Loffier's endocarditisを伴ったHypereosinophilic syndromeの1例

A case of hypereosinophilic syndrome associated with Löffler's endocarditis

　1936年Löffler^1）は，進行性うっ血性心不全に好酸球増多を伴い，剖検上両心室壁の心内膜血栓とその直下の線維性増殖を認める症例（Löffler’s Fibroplastic Parie—tal Endocarditis）を一つの独立した疾患像として報告した。それ以来，著明な好酸球増多と臓器障害を呈する類縁疾患としてレフラー症候群，播種性好酸球性膠原病，好酸球性白血病，アレルギー性肉芽腫性血管炎などが報告されているが，これらの疾患概念については不明な点が多い。1968年，HandyおよびAnderson^2）が著明な成熟型好酸球増多，心肺症状，肝脾腫大を合併する症候群をHypereosinophilic syndrome （以下HES）と呼ぶことを提唱して以来，本症候群の概念が固まりつつある。今回我々は，臨床的検索のごく初期に大動脈の巨大血栓で急死したHESの1例を経験したので，病理学的特徴を述べるとともに，若干の考察を加え報告する。

This is a case report of a 29 year-old male with hypereosinophilic syndrome associated with Löffler's endocarditis.

The patient was relatively well except for recent onset of palpitation and shortness of breath. At a time of laboratory examination for blood donation, he was pointed out to have moderate degree of anemia. Other laboratory examination showed WBC 16,100/mm³ and eosinophils 65%. Just before begin-ing of further detail examination, he suddenly fell down without consciousness on the way to office. He was transferred to cur hospital but was dead soon after.

The autopsy findings revealed large mural thrombi filling both ventricles of the heart. The large casts of the thrombi stripped off from the left ventricle, resulting in aortic emboli at the portion of the aortic arch and the thoracic aorta. Microscopically, diffuse infiltration of mature eosinophils were found in various organs, that is, the bone marrow, liver, spleen and lungs. In addition to the above findings, small pulmonary and splenic arteries showed the perivascular cuffing of eosinophils with thromboe-mbolic occlusion.

As this case, the difficult for establishing the diagnosis is present because of many overlaps bet-ween Loffler's endocarditis, disseminated eosinophilic collagen disease, eosinophilic leukemia, etc. There-fore, it is conceivable that this case has closer relationship to the broader concept of hypereosino-philic syndrome.