Lab Med Online.  2023 Jan;13(1):36-41. 10.47429/lmo.2023.13.1.36.

Therapeutic Leukapheresis for Plasma Cell Leukemia Presenting with Hyperleukocytosis: The First Case in Korea

• Park S
• Park JH
• Chung Y
• Ko DH
• Kim H

• KMID: 2552723
• DOI: http://doi.org/10.47429/lmo.2023.13.1.36

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 335.60×10 ⁹ /L, plasma cell 88%) and leukostasis. Although the leukocytes in peripheral blood were effectively removed (leukocyte count 171.29×10 ⁹ /L, 48% reduction after first TL; leukocyte count 84.53×10 ⁹ /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.

Keyword

Leukapheresis; Plasma cell leukemia; Leukocytosis; Multiple myeloma

Figure

• 

  Fig. 1 Images of the patient’s relevant test results during the clinical course. (A) Sagittal T1-weighted spine MRI showing diffuse myeloma involvement in the axial skeleton and T11/12 compression fracture with T12 significant retropulsion at the time of initial diagnosis as non-secretory myeloma. (B) Serum protein electrophoresis showing no monoclonal spike when diagnosed as plasma cell leukemia. (C) Serum immunofixation electrophoresis showing no band when diagnosed as plasma cell leukemia. Abbreviation: MRI, magnetic resonance imaging.

• 

  Fig. 2 Images related to the patient at the time of therapeutic leukapheresis. (A) Morphological features of the peripheral blood smear (Wright-Giemsa stain, ×1,000) on hospital day 1 showing numerous abnormal plasma cells. (B) Product of the first therapeutic leukapheresis procedure performed on hospital day 2. A thick layer of white blood cells is present in the middle area.

• 

  Fig. 3 Changes in peripheral blood WBC count and serum creatinine level before and after admission. A significant increase in the WBC count was observed at the time of admission compared to 58 and 28 days before admission. The WBC count effectively decreased after therapeutic leukapheresis. Solid arrows indicate the days on which therapeutic leukapheresis was performed. The hollow arrow indicates the duration of chemotherapy from hospital day 5 to day 8. Abbreviation: WBC, white blood cell.

Reference

1. Fernández de Larrea C, Kyle RA, Durie BG, Ludwig H, Usmani S, Vesole DH, et al. 2013; Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia. 27:780–91. DOI: 10.1038/leu.2012.336. PMID: 23288300. PMCID: PMC4112539.

2. Sant M, Allemani C, Tereanu C, De Angelis R, Capocaccia R, Visser O, et al. 2010; Incidence of hematologic malignancies in Europe by morphologic subtype: results of the HAEMACARE project. Blood. 116:3724–34. DOI: 10.1182/blood-2010-05-282632. PMID: 20664057.

3. Godbey EA, Dalton JB, Lai G, Sanford KW, Roseff SD. 2020; First reported case of leukocytapheresis in plasma cell leukemia. Transfusion. 60:11–2. DOI: 10.1111/trf.15564. PMID: 31682280.

4. Noel P, Kyle RA. 1987; Plasma cell leukemia: An evaluation of response to therapy. Am J Med. 83:1062–8. DOI: 10.1016/0002-9343(87)90942-9. PMID: 3503574.

5. Tuazon SA, Holmberg LA, Nadeem O, Richardson PG. 2021; A clinical perspective on plasma cell leukemia; current status and future directions. Blood Cancer J. 11:23. DOI: 10.1038/s41408-021-00414-6. PMID: 33563906. PMCID: PMC7873074.

6. Granell M, Calvo X, Garcia-Guiñón A, Escoda L, Abella E, Martínez CM, et al. 2017; Prognostic impact of circulating plasma cells in patients with multiple myeloma: implications for plasma cell leukemia definition. Haematologica. 102:1099–104. DOI: 10.3324/haematol.2016.158303. PMID: 28255016. PMCID: PMC5451342.

7. Ravi P, Kumar SK, Roeker L, Gonsalves W, Buadi F, Lacy MQ, et al. 2018; Revised diagnostic criteria for plasma cell leukemia: results of a Mayo Clinic study with comparison of outcomes to multiple myeloma. Blood Cancer J. 8:116. DOI: 10.1038/s41408-018-0140-1. PMID: 30442928. PMCID: PMC6238010.

8. Ramsingh G, Mehan P, Luo J, Vij R, Morgensztern D. 2009; Primary plasma cell leukemia: a Surveillance, Epidemiology, and End Results database analysis between 1973 and 2004. Cancer. 115:5734–9. DOI: 10.1002/cncr.24700. PMID: 19877113.

9. Okafor C, Ward DM, Mokrzycki MH, Weinstein R, Clark P, Balogun RA. 2010; Introduction and overview of therapeutic apheresis. J Clin Apher. 25:240–9. DOI: 10.1002/jca.20247. PMID: 20806281.

10. Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. 2019; 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. 34:171–354. DOI: 10.1002/jca.21705. PMID: 31180581.

11. Kwaan HC. 2013; Hyperviscosity in plasma cell dyscrasias. Clin Hemorheol Microcirc. 55:75–83. DOI: 10.3233/CH-131691. PMID: 23455837.

12. Mehta J, Singhal S. 2003; Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 29:467–71. DOI: 10.1055/s-2003-44554. PMID: 14631546.

13. Kosmo MA, Gale RP. 1988; Plasma cell leukemia with IgA paraproteinemia and hyperviscosity. Am J Hematol. 28:113–5. DOI: 10.1002/ajh.2830280210. PMID: 3394712.

14. Geraci JM, Hansen RM, Kueck BD. 1990; Plasma cell leukemia and hyperviscosity syndrome. South Med J. 83:800–5. DOI: 10.1097/00007611-199007000-00020. PMID: 2196691.

15. Giammarco S, Chiusolo P, Piccirillo N, Di Giovanni A, Metafuni E, Laurenti L, et al. 2017; Hyperleukocytosis and leukostasis: management of a medical emergency. Expert Rev Hematol. 10:147–54. DOI: 10.1080/17474086.2017.1270754. PMID: 27967252.

16. Pagano L, Valentini CG, De Stefano V, Venditti A, Visani G, Petrucci MT, et al. 2011; Primary plasma cell leukemia: a retrospective multicenter study of 73 patients. Ann Oncol. 22:1628–35. DOI: 10.1093/annonc/mdq646. PMID: 21252060.

17. Jurczyszyn A, Radocha J, Davila J, Fiala MA, Gozzetti A, Grząśko N, et al. 2018; Prognostic indicators in primary plasma cell leukaemia: a multicentre retrospective study of 117 patients. Br J Haematol. 180:831–9. DOI: 10.1111/bjh.15092. PMID: 29315478.

18. Jurczyszyn A, Castillo JJ, Avivi I, Czepiel J, Davila J, Vij R, et al. 2019; Secondary plasma cell leukemia: a multicenter retrospective study of 101 patients. Leuk Lymphoma. 60:118–23. DOI: 10.1080/10428194.2018.1473574. PMID: 29965787.

19. Moiz B, Ali SS. 2012; Plasma cell leukemia in pregnancy. Blood. 120:3633. DOI: 10.1182/blood-2012-04-425074. PMID: 23281530.

20. Rastogi P, Ahluwalia J, Parathan KK, Malhotra P. 2017; Plasma cell leukemia presenting with hyperleukocytosis and anaplasia. Indian J Hematol Blood Transfus. 33:128–9. DOI: 10.1007/s12288-016-0740-5. PMID: 28194071. PMCID: PMC5280869.