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 Table of Contents  
Year : 2016  |  Volume : 7  |  Issue : 3  |  Page : 95-101

Clinical profile of plasma cell leukemia at Tertiary Care Hospital in Kashmir, India

1 Department of Medical Oncology, Sher I Kashmir Institute of Medical Sciences, Soura, India
2 Department of Clinical Hematology, Sher I Kashmir Institute of Medical Sciences, Soura, India
3 Department of Prosthodonsia, Govt. Dental College, Srinagar, Jammu and Kashmir, India

Date of Web Publication26-Oct-2016

Correspondence Address:
Javvid Muzamil
Department of Medical Oncology, Married Hostel, Room Number F16, Sher I Kashmir Institute of Medical Sciences, Soura, Srinagar - 190 011, Jammu and Kashmir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1658-5127.192988

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Introduction: Plasma cell dyscrasias represent 1.4–2% of all malignancies and among hematologic malignancies; it constitutes 10% of the tumors. Plasma cell dyscrasias are composed of multiple myeloma (MM), and primary and secondary plasma cell leukemia.
Materials and Methods: Primary plasma cell leukemia (pPCL) is a rare and aggressive disease, representing 1–4% of plasma cell dyscrasias.
Results: The prognosis of this is very poor with median survival of 8–11 months in different reported series.
Discussion: We are reporting a study from our hospital over a period of 8 years and 9 months, in which pPCL was found in 1.8% of MM patients, with slight male predominance and earlier age than MM, and all had high disease burden with high lactate dehydrogenase (LDH), β2-microglobulin, and plasmacytosis.
Conclusion: This disease had very aggressive behavior, especially with light chain lambda disease, and all patients succumbed within 8 months of treatment.

Keywords: Lactate dehydrogenase (LDH), multiple myeloma (MM), per iodic acid schiff (P.A.S.), peripheral blood film (PBF), primary plasma cell leukemia (pPCL), secondary plasma cell leukemia (sPCL), Sudan black stain (S.B.)

How to cite this article:
Muzamil J, Aziz SA, Bhat GM, Lone AR, Bhat S, Nabi F. Clinical profile of plasma cell leukemia at Tertiary Care Hospital in Kashmir, India. J Appl Hematol 2016;7:95-101

How to cite this URL:
Muzamil J, Aziz SA, Bhat GM, Lone AR, Bhat S, Nabi F. Clinical profile of plasma cell leukemia at Tertiary Care Hospital in Kashmir, India. J Appl Hematol [serial online] 2016 [cited 2023 May 29];7:95-101. Available from: https://www.jahjournal.org/text.asp?2016/7/3/95/192988

  Introduction Top

Primary plasma cell leukemia (pPCL) is a rare and aggressive disease with a prevalence of only 1–4% of all plasma cell malignancies.[1],[2],[3] It is defined by the presence of >2 × 109/l peripheral blood plasma cells or plasmacytosis accounting for >20% of the white cell count.[1] The prognosis of pPCL is very poor with a median overall survival (OS) of 8–11 months.[1],[4] However, the outcome of pPCL has improved by the introduction of autologous stem cell transplantation as well as novel agents like bortezomib.[1]

Compared to multiple myeloma (MM), the presenting signs and symptoms of pPCL have a more rapid onset with higher tendency of hyper-metabolic symptoms (weight loss, fever, sweating, fatigue, and weakness), extra-medullary manifestations, hypercalcemia, renal involvement, bone marrow failure, and higher β2-microglobulin but rarely osteolytic bone lesions.[7] Furthermore, the presence of poor-risk cytogenetic alterations known from MM is markedly higher.[1],[4],[8] PCL is classified as primary when it presents “de novo” in patients with no evidence of previous MM and as secondary when it is observed as a leukemic transformation of relapsed or refractory disease in patients with previously recognized MM.[9] 60–70% of PCL are primary, and the remaining 30–40% are secondary.[10] More recent data suggest that there is an increasing incidence of secondary PCL, now accounting for about 50% of the cases.[4] We are reporting our experience about primary plasma cell leukemia for almost 10-year period and found different biology in our patients and very poor prognosis.

Clinical study: It is discussed under following headings.

