifestations, many of which may be common to other LPDs. Differential diagnosis is based on: (1) clinical and laboratory features (anemia, organomegaly, lymphadenopathy, IgM paraproteinemia), (2) cell morphology (lymphocytes, lymphoplasmacytes, few plasma cells), (3) histopathology of bone marrow or lymph node, (4) immunophenotype (CD5 expression and the intensity of CD5, CD20, and CD79b antigens may help in discrimination from other LPDs and atypical CLL), and (5) characteristic genetic features (present in other LPDs). Based on the former, diagnosis is usually easy. It may be harder in LPL cases not secreting IgM. We consider that WM should be, for the time being, handled as a separate entity. Further studies are necessary.
华氏巨球蛋白血症（WM）是一种罕见的淋巴增殖性疾病（LPD），其特征为骨髓（BM）存在淋巴浆细胞浸润和/或偶尔累及其他组织，并伴有血清单克隆IgM。该疾病属于淋巴浆细胞淋巴瘤（LPL）亚型。WM究竟是一个独立的疾病实体，还是仅仅是低级别B细胞淋巴瘤的一种分泌IgM的亚型，目前仍存在争议。在我们的67例患者系列中，WM的中位总生存期较长，为110个月，男女比例为1.2/1。临床特征包括广泛的表现形式，其中许多可能与其他LPDs相同。鉴别诊断基于：（1）临床和实验室特征（贫血、器官肿大、淋巴结病、IgM副蛋白血症），（2）细胞形态（淋巴细胞、淋巴浆细胞、少量浆细胞），（3）骨髓或淋巴结的组织病理学，（4）免疫表型（CD5表达以及CD5、CD20和CD79b抗原的强度可能有助于与其他LPDs和非典型慢性淋巴细胞白血病鉴别），以及（5）特征性基因特征（存在于其他LPDs中）。基于前者，诊断通常容易。在不分泌IgM的LPL病例中可能较难。我们认为，目前应将WM作为一个独立的实体来处理。有必要进行进一步的研究。
