고백혈구증가증을 동반한 형질세포백혈병에서 치료적 백혈구성분채집술 1예: 국내 첫 증례
Therapeutic Leukapheresis for Plasma Cell Leukemia Presenting with Hyperleukocytosis: The First Case in Korea
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초록
형질세포백혈병은 형질세포질환 중에서 드물게 발생한다. 형질세포백혈병에서 고감마글로불린혈증에 대해 치료적 혈장교환술을 시행한 보고는 있으나, 고백혈구증가증에 대해 치료적 백혈구성분채집술을 시행한 사례는 국내에서 보고된 바가 없기에 문헌 고찰과 함께 이 증례를 보고하고자 한다. 78세 여자 환자가 내원 5년 전 비분비성 골수종으로 진단된 후 치료 중이었으나, 6개월 전 이차성 형질세포백혈병으로 진단 받고 항암화학요법을 변경하였다. 내원 1주일 전부터 시작된 호흡곤란과 기억력 저하로 입원하였으며, 고백혈구증가증(백혈구 수 335.60×109/L, 형질세포 88%) 및 백혈구울혈에 인한 증상의 완화를 위하여 치료적 백혈구성분채집술을 2회 시행하였다. 말초혈액의 백혈구는 효과적으로 제거되었으나(첫 번째 시술 후 백혈구 수 171.29×109/L로 48% 감소, 두 번째 시술 후 백혈구 수 84.53×109/L로 74% 감소), 섬망으로 인한 의사소통의 어려움으로 증상의 호전 여부는 정확히 평가하기 어려웠다. 이후 항암화학요법을 시행하였으나 다발성 장기부전이 발생하였고 환자는 내원 13일째 사망하였다. 본 증례는 드물게 발생하는 고백혈구증가증이 동반된 형질세포백혈병에 대한 기존 문헌에서의 사례 대비 백혈구 수가 가장 높았으며, 비록 환자가 사망하였으나 치료적 백혈구성분채집술을 시행하여 백혈구 수를 성공적으로 감소시키고 질병의 악화를 지연시키는 단기적 효과의 가능성을 제시한 국내 첫 번째 사례라는 데 의의가 있다
Abstract
Plasma cell leukemia (PCL) is a rare plasma cell disorder. Although therapeutic plasma exchange has reportedly been performed for hypergammaglobulinemia in PCL, there are no reports of therapeutic leukapheresis (TL) having been performed for hyperleukocytosis in Korea. Here, we report a rare case of TL performed for hyperleukocytosis in PCL with a literature review. A 78-year-old female patient was diagnosed and treated for nonsecretory myeloma 5 years ago before the present visit. She was diagnosed with secondary PCL 6 months ago after which her chemotherapy regimen was changed. The patient developed worsening dyspnea and memory loss one week before admission. She was hospitalized and two sessions of TL were performed to alleviate the symptoms caused by hyperleukocytosis (leukocyte count 335.60×109/L, plasma cell 88%) and leukostasis. Although the leukocytes in peripheral blood were effectively removed (leukocyte count 171.29×109/L, 48% reduction after first TL; leukocyte count 84.53×109/L, 74% reduction after second TL), it was difficult to accurately assess whether the symptoms improved, owing to communication difficulties due to delirium. Although chemotherapy was administered, the patient died of multiple organ failure on the 13th day after admission. This case presented the highest leukocyte count among reports in the existing literature on rare cases of PCL with hyperleukocytosis. Although the patient eventually died, this is the first case of TL performed to successfully decrease leukocytes in PCL in Korea, presenting the potential for delaying the exacerbation of disease progression.
참고문헌 (20)
- Fernández de Larrea C, "Plasma cell leukemia : consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group", Leukemia, 27, pp.780~791 (2013)
- Sant M, "Incidence of hematologic malignancies in Europe by morphologic subtype : results of the HAEMACARE project", Blood, 116, pp.3724~3734 (2010)
- Godbey EA, "First reported case of leukocytapheresis in plasma cell leukemia", Transfusion, 60, pp.11~12 (2020)
- Noel P, "Plasma cell leukemia : An evaluation of response to therapy", Am J Med, 83, pp.1062~1068 (1987)
- Tuazon SA, "A clinical perspective on plasma cell leukemia; current status and future directions", Blood Cancer J, 11, pp.23~ (2021)
- Granell M, "Prognostic impact of circulating plasma cells in patients with multiple myeloma : implications for plasma cell leukemia definition", Haematologica, 102, pp.1099~1104 (2017)
- Ravi P, "Revised diagnostic criteria for plasma cell leukemia : results of a Mayo Clinic study with comparison of outcomes to multiple myeloma", Blood Cancer J, 8, pp.116~ (2018)
- Ramsingh G, "Primary plasma cell leukemia : a Surveillance, Epidemiology, and End Results database analysis between 1973 and 2004", Cancer, 115, pp.5734~5739 (2009)
- Okafor C, "Introduction and overview of therapeutic apheresis", J Clin Apher, 25, pp.240~249 (2010)
- Padmanabhan A, "Guidelines on the Use of Therapeutic Apheresis in Clinical Practice — Evidence-Based Approach from the Writing Committee of the American Society for Apheresis : The Eighth Special Issue", J Clin Apher, 34, pp.171~354 (2019)
- Kwaan HC, "Hyperviscosity in plasma cell dyscrasias", Clin Hemorheol Microcirc, 55, pp.75~83 (2013)
- Mehta J, "Hyperviscosity syndrome in plasma cell dyscrasias", Semin Thromb Hemost, 29, pp.467~471 (2003)
- Kosmo MA, "Plasma cell leukemia with IgA paraproteinemia and hyperviscosity", Am J Hematol, 28, pp.113~115 (1988)
- Geraci JM, "Plasma cell leukemia and hyperviscosity syndrome", South Med J, 83, pp.800~805 (1990)
- Giammarco S, "Hyperleukocytosis and leukostasis : management of a medical emergency", Expert Rev Hematol, 10, pp.147~154 (2017)
- Pagano L, "Primary plasma cell leukemia : a retrospective multicenter study of 73 patients", Ann Oncol, 22, pp.1628~1635 (2011)
- Jurczyszyn A, "Prognostic indicators in primary plasma cell leukaemia : a multicentre retrospective study of 117 patients", Br J Haematol, 180, pp.831~839 (2018)
- Jurczyszyn A, "Secondary plasma cell leukemia : a multicenter retrospective study of 101 patients", Leuk Lymphoma, 60, pp.118~123 (2019)
- Moiz B, "Plasma cell leukemia in pregnancy", Blood, 120, pp.3633~ (2012)
- Rastogi P, "Plasma cell leukemia presenting with hyperleukocytosis and anaplasia", Indian J Hematol Blood Transfus, 33, pp.128~129 (2017)