A comparison of haemophagocytic syndrome signs in aortic aneurysm and angina pectoris cases at postoperative death: HPS diagnosis and prediction

Abstract

Although hemophagocytic syndrome (HPS) signs are more severe in aortic aneurysm cases than in angina pectoris cases, HPS-associated postoperative death should be considered more in both cases. This study analyzed a total of 183 Japanese patients, with 101 aneurysms, 79 anginas, and 3 with both, who underwent aortic replacement (AR) or/and aortocoronary bypass grafting (ACBG) during a period of 1.92 years. In seven aneurysm and eight angina cases with 7.6–12.1 g/dl of hemoglobin (Hb), iron metabolism was studied. As clinical outcomes, diabetes, chronic renal failure (CRF), and thrombosis were more prevalent in the angina cases than in the aneurysm cases. In six cases of aneurysm (6:101) who died after AR, five who died of acute myocardial infarction (AMI) or pulmonary embolism (PE) during postoperative 3–101 days developed HPS, and no detail data was available for one case. Among the five diabetic cases of angina (5:79) who died 7–74 days after ACBG, four cases with cerebral infarction (CI) had infection-associated HPS, and one case had severe kidney atrophy without CI and HPS. Postoperative HPS signs in the aneurysm cases were also generally more severe than those of the angina cases. The aneurysm case with no CRF showed mostly typical HPS and died of AMI. She had pancytopenia, anemia with anisocytosis and nucleated red cells, leukopenia with immature myeloid cells, thrombocytopenia with coagulopathy, elevated lactate dehydrogenase, hyperbilirubinemia, liver dysfunction, and numerous phagocytes containing hemosiderin in the specimen of the aorta examined before the operation. Preoperatively, she also showed high levels of serum ferritin (SF) and low levels of both serum iron (S–Fe) and total iron-binding capacity (TIBC). All the 15 patients in whom iron metabolism was evaluated had low levels of S–Fe. Low levels of TIBC and high levels of SF were observed in 11:15 patients. Cardiac hemophagocytosis with no CRF was a suggestive of HPS. AR and ACBG operations became a direct trigger of HPS. Postoperative AMI, sepsis, and PE became a HPS trigger, but CI alone and CRF did not. For postoperative survival, it was important to protect the patients from infections.