单克隆大规模球蛋白Waldenstrom巨球蛋白血症

Anu Bajaj
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摘要

Wald Enstrom巨球蛋白血症是一种由瑞典医生Jan Gosta Waldenstrom(1906-1996)命名的疾病。这种特殊疾病最初是在1944年被记录下来的[1,2]。Waldenstrom巨球蛋白血症可以定义为除了局限于骨髓的恶性淋巴浆细胞浸润外,还出现血清副蛋白,如免疫球蛋白M (Ig M)。淋巴浆细胞性淋巴瘤(LPL)可以理解为一种由微型B淋巴细胞、浆细胞样淋巴细胞和成熟浆细胞组成的肿瘤。肿大通常累及骨髓,偶尔出现于淋巴结和脾脏。淋巴浆细胞性淋巴瘤在大多数(95%)病例中伴有华登斯特罗姆大球蛋白血症[1,2]。这两种情况可能以免疫球蛋白M (Ig M)单克隆伽玛病为特征,并伴有局限于骨髓的淋巴浆细胞样淋巴瘤的出现。淋巴浆细胞样淋巴瘤可并发丙型肝炎病毒(HCV)感染。可以描述家族性流行。估计有1.4%的微型B淋巴细胞肿瘤可能由淋巴浆细胞样淋巴瘤引起[1,2]。
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The Monoclonal, Massive Globulin- Waldenstrom Macroglobulinaemia
Wald Enstrom macrogobulinaemia is a disorder designated with a nomenclature of a Swedish physician Jan Gosta Waldenstrom (1906-1996). The exceptional disease was initially scripted in 1944 [1,2]. Waldenstrom macroglobulinaemia may be defined as the appearance of a serum para-protein such as immunoglobulin M (Ig M) in addition to a malignant lymphoplasmacytic infiltrate confined to the bone marrow. Lymphoplasmacytic Lymphoma (LPL) may be cogitated as a neoplasm comprising of miniature B lymphocytes, plasmacytoid lymphocytes and mature plasma cells. The tumefaction generally implicates the bone marrow with an occasional presence in the lymph node and spleen. Lymphoplasmacytic lymphoma is accompanied by Waldenstrom macroglobulinaemia in a majority (95%) of instances [1,2]. The dual conditions may be denominated by an immunoglobulin M (Ig M) monoclonal gammopathy accompanied by an emergence of a lymphoplasmacytoid lymphoma restricted to the bone marrow. Lymphoplasmacytoid lymphoma may concur with an infection of hepatitis C virus (HCV). A familial prevalence may be delineated. An estimated 1.4% of neoplasm of miniature B lymphocytes may be cogitated by lymphoplasmacytoid lymphoma [1,2].
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