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 Table of Contents  
Year : 2015  |  Volume : 6  |  Issue : 4  |  Page : 168-171

Hairy-plasma cell leukemia

1 Department of Pathology, College of Medicine, Qassim University, Qassim, Saudi Arabia
2 Department of Pathology, Hematology Section, King Khalid University Hospital, Riyadh, Saudi Arabia
3 Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
4 Department of Hematology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

Date of Web Publication16-Dec-2015

Correspondence Address:
Amr Hanbali
Department of Hematology, King Faisal Specialist Hospital and Research Center, Riyadh
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1658-5127.171992

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Plasma cell leukemia (PCL) is defined by the presence of more than 2 × 109/L PCs in the peripheral blood. It is a very rare type of leukemia with poor outcome. In this case, we report a PCL case with peripheral blood morphology of hairy cell leukemia. We describe the clinical and pathological presentation of a 44-year-old woman who found to have peripheral abnormal hairy lymphoid looking cells confirmed to be lambda specific plasma leukemic cells by flow cytometry. Eventually, this patient managed with autologous stem cell transplantation after conformation of such rare diagnosis and five cycles of VCD regimen “Bortezomib, Cyclophosphamide, and Dexamethasone” based chemotherapy regimen and showed a good response so far.

Keywords: Hairy cells, hairy cell leukemