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  Table of Contents  
ORIGINAL ARTICLE
Year : 2016  |  Volume : 53  |  Issue : 4  |  Page : 619-620
 

Plasma cell leukemia: Single institution experience


Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India

Date of Web Publication21-Apr-2017

Correspondence Address:
Ankit Agarwal
Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.204775

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 » Abstract 

BACKGROUND: The first case of plasma cell leukemia (PCL) was recognized by Gluzinski and Reichentein. It is the most aggressive among the monoclonal gammopathies. It is diagnosed by the presence of more than 20% plasma cells in the peripheral blood or an absolute plasma cell count of >2000/mm3. Because of the relatively low incidence, most data come from case reports and retrospective studies. No prospective series have been published, and only seven reports including more than twenty patients have been identified. We report a retrospective series of 18 patients identified as PCL. AIM: To study the clinical features and outcome of patients with PCL. MATERIALS AND METHODS: A retrospective study was conducted from the year 2006 to 2015 wherein all the patients diagnosed with PCL were identified. Complete clinical and treatment details and outcome were obtained from the records. RESULTS: There were total 18 cases of PCL (3.7% of cases with multiple myeloma) diagnosed between the year 2006 and 2015. 16 cases (84%) were primary PCL, and two cases were secondary PCL. Twelve patients were males and six were females. The median age was 56.5 years. All patients had aggressive clinical course and median overall survival even with immunomodulatory agents was only 3 months. CONCLUSION: PCL is a very aggressive disease, and no prospective trials have been conducted. Patients with PCL require induction with immunomodulators, proteasome inhibitors, and further trials are needed to evaluate the role of autologous stem cell transplant in this disease.


Keywords: Plasma cell leukemia, poor prognosis, primary, secondary


How to cite this article:
Babu K G, Jacob LA, Agarwal A, Lakshmaiah K, Lokanatha D, Babu MS, Rajeev L, Lokesh K, Rudresha A. Plasma cell leukemia: Single institution experience. Indian J Cancer 2016;53:619-20

How to cite this URL:
Babu K G, Jacob LA, Agarwal A, Lakshmaiah K, Lokanatha D, Babu MS, Rajeev L, Lokesh K, Rudresha A. Plasma cell leukemia: Single institution experience. Indian J Cancer [serial online] 2016 [cited 2019 Aug 23];53:619-20. Available from: http://www.indianjcancer.com/text.asp?2016/53/4/619/204775



 » Introduction Top


The first case of plasma cell leukemia (PCL) was recognized by Gluzinski and Reichentein.[1] It is the most aggressive among the monoclonal gammopathies. It is diagnosed by the presence of more than 20% plasma cells in the peripheral blood or an absolute plasma cell count of >2000/mm 3.[2],[3] Studies indicate that the incidence of PCL is around 2%–4% of all cases of multiple myeloma.[4],[5],[6],[7] PCL is classified as primary (de novo) and secondary (leukemic transformation of multiple myeloma) and data suggest that primary PCL constitute 60%–70% of total PCL cases.[8],[9] Because of the relatively low incidence and prevalence of this entity, most clinical data come from isolated case reports and small retrospective studies.[9],[10] No prospective series have been published, and only seven reports including more than twenty patients have been identified.[3],[4],[5],[6],[7],[11],[12],[13] We report a retrospective series of 18 patients identified as PCL.

Aim

This study aims to study the clinical features and outcome of patients with PCL.


 » Materials and Methods Top


A retrospective study was conducted from the year 2006 to 2015 wherein all the patients diagnosed with PCL were identified. Complete clinical details, treatment details, and outcome of treatment given were obtained from the records. Immunophenotyping reports, protein electrophoresis, and radiographs were obtained. The panel included CD2, CD3, CD5, CD10, CD11c, CD19, CD23, CD25, SIgM, kappa, lambda, CD38, CD45, CD56, CD103, CD117, and CD138.


 » Results Top


There were total 18 cases of PCL (3.7% of cases with multiple myeloma) diagnosed between the year 2006 and 2015. Sixteen cases (89%) were primary PCL, and two cases were secondary PCL. Twelve patients were males and six were females. The median age was 56.5 years (33–69 years). The most common clinical presentation was easy fatigability. Nine patients had splenomegaly, and three had hepatomegaly. All the patients had Hb <10 g%. Total leukocyte count was between 3000/mm 3 and 38,000/mm 3. Ten patients (56%) had elevated creatinine levels. Fifteen (85%) patients had lytic lesions in the bone and four patients among them had elevated serum calcium levels. Ten (56%) patients had IgG kappa type monoclonal gammopathy on serum immunofixation and electrophoresis. One patient had IgM, and three patients had IgA type of monoclonal gammopathy. Four patients had light chain disease. All the patients had reversal of albumin/globulin ratio. Median plasma cells in the bone marrow aspirate were 40%.

The patients with secondary PCL were relapsed (within 6 months) multiple myeloma postautologous stem cell transplant. Both the patients with secondary PCL received bortezomib, cyclophosphamide and dexamethasone induction. They never achieved complete remission and died within 5 months of relapse due to sepsis. All the patients with primary PCL also had an aggressive clinical course. Four patients presented with features of sepsis and three among them died within 1 week of diagnosis. The fourth patient refused treatment and was discharged against advice. Twelve patients received thalidomide (T) and dexamethasone (D) induction. Eight of these twelve patients who received T + D induction had a partial response after 2 months.


 » Discussion Top


We report a retrospective study conducted at our institution from 2006 to 2015 wherein we identified 18 cases of PCL and studied their clinical features and outcome of treatment. The median age at diagnosis was 56.5 years which is comparable to the other published reports from India. All the patients had a very aggressive clinical course (particularly patients with poor performance status) even when treated with immunomodulatory agents. Proteasome inhibitor Bortezomib could be given to only two patients who died within 5 months of diagnosis. Median overall survival for the patients in our study was 3 months. An unusual finding in our study was an increased number of patients with lytic bone lesions. In seven series, historically median survival, without novel therapies, has ranged from 6.8 to 12.6 months.[3],[4],[5],[6],[7],[11],[12],[13] The best survival data, incorporating hematopoietic stem cell transplantation, reported a median survival longer than 3 years.[14] The discouraging survival results in primary PCL are due to two facts: (1) Its aggressive presentation with severe complications leading to early death within the first months from diagnosis, and (2) the lack of effective therapy to achieve sustained responses. Early mortality is still of concern and reflects the aggressiveness of the disease. Unfortunately, data from transplantation registries or clinical trials have a systematic bias to exclude patients not fulfilling the entry criteria and/or experiencing early death. Secondary PCL is usually a terminal event with a median OS of only 1 month. [Table 1] shows various published Indian studies and comparison with our study.[15],[16],[17],[18],[19]
Table 1: Comparing our study with the other published Indian studies

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 » Conclusion Top


PCL has a very aggressive clinical course even with novel agents like immunomodulators and proteasome inhibitors. Further randomized trials are needed to evaluate the chemotherapy regimens which can induce long-term remissions.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 » References Top

1.
Gluzinski A, Reichentein M. Myeloma und leucaemia lymphatica plasmocellularis. Wien Klin Wochenschr 1906;19:336.  Back to cited text no. 1
    
2.
Kyle RA, Maldonado JE, Bayrd ED. Plasma cell leukemia. Report on 17 cases. Arch Intern Med 1974;133:813-8.  Back to cited text no. 2
    
3.
Noel P, Kyle RA. Plasma cell leukemia: An evaluation of response to therapy. Am J Med 1987;83:1062-8.  Back to cited text no. 3
    
4.
Dimopoulos MA, Palumbo A, Delasalle KB, Alexanian R. Primary plasma cell leukaemia. Br J Haematol 1994;88:754-9.  Back to cited text no. 4
    
5.
García-Sanz R, Orfão A, González M, Tabernero MD, Bladé J, Moro MJ, et al. Primary plasma cell leukemia: Clinical, immunophenotypic, DNA ploidy, and cytogenetic characteristics. Blood 1999;93:1032-7.  Back to cited text no. 5
    
6.
Ramsingh G, Mehan P, Luo J, Vij R, Morgensztern D. Primary plasma cell leukemia: A Surveillance, Epidemiology, and End Results database analysis between 1973 and 2004. Cancer 2009;115:5734-9.  Back to cited text no. 6
    
7.
Tiedemann RE, Gonzalez-Paz N, Kyle RA, Santana-Davila R, Price-Troska T, Van Wier SA, et al. Genetic aberrations and survival in plasma cell leukemia. Leukemia 2008;22:1044-52.  Back to cited text no. 7
    
8.
International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: A report of the International Myeloma Working Group. Br J Haematol 2003;121:749-57.  Back to cited text no. 8
    
9.
Bladé J, Kyle RA. Nonsecretory myeloma, immunoglobulin D myeloma, and plasma cell leukemia. Hematol Oncol Clin North Am 1999;13:1259-72.  Back to cited text no. 9
    
10.
Costello R, Sainty D, Bouabdallah R, Fermand JP, Delmer A, Diviné M, et al. Primary plasma cell leukaemia: A report of 18 cases. Leuk Res 2001;25:103-7.  Back to cited text no. 10
    
11.
Colovic M, Jankovic G, Suvajdzic N, Milic N, Dordevic V, Jankovic S. Thirty patients with primary plasma cell leukemia: A single center experience. Med Oncol 2008;25:154-60.  Back to cited text no. 11
    
12.
Peijing Q, Yan X, Yafei W, Dehui Z, Zengjun L, Junyuan Q, 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.  Back to cited text no. 12
    
13.
Pagano L, Valentini CG, De Stefano V, Venditti A, Visani G, Petrucci MT, et al. Primary plasma cell leukemia: A retrospective multicenter study of 73 patients. Ann Oncol 2011;22:1628-35.  Back to cited text no. 13
    
14.
Mahindra A, Kalaycio ME, Vela-Ojeda J, Vesole DH, Zhang MJ, Li P, et al. Hematopoietic cell transplantation for primary plasma cell leukemia: Results from the Center for International Blood and Marrow Transplant Research. Leukemia 2012;26:1091-7.  Back to cited text no. 14
    
15.
Majumdar N, Kumar R, Anand M, Kalita D, Ghara N, Chopra A, et al. Plasma cell leukemia – A study of 28 cases from India. Hematology 2009;14:198-203.  Back to cited text no. 15
    
16.
Tembhare PR, Subramanian PG, Sehgal K, Yajamanam B, Kumar A, Gadge V, 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;54:294-8.  Back to cited text no. 16
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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;28:10-4.  Back to cited text no. 17
    
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;28:170-4.  Back to cited text no. 18
    
19.
Rajeswari G, Paul TR, Uppin MS, Uppin SG, Rao DR, Raju D DS, et al. Plasma cell leukemia: A case series from South India with emphasis on rarer variants. Indian J Med Paediatr Oncol 2014;35:211-4.  Back to cited text no. 19
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