| Min Fengling,Zhai Lijia,Zhou Wei,Gao Xiaohui,Zhang Lina.. Two cases of chronic myelomonocytic leukemia combined with monoclonal gammopathy of undetermined significance and a literature review. Oncol Transl Med, 2017, 3: 41-46. |
| 慢性粒单细胞白血病伴意义未明单克隆球蛋白病两例及文献复习 |
| Two cases of chronic myelomonocytic leukemia combined with monoclonal gammopathy of undetermined significance and a literature review |
| Received:October 07, 2016 Revised:February 23, 2017 |
| DOI：10.1007/s10330-016-0193-3 |
| 中文关键词: 慢性粒单细胞白血病;意义未明单克隆球蛋白病;骨髓增殖性肿瘤 |
| 英文关键词: myeloproliferative neoplasms (MPN); myelodysplastic syndrome (MDS); monoclonal gammopathy of undetermined significance (MGUS) |
| 基金项目: |
| Author Name | Affiliation | E-mail | | Min Fengling* | Department of Hematology, The Affiliated Hospital of Yangzhou University, The First People's Hospital, Yangzhou 225001, China | 1014121694@qq.com | | Zhai Lijia | Department of Hematology, The Affiliated Hospital of Yangzhou University, The First People's Hospital, Yangzhou 225001, China | zhailijia@sina.com | | Zhou Wei | Department of Hematology, The Affiliated Hospital of Yangzhou University, The First People's Hospital, Yangzhou 225001, China | zhangj@sina.com | | Gao Xiaohui | Department of Hematology, The Affiliated Hospital of Yangzhou University, The First People's Hospital, Yangzhou 225001, China | hmlygxh@163.com | | Zhang Lina. | Department of Hematology, The Affiliated Hospital of Yangzhou University, The First People's Hospital, Yangzhou 225001, China | zhanglina0512@163.com |
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| 中文摘要: |
| 目的 关注骨髓增生异常综合症（MDS）或骨髓增殖性肿瘤(MPN)合并意义未明单克隆球蛋白病(MGUS)，探讨两者可能存在的潜在关系。方法 报道两例诊断CMML合并MGUS病例，同时复习有关MDS或MPN与MGUS并存病例的文献。结果 病例1为77岁男性，血常规发现血象异常，诊断为慢性粒单细胞白血病伴意义未明IgM型单克隆球蛋白病，病程4年，CMML逐渐进展，表现为贫血、血小板减少、自身免疫性溶血、骨髓幼稚细胞增加等，而MGUS尽管IgM数值高低波动，却并未出现IgM相关的脏器损害，最终死于肺部感染。病例2为78岁男性，因发热、咳嗽就诊后发现外周血单核细胞升高，骨髓涂片提示粒系明显活跃，早中晚幼粒比例增高，可见病态粒细胞，单核细胞比例增高，可见幼稚单核细胞。血清IgG升高，免疫固定电泳发现IgG-K型M蛋白，IgG-λ型M蛋白，诊断为慢性粒单细胞白血病伴意义未明IgG型单克隆球蛋白病，随访1年，两种疾病稳定。结论 结合本文病例并复习相关文献，上述两种肿瘤共存于一机体，病程发展各不相同，推测可能为不同克隆起源。MGUS能否作为一种MDS或MPN/MDS的危险因素或者相反、克隆性浆细胞在急性白血病发生发展中的作用尚需进一步研究。 |
| 英文摘要: |
| To describe myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) combined with
monoclonal gammopathy of undetermined significance (MGUS) in order to investigate the potential
association between these 2 diseases. Two cases of confirmed chronic myelomonocytic leukemia (CMML)
combined with MGUS were reported. In addition, prior publications of cases with combined MDS or MPN
with MGUS were reviewed. The first case was of a 77-year-old man whose routine blood tests showed
abnormal hemogram results. The diagnosis was CMML combined with IgM monoclonal gammopathy,
and the disease course was 4 years. The CMML gradually progressed and the patient presented with
anemia, thrombocytopenia, autoimmune hemolysis, and an increase in the number of immature cells in
the bone marrow. Although the MGUS caused fluctuations in the concentrations of IgM, no IgM-associated
organ damage was observed. Eventually, this patient died from a lung infection. The second case was
of a 78-year-old man who sought treatment because of fever and a cough. An increase in the number of
monocytes was discovered in the peripheral blood. Bone marrow smear results suggested obvious active
granulocytes and an increase in the percentages of promyelocytes, myelocytes, and metamyelocytes.
Unhealthy granulocytes and immature monocytes could also be observed, and the percentage of monocytes
was increased. In addition, serum IgG levels were increased, and immunofixation electrophoresis results
showed IgG-κ type M proteins. The diagnosis was CMML combined with IgG monoclonal gammopathy.
These diseases were stable and follow-up was conducted for 1 year after diagnosis. The cases in this
study combined with those that were reviewed in the relevant literature indicate that the presence of these
2 diseases in the same patient might not be a coincidence. The development of the 2 diseases in case 1
was different, and we speculate that they might have had different clonal origins. Whether CMML is a risk
factor for MGUS and the role of clonal plasma cells in the occurrence and development of MDS and MDS/
MPN requires further studies on a larger number of cases. |
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