  Materials and Methods Top

A study was conducted at our hospital with hemto-oncological setup for the past 28 years. A cancer registry is maintained for the past 15 years. Clinical survey was conducted on plasma cell leukemia for the last 8 years beginning from 2007 till September 2015. The first patient was enrolled in January 2007 and the last patient was enrolled in December 2014. All patients of MM and de-novo plasma cell leukemia were enrolled in the study. Patient characteristics were noted down and all patients underwent complete hemogram with peripheral blood film (PBF) examination. If PBF was abnormal with presence of plasma cells with either 2000 cells/μl or 20% of total cell count, diagnosis of PCL was established. Rest of the patients of MM were excluded from the study with normal PBF. These patients further underwent investigations like complete liver function and kidney function tests, lactate dehydrogenase (LDH), uric acid, acid base with electrolytes, β2-microglobulin, skeletal survey, serum and urinary electrophoresis with immunofixiation, light chain assay in urine and serum, bone marrow aspiration with biopsy, immunophenotyping, and cytogenetic. Patients were treated with borteozomib-based chemotherapy, and clinical outcome and OS were noted down.

Study and Observation (Results)

In this study, a total of 441 patients with plasma cell dyscrasias were enrolled, out of which 433 patients had MM and eight patients had pPCL. The median age of patients with MM observed was 50 years, with the youngest patient being 35 years and the oldest being 70 years. The median age of patients with pPCL was 53 years, with the youngest patient being 45 years and the oldest being 60 years. The male to female ratio in MM was 4:1 and in pPCL it was 1.7:1. So the percentage of pPCL was 1.89% among all plasma cell dyscrasias. Further study was concentrated on patients with pPCL.

Fifty percent of patients were hypertensive on treatment and 25% were diabetic on oral hypoglycemic agents. There was no other co-morbidity observed in this series. These patients presented with multiple symptoms and signs, which are outlined in [Table 1]. The most common symptom reported was fever followed by bone pain, which are in contrary to MM and the most common sign observed was pallor followed by hepato-splenomegaly and lymphadenopathy, which are again rarely seen in MM.
Table 1: Symptomatology of pPCL

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All patients were subjected to laboratory tests in the form of complete hemogram, full chemistry, acid base, and electrolytes, as depicted in [Table 2]. On peripheral blood examination, all patients were anemic with median HB of 9.2 g/dl, had lower total leucocyte count (TLC) with median of 4.15 × 103/μl, and had very low platelet count with median of 20 × 103/μl. On peripheral blood examination, different degree of plasmacytosis was observed in blood ranging from 22 to 38% and many were having plasmablastic appearance [Figure 1], with median plasma cell percentage of 28% and median peripheral plasma cell count of 1162 per μl. Skeletal survey was having multiple lytic lesions [Figure 2] in every patient, with atypical finding in one patient having splenic hypo-echoic lesions [Figure 3], finding confirmed by aspiration having plasma cell infiltration. On serum chemistry examination, they all had normal kidney and liver functions with lower serum proteins and albumin, and raised LDH and gamma globulins [Table 2].
Table 2: Laboratory investigation of pPCL patients:

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Figure 1: PBF showing plasma cells with plasma blasts with prominent nucleolus

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Figure 2: X-rays showing lytic lesions in both skull and femur

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Figure 3: CECT abdomen showing hypoechoic lesions in spleen

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Then the patients were subjected to MM profile, which is tabulated as [Table 3]. All patients had very high β2-microglobulin with median value of 5.8 mg/l and positive serum M spike with undetectable M spike in urine on protein electrophoresis. But urine immunofixation was positive for M spike in λ region. On immunoglobulin assay, all immunoglobulins’ like Ig G, Ig A, and Ig M were markedly suppressed with very high levels of lambda (λ) free light chains in serum. The median value of lambda (λ) free light chains in serum was 3250 mg/l. All our patients had significant light chain disease and that too lambda chain.
Table 3: Multiple myeloma profile -

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Serum immune flow-cytometry of peripheral blood revealed the plasma cell leukemia phenotype, which is also depicted in flow diagrams [Figure 4]. It showed high expression and positivity of CD38, CD138, and its co-expression but it was having very low expression and negativity of CD27, CD56, and CD20. Different CD marker positivity intensity is given in [Table 4].
Figure 4: Flow diagram showing plasma cell immunophenotype

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Table 4: Immunoflow cytometery in pPCL-

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Bone marrow aspiration and biopsy revealed plasmacytosis between 40 and 90%, with median plasmacytosis of 70%. These bone marrow aspiration slides revealed high degree of plasmacytosis with all having plasmablastic appearance; rest of the cell lines were markedly suppressed [Figure 5]. Under immunohistochemistry, cells were per iodic acid Schiff (P.A.S.), Sudan black stain (S.B.), and myeloperoxidase negative. On conventional cytogenetic karyotype, no abnormality was noted down. Fluorescence In Situ Hybridization (FISH) was not conducted due to financial constraints.
Figure 5: Bone marrow showing immature plasma cells with plasma blasts

