Familial Waldenstrom macroglobulinemia: report of 6 cases and review of literature
10.3760/cma.j.cn115356-20201027-00258
- VernacularTitle:家族性华氏巨球蛋白血症6例并文献复习
- Author:
Guangyu QI
1
;
Meiling ZHOU
;
Weixin CAI
;
Xu LI
;
Tianrong CHEN
;
Yuqing MIAO
;
Hao XU
;
Yuexin CHENG
Author Information
1. 徐州医科大学附属盐城医院 盐城市第一人民医院 南通大学第四附属医院血液内科，盐城 224006
- Keywords:
Waldenstrom macroglobulinemia;
Monoclonal gammopathy of undetermined significance;
Genetic diseases
- From:
Journal of Leukemia & Lymphoma
2022;31(2):99-102
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the familial inheritances, clinical features, treatments and outcomes of familial Waldenstrom macroglobulinemia (WM) patients.Methods:The clinical manifestations, laboratory examinations, diagnosis and treatments, and follow-up data of 6 familial WM patients who were admitted to Yancheng No.1 People's Hospital from June 2002 to July 2019 were retrospectively analyzed, and the literature was reviewed.Results:Among 6 WM patients, 4 patients had dizziness and fatigue at the onset, 1 patient had recurrent low-grade fever and abnormal sweating as the first manifestations, 1 patient was hospitalized due to pulmonary infection, and WM was found later. Two brothers of the patients were diagnosed with WM, another 2 brothers of the patients had IgM-type monoclonal gammopathy of undetermined significance (MGUS) during the physical examination. All the 6 patients were middle-aged/elderly men, with a median age of 63 years old (51-70 years old). The median follow-up time were 71.5 months (4-217 months), and by the end of the follow-up (June 2020), 2 cases died of pulmonary infection, and 1 of them developed acute myeloid leukemia; the other 4 cases were in regular chemotherapy. Two IgM-MGUS patients were followed up without symptoms.Conclusions:WM patients have familial aggregation, and their clinical manifestations are highly heterogeneous. Patients with family history may have poor prognosis. It is necessary to strengthen the awareness of WM and family history screening.
