Acute Promyelocytic Leukemia Developed in a Patient with Systemic Lupus Erythematosus

Abstract

A 19-year-old, woman who had been diagnosed as systemic lupus erythematosus (SLE) a year ago, was admitted because of fever, dizziness, and sustained postoperative bleeding after a hemorroidectomy. On admission, a CBC revealed pancytopenia (Hb 6.2 g/dL, WBC 1,200/μL, platelets 11,000/μL) with a shift to the left, and the FDP and D-dimer were positive. She was treated for sepsis and disseminated intravascular coagulation. Granulocyte colony-stimulating factor(G-CSF) was administrated twice for severe neutropenia. An increase in WBC and immature myeloid cells, mainly hypergranular promyelocytes on the peripheral blood followed and was considered to be the effect of G-CSF. To evaluate the cause of pulmonary infiltrates, bronchoalveolar lavage (BAL) was peformed on the 5th day of admission. The BAL fluid revealed many promyelocytes and myelocytes with occasional structures recognized as Auer rods. Acute promyelocytic leukemia (APL) was confirmed on the bone marrow study and chromosome analysis. Unfortunately, the patient died of septic shock on the 9th day of admission. We report here a very rare case of APL diagnosed in a SLE patient, the diagnosis of which was somewhat delayed due to the use of G-CSF and superimposed sepsis.

일년 전 SLE로 진단받은 19세 여자 환자가 치핵절제술 후 발열, 현기증 및 수술부위 출혈로 입원하였다. 입원당시 CBC는 좌방이동을 동반한 범혈구감소증(혈색소 6.2 g/dL, 백혈구수1,20/ L, 혈소판수 11,000/ L)을 보였으며, 섬유소분해산물과D-dimer는 양성이었다. 패혈증 및 DIC로 치료하였으며, 중증 호중구감소증으로 G-CSF를 두 차례 투여하였다. 말초혈액에서 과립이 많은 전골수구가 대부분인 미성숙 골수구와 백혈구의 증가가 관찰되었으나, G-CSF를 투여한 효과로 간주되었다. 입원 5일째, 폐 침윤의 원인을 규명하기 위한 기관지폐포세척액에서 Auer체를 포함하고 있는 전골수구가 관찰되어 골수검사 및 염색체 검사를 하였고, 급성전골수성백혈병으로 진단되었다. 환자는 입원 9일째, 패혈증성 쇽으로 사망하였다. 저자들은 SLE 환자에서 G-CSF의 사용 및 패혈증으로 인하여 진단이 지연되었던 급성전골수성백혈병이 동반된 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.