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Finally, based on all these parameters, the patients were diagnosed as primary plasma cell leukemia expressing lambda light chains exclusively with suppressed rest of immunoglobulins’ and the patients were put on weekly intravenous borteozomib 2 mg and dexamethasone 40 mg based treatment along with other supportive treatment in the form of tumor lysis prophylaxis, antivirals, antimicrobials, and blood products. On average, 15 weeks of treatment was received, with longest treatment received being 24 weeks. Our patients were not transplant candidates on the basis of general performance. Interim assessment of disease was done after 8 weeks of treatment with complete hemogram, PBF, and myeloma profile. There was no plasma cell seen on PBF examination and serum light chains were reduced by 80%.

Most of our patients dropped general performance from ECOG PS II to PS IV. They developed renal failure and respiratory tract infection, which were treated with broad-spectrum antibiotics, dialysis support, and other intensive care support. All our patients succumbed to different kinds of infection. The median survival seen was 5 months and the longest survival was 8 months. There was no patient available after 8 months for disease assessment.

So, we conclude that pPCL is a very rare disease in plasma cell dyscrasias contributing to 1.89% of all plasma cell dyscrasias, with slight male predominance and earlier age than MM, and all had high disease burden with high LDH, β2-microglobulin, and plasmacytosis. This disease had very aggressive behavior, especially with light chain lambda disease, and all patients succumbed within 8 months of treatment.

  Discussion Top

pPCL is the most aggressive form of the plasma cell dyscrasias. It is defined by the presence of >2 × 109/l peripheral blood plasma cells or plasmacytosis accounting for >20% of the differential white cell count, and it does not arise from preexisting MM.[3],[9] We also used the same criteria for diagnosis and all our patients had low median TLC, with median plasma cell of 1160/μl (28%). pPCL is rare, with only 1–4% of patients with MM presenting as pPCL.[1],[2],[4] In addition, <1% of patients presenting with extreme leukocytosis (>50 × 109/l) are diagnosed with PCL.[15] In our study as well, the total patients with pPCL were 1.89% among 441plasma cell dyscrasias which corresponds to other international studies. None of our patients has TLC more than 10 × 103/dl.

In PCL, tumor cells accumulate in the bone marrow (BM) but also have an increased capacity to recirculate in blood, with subsequent egression and formation of extra-medullary disease. The dissemination of tumor cells out of the BM is not only related to changes in expression of adhesion molecules and chemokine receptors but also to the presence of several molecular aberrations, which contribute to BM microenvironment-independent tumor growth, inhibition of apoptosis, and escape from immune surveillance. Interestingly, gene expression profiling identifies pPCL as a distinct molecular entity among myeloma samples.[14]

Compared to MM, tumor cells from patients with pPCL and secondary plasma cell leukemia (sPCL) have reduced expression of the adhesion molecules NCAM (neural cell adhesion molecule/CD56) and LFA-1 (leukocyte function-associated antigen-1), which may contribute to the extra-medullary accumulation of tumor cells in PCL.[1] The absence of CD56 or LFA-1 is associated with reduced binding of tumor cells to BM stromal cells.[19],[20] Furthermore, loss of CD56 results in increased production of matrix metalloproteinase-9, which leads to destruction of the basal membrane and extracellular matrix.[21] In our study, all patients were negative for CD56 and CD27.

Patients with pPCL have a younger age at presentation compared to patients with MM or sPCL.[4],[11],[12],[22],[23] However, their performance status at diagnosis is usually worse,[1] which may be related to the more advanced stage of disease (Durie-Salmon stage III: ∼80–96%; International Staging System stage III: ∼63–80%).[1],[6],[7],[11] Extra-medullary involvement, such as hepatomegaly, splenomegaly, lymphadenopathy, leptomeningeal infiltration, or extra-medullary plasmacytomas, is more frequent in pPCL,[1],[2],[4],[7],[12],[13],[14],[23],[25],[26],[27] with extensive bone disease being more common in patients with MM.[1],[4],[25] All our patients were younger than patients with MM, with median age being 53 years. Performance of all our patients was also worse at presentation. However, the stage as per Durie-Salmon staging system was II, but as per ISS, it was III. Extra-medullary involvement was very high, with hepatomegaly in 60%, splenomegaly in 50%, and lymphadenopathy in 45% and none had central nervous system (CNS) involvement. Bone involvement was seen in less than half of the patients.

