Nayak Hemanta Kumar, Kar Premashish, Bagchi Avishek, Kapoor Neha, Kapahtia Siddarth, Sonika Ujjwal, Kumar Naresh, Kumar Suresh
Department of Medicine, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India.
JOP. 2013 Jan 10;14(1):92-5. doi: 10.6092/1590-8577/1271.
Waldenstrom macroglobulinemia is a rare lymphoplasmacytic lymphoma characterized by a wide range of clinical presentations related to direct tumor infiltration and the production of IgM. Most commonly it presents with cytopenia, hepatosplenomegaly, lymphadenopathy, constitutional symptoms, and hyperviscosity syndrome.
We report a case of Waldenstrom macroglobulinemia in an 60-year-old female who initially presented with intermittent abdominal pain. The patient had no peripheral lymphadenopathy. On extensive investigation she was found to have pancreatic mass. The diagnosis of Waldenstrom macroglobulinemia was established after cytomorphology and immunohistochemical analysis of the patient's bone marrow revealed the presence of a lymphoid/lymphoplasmacytoid-like bone marrow infiltrate along with an elevated serum IgM level. The patient responded both clinically and serologically to chemotherapy. This case is unusual because the patient lacked all common clinical features of Waldenstrom macroglobulinemia with exception of anemia.
To our knowledge this is the first report of a patient with Waldenstrom macroglobulinemia presenting with a pancreatic mass adding to the spectrum of clinical presentations seen in this disease. This adds to the wide variety of gastrointestinal related clinical presentations of Waldenstrom macroglobulinemia and points to the need for considering Waldenstrom macroglobulinemia along with other lymphoid neoplasms in the differential diagnosis of pancreatic lesions.
华氏巨球蛋白血症是一种罕见的淋巴浆细胞性淋巴瘤,其特征是与肿瘤直接浸润及IgM产生相关的广泛临床表现。最常见的表现为血细胞减少、肝脾肿大、淋巴结病、全身症状和高黏滞综合征。
我们报告一例60岁女性华氏巨球蛋白血症病例,该患者最初表现为间歇性腹痛。患者无外周淋巴结病。经过广泛检查,发现她有胰腺肿块。对患者骨髓进行细胞形态学和免疫组化分析后,发现存在淋巴样/淋巴浆细胞样骨髓浸润,同时血清IgM水平升高,从而确诊为华氏巨球蛋白血症。该患者对化疗有临床和血清学反应。此病例不寻常,因为除贫血外,患者缺乏华氏巨球蛋白血症的所有常见临床特征。
据我们所知,这是首例以胰腺肿块为表现的华氏巨球蛋白血症患者报告,丰富了该疾病的临床表现谱。这增加了华氏巨球蛋白血症与胃肠道相关的多种临床表现,并指出在胰腺病变的鉴别诊断中,除其他淋巴肿瘤外,还需考虑华氏巨球蛋白血症。