무증상골수종이 동반된 경쇄아밀로이드증 환자의 출혈 경향
Bleeding Tendency of a Light Chain (AL) Amyloidosis Patient Accompanied by Asymptomatic Plasma Cell Myeloma
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초록
최근 발생한 출혈성 경향을 주소로 내원한 66세 남자 환자에서무증상골수종이 동반된 아밀로이드증을 진단하였다. 이 환자에서관찰되는 출혈경향의 원인을 파악하기 위해 시행된 응고검사결과,첫째는 PT와 aPTT가 연장되면서 혼합검사에서 교정되지 않고, 응고인자 I, II, V, VII, 그리고 X가 거의 정상수치를 보여 비특이적 억제인자의 존재가능성이 제시되었다. 둘째로 PFA-100의 폐쇄시간의 연장과 ristocetin에 대한 혈소판응집반응도 소실된 소견으로후천폰빌레브란트증후군이 의심되었다. 기저질환에 대한 치료 후,환자의 출혈 증상은 호전되었다. 아밀로이드증 환자가 출혈성향을보이는 경우 후천폰빌레브란트증후군의 가능성을 고려하고 이를확인하는 것이 필요하다.
Abstract
We present a case of abrupt-onset hemorrhagic tendency in a patient with amyloidosis who also had asymptomatic plasma cell myeloma. The patient was a 66-yr-old man with no previous history of hemorrhagic tendency and no family history of hemorrhagic disease. On examination, the prothrombin time and activated partial thromboplastin time were found to be prolonged and were not corrected even after a mixing test; moreover,the levels of coagulation factors I, II, V, VII, and X were almost normal. We therefore considered the presence of a nonspecific coagulation inhibitor. Although the von Willebrand factor (vWF) activity and vWF antigen level were normal due to sampling following transfusion, the increased closure time on PFA-100 (Siemens) analysis and the absence of ristocetin-induced platelet aggregation suggested the presence of acquired von Willebrand syndrome (vWS). After chemotherapy, the patient showed alleviation in the bleeding symptoms. Therefore, testing for acquired vWS should be considered when a patient has a history of recent bleeding with underlying amyloidosis.
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