There are various parameters which detect tumor burden in PCL, for example plasma cell percentage, LDH, and β2-microglobulin. The median percentage of BM plasma cells is significantly higher in pPCL than in MM[1],[4],[26] as was seen in our patients as well. We documented bone marrow plasmacytosis between 40 and 90%, with median plasmacytosis of 70% and mostly plasmablastic appearance. In addition, renal failure is more common in pPCL, which can be partly explained by the higher incidence of light-chain disease.[1],[4],[14],[23] Furthermore, hypercalcemia, anemia, thrombocytopenia, elevated plasma cell labeling index, increased LDH, and gene expression profile (GEP)-defined high-risk disease are more frequent at presentation in pPCL compared to MM.[1],[2],[4],[6],[7],[14],[25],[27] In our series, none of our patients had renal failure at the beginning but it developed in all of them over the course of the disease. All our patients had lambda light chain disease only, with high degree of thrombocytopenia, moderate anemia and high LDH.

Peripheral blood examination in pPCL shows circulating tumor cells and typically a leuko-erythroblastic blood picture in up to 67% of patients.[4],[25] BM biopsy typically demonstrates extensive BM involvement, disrupting normal hematopoiesis. In some cases, tumor resembles normal plasma cells, whereas in others, lympho-plasmacytoid or immature plasma cells predominate.[28],[29],[30] Our series also highlighted the presence of plasma blastic or immature plasma cells both in blood and bone marrow.

The most striking immune-phenotypic feature is increased expression of CD20 and CD23, and down-regulation of CD56 may be related to the high incidence of t(11;14) in pPCL.[1] Tumor cells are positive for CD38 and CD138 in both PCL and MM.[1],[16],[28] We also demonstrated similar results in our series with high expression of CD38/138 and loss of CD56.

There is a paucity of literature on the treatment of pPCL, and no randomized trials have been reported exclusively for patients with pPCL. The prognosis of pPCL after conventional chemotherapy without novel agents is poor, with median OS of ∼7 months.[1],[4],[6],[12],[23],[30],[34] The introduction of immune-modulatory drugs and proteasome inhibitors has significantly improved the survival of patients with MM.[35],[36] Increasing evidence suggests that these agents also improve outcome of pPCL, but the benefit may be less pronounced compared to classic MM. A retrospective analysis performed by the Inter-groupe Francophone du Myélome showed that patients with pPCL treated with novel agents had a survival of 15 months compared to 8 months for patients who did not receive novel agents as part of their treatment.[37] Several case reports and small case series suggest that bortezomib, alone or in combination with other agents, is effective in newly diagnosed pPCL and may also be active in refractory pPCL or sPCL.[5],[34] In pPCL, the efficacy of combinations of novel agents, such as lenalidomide, bortezomib, and dexamethasone (RVD),[44] bortezomib, thalidomide, and dexamethasone (VTD),[5],[34],[37] or melphalan, prednisone, bortezomib, and thalidomide (VMPT),[5] appears very promising. In our series, we treated all patients with borteozomib and dexamethasone, and all were transplant-ineligible based on their performance status. For initial 8 weeks of therapy, all our patients responded evidence by interim assessment, but subsequently all progressed fast with deterioration in renal functions and superadded infections. The median survival we documented was 5 months and the longest survival of 8 months.

There were some peculiarities in our patients. All were young, and had moderate anemia with critical thrombocytopenia. All had high LDH and β2-microglobulin with high degree of plasmacytosis in bone marrow and blood. All our patients had lambda light chain disease with suppressed other immunoglobulins’. Response to treatment was very short-lived and the median survival was lower than evidenced by modern treatment strategies, partly due to different genetics in our patients.

  Conclusion Top

Primary plasma cell leukemia is a very aggressive disease and should be picked up early and treated aggressively. The authors have recommended that at least 20% of plasma cells be seen in PBF, so as to diagnose this condition. As aggressiveness of this disease is concerned, these criteria need to be revised and less threshold of monoclonal plasma cells percentage be seen in PBF for its diagnosis. Further work needs to be done for classifying this leukemia, refining its staging and refining its treatment.

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Conflicts of interest

There are no conflicts of interest.

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2], [Table 3], [Table 4]


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