期刊
ONCOLOGIST
卷 27, 期 9, 页码 751-759出版社
OXFORD UNIV PRESS
DOI: 10.1093/oncolo/oyac093
关键词
signet ring; microangiopathic hemolytic anemia; gastric cancer; systematic review; case-control study
类别
Microangiopathic hemolytic anemia (MAHA) is a rare paraneoplastic syndrome associated with gastric signet ring cell carcinoma (SRCC), and its clinical and prognostic features have been poorly described. Patients with MAHA commonly present with symptoms such as back pain, bone pain, and dyspnea. The study found that patients with gastric SRCC and MAHA have significantly shorter survival compared to those with metastatic gastric SRCC alone.
Background: Microangiopathic hemolytic anemia (MAHA) is a rare paraneoplastic syndrome that has been reported in patients with gastric signet ring cell carcinoma (SRCC). Clinical and prognostic features of MAHA in this setting have been poorly described. Materials and Methods: We conducted a systematic review in 8 databases of gastric SRCC complicated by MAHA and performed a case-control study assessing factors associated with survival in patients with gastric SRCC and MAHA in our pooled cohort compared with age-, sex-, and stage-matched cases of gastric SRCC from the Surveillance, Epidemiology, and End Results (SEER) database. Descriptive analyses were performed and multivariable Cox-proportional hazards regression modeling was used to determine factors associated with overall survival. Results: All identified patients (n = 47) were symptomatic at index presentation, commonly with back/bone pain, and dyspnea. Microangiopathic hemolytic anemia was the first manifestation of gastric SRCC in 94% of patients. Laboratory studies were notable for anemia (median 7.7 g/dL), thrombocytopenia (median 45.5 x 10(3)/mu L), and hyperbilirubinemia (median 2.3 mg/dL). All patients with MAHA had metastatic disease at presentation, most often to the bone, bone marrow, and lymph nodes. Median survival in patients with gastric SRCC and MAHA was significantly shorter than a matched SEER-derived cohort with metastatic gastric SRCC (7 weeks vs 28 weeks, P< .01). In multivariate analysis, patients with MAHA were at significantly increased risk of mortality (HR 3.28, 95% CI 2.11-5.12). Conclusion: Microangiopathic hemolytic anemia is a rare, late-stage complication of metastatic gastric SRCC and is associated with significantly decreased survival compared with metastatic gastric SRCC alone.
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