Landry Ian, Vest Mallorie, Williams Anthony
Medicine, Icahn School of Medicine at Mount Sinai, NYC Health + Hospitals/Queens, Queens, USA.
Internal Medicine, Icahn School of Medicine at Mount Sinai, NYC Health + Hospitals/Queens, Queens, USA.
Cureus. 2021 Oct 27;13(10):e19085. doi: 10.7759/cureus.19085. eCollection 2021 Oct.
Myelodysplastic syndrome (MDS) is a premalignant condition characterized by clonal proliferation and ineffective hematopoiesis. The subtype of MDS associated with deletion in the long arm of chromosome 5 is generally associated with older females and carries a good prognosis as it rarely transforms to acute myeloid leukemia. The mechanisms of leukemic transformation are still poorly understood and likely involve a variety of somatic mutations and epigenetic modifications. We present the case of a 70-year-old female with known MDS with deletion 5(q) who presented with anemia, thrombocytopenia, and guaiac positive stool who was subsequently found to be positive for infection. During the course of her treatment, she developed significant leukocytosis, splenic infarction, and acute hypoxic respiratory failure requiring high flow nasal cannula. Flow cytometry returned positive for increased blasts of more than 30%. She was transferred to a tertiary care facility for cytoreductive therapy and developed leukostasis and Sweet's syndrome.
骨髓增生异常综合征(MDS)是一种以克隆性增殖和无效造血为特征的癌前病变。与5号染色体长臂缺失相关的MDS亚型通常与老年女性相关,预后良好,因为它很少转化为急性髓系白血病。白血病转化的机制仍知之甚少,可能涉及多种体细胞突变和表观遗传修饰。我们报告一例70岁女性,已知患有5(q)缺失的MDS,表现为贫血、血小板减少和粪便隐血阳性,随后被发现感染呈阳性。在治疗过程中,她出现了显著的白细胞增多、脾梗死和急性低氧性呼吸衰竭,需要高流量鼻导管吸氧。流式细胞术检测显示原始细胞增加超过30%呈阳性。她被转至三级医疗机构进行减瘤治疗,并出现了白细胞淤滞和Sweet综合征。