Blood Res.  2016 Mar;51(1):23-30. 10.5045/br.2016.51.1.23.

Plasma cell leukemia in North India: retrospective analysis of a distinct clinicohematological entity from a tertiary care center and review of literature

• Bommannan
• Sachdeva M
• Malhotra P
• Kumar N
• Sharma P
• Naseem S
• Ahluwalia
• Das R
• Varma N
• Prakash G
• Khadwal A
• Srinivasan R
• Varma S

• KMID: 2172741
• DOI: http://doi.org/10.5045/br.2016.51.1.23

Abstract

BACKGROUND
Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm. In PCL, clonal plasma cells comprise ≥20% of the peripheral blood (PB) leukocytes and/or the absolute clonal PB plasma cell count is ≥2×10(9)/L. Primary PCL (PPCL) originates de novo, whereas, secondary PCL (SPCL) evolves from pre-existing multiple myeloma.
METHODS
Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively.
RESULTS
Between January 2007 and December 2014, ten PPCL and four SPCL patients were investigated (8 PPCLs and 3 SPCLs had complete clinical data). All were North Indians, sharing common geography and ethnicity. Our cohort showed less frequent renal failure, more frequent hepatomegaly, and non-secretory type disease. In contrast to western literature, flow cytometric immunophenotyping of our cohort revealed altered expression of CD138 (67%), CD56 (33%), and CD20 (0%). With novel therapeutic agents, these PPCL patients had a median overall survival of 15 months.
CONCLUSION
We highlight that our PPCL patients from North India had distinct clinicohematological and immunophenotypic profiles. The significance of our findings must be tested in a larger patient cohort and must be supported by molecular and cytogenetic investigations to unmask possible significant effects on pathogenesis.

Keyword

Clinicohematological profile; North India; Plasma cell leukemia; Immunophenotype; Survival

MeSH Terms

Cohort Studies
Cytogenetics
Geography
Hepatomegaly
Humans
Immunophenotyping
India*
Leukemia, Plasma Cell*
Leukocytes
Multiple Myeloma
Neoplasms, Plasma Cell
Plasma Cells*
Plasma*
Renal Insufficiency
Retrospective Studies*
Tertiary Care Centers*
Tertiary Healthcare*

Figure

• 

  Fig. 1 (A, B) Circulating plasma cells with lymphoplasmacytoid morphology, (C, D) circulating plasma cells with blast like morphology (Magnification ×1,000, May-Grünwald-Giemsa staining).

Reference

1. Kyle RA, Maldonado JE, Bayrd ED. Plasma cell leukemia. Report on 17 cases. Arch Intern Med. 1974; 133:813–818. PMID: 4821776.

2. Swerdlow SH, Campo E, Harris NL, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. Lyon, France: IARC Press;2008.

3. Fernández de Larrea C, Kyle RA, Durie BG, et al. Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia. 2013; 27:780–791. PMID: 23288300.

4. Cifola I, Lionetti M, Pinatel E, et al. Whole-exome sequencing of primary plasma cell leukemia discloses heterogeneous mutational patterns. Oncotarget. 2015; 6:17543–17558. PMID: 26046463.

5. Raj RS, Najeeb S, Aruna R, Pavithran K, Thomas M. Primary plasma cell leukemia occuring in the young. Indian J Cancer. 2003; 40:116–117. PMID: 14716116.

6. Majumdar N, Kumar R, Anand M, et al. Plasma cell leukemia-a study of 28 cases from India. Hematology. 2009; 14:198–203. PMID: 19635182.

7. Johnson MR, Del Carpio-Jayo D, Lin P, et al. Primary plasma cell leukemia: morphologic, immunophenotypic, and cytogenetic features of 4 cases treated with chemotherapy and stem cell transplantation. Ann Diagn Pathol. 2006; 10:263–268. PMID: 16979517.

8. Woodruff RK, Malpas JS, Paxton AM, Lister TA. Plasma cell leukemia (PCL): A report on 15 patients. Blood. 1978; 52:839–845. PMID: 687831.

9. Noel P, Kyle RA. Plasma cell leukemia: an evaluation of response to therapy. Am J Med. 1987; 83:1062–1068. PMID: 3503574.

10. Dimopoulos MA, Palumbo A, Delasalle KB, Alexanian R. Primary plasma cell leukaemia. Br J Haematol. 1994; 88:754–759. PMID: 7819100.

11. García-Sanz R, Orfão A, Gonzalez M, et al. Primary plasma cell leukemia: clinical, immunophenotypic, DNA ploidy, and cytogenetic characteristics. Blood. 1999; 93:1032–1037. PMID: 9920853.

12. Saccaro S, Fonseca R, Veillon DM, et al. Primary plasma cell leukemia: report of 17 new cases treated with autologous or allogeneic stem-cell transplantation and review of the literature. Am J Hematol. 2005; 78:288–294. PMID: 15795922.

13. Musto P, Rossini F, Gay F, et al. Efficacy and safety of bortezomib in patients with plasma cell leukemia. Cancer. 2007; 109:2285–2290. PMID: 17469169.

14. Albarracin F, Fonseca R. Plasma cell leukemia. Blood Rev. 2011; 25:107–112. PMID: 21295388.

15. Gogia A, Raina V, Gupta R. Massive ascites as an presenting feature of plasma cell leukemia. Indian J Cancer. 2013; 50:301.

16. Tembhare PR, Subramanian PG, Sehgal K, et al. Immunophenotypic profile of plasma cell leukemia: a retrospective study in a reference cancer center in India and review of literature. Indian J Pathol Microbiol. 2011; 54:294–298. PMID: 21623077.

17. Naseem S, Kaur S, Gupta R, Kashyap R, Nityanand S. Plasma cell leukemia: case series from a tertiary center with review of literature. Indian J Hematol Blood Transfus. 2012; 28:10–14. PMID: 23449039.

18. Kar R, Priyadarshini SG, Niraimathi M, Basu D, Badhe BA. Clinico-pathological spectrum of primary plasma cell leukemia diagnosed at a tertiary care centre in South India over 5 year period. Indian J Hematol Blood Transfus. 2012; 28:170–174. PMID: 23997454.

19. Majhi U, Murhekar K, Sundersingh S, Rajalekshmi KR. Primary plasma cell leukaemia with unusual presentations: a case series. Indian J Hematol Blood Transfus. 2014; 30(Suppl 1):390–393. PMID: 25332628.

20. Agarwal AD, Brahmbhatt BS, Parikh BP, Shah MJ. Plasma cell leukemia: a retrospective study of 10 cases. Indian J Cancer. 2014; 51:18–19. PMID: 24947090.

21. Rajeswari G, Paul TR, Uppin MS, et al. Plasma cell leukemia: A case series from South India with emphasis on rarer variants. Indian J Med Paediatr Oncol. 2014; 35:211–214. PMID: 25336792.

22. Durie BG, Harousseau JL, Miguel JS, et al. International uniform response criteria for multiple myeloma. Leukemia. 2006; 20:1467–1473. PMID: 16855634.

23. Gluzinski A, Reichenstein M. Myeloma und leucaemia lymphatica plasmocellularis. Wien Klin Wochenschr. 1906; 12:336–339.

24. Pagano L, Valentini CG, De Stefano V, et al. Primary plasma cell leukemia: a retrospective multicenter study of 73 patients. Ann Oncol. 2011; 22:1628–1635. PMID: 21252060.

25. Royer B, Magrangeas F, Lioure B, et al. First large prospective study for patients with primary plasma cell leukemia: Bortezomib-doxorubicine-dexamethasone/bortezomib-cyclophosphamidedexamethasone regimens as induction before stem cell transplantation followed by consolidation with lenalidomide-bortezomib-dex or allograft. (a study of the IFM group) Blood (ASH Annual Meeting Abstracts). 2013; 122(Suppl):761.

26. Peijing Q, Yan X, Yafei W, et al. A retrospective analysis of thirty-one cases of plasma cell leukemia from a single center in China. Acta Haematol. 2009; 121:47–51. PMID: 19339770.

27. van de Donk NW, Lokhorst HM, Anderson KC, Richardson PG. How I treat plasma cell leukemia. Blood. 2012; 120:2376–2389. PMID: 22837533.

28. Colović M, Janković G, Suvajdzić N, Milić N, Dordević V, Janković S. Thirty patients with primary plasma cell leukemia: a single center experience. Med Oncol. 2008; 25:154–160. PMID: 18488157.

29. Tiedemann RE, Gonzalez-Paz N, Kyle RA, et al. Genetic aberrations and survival in plasma cell leukemia. Leukemia. 2008; 22:1044–1052. PMID: 18216867.

30. Toma VA, Retief FP, Potgieter GM, Anderson JD. Plasma cell leukaemia. Diagnostic problems in our experience with 11 cases. Acta Haematol. 1980; 63:136–145. PMID: 6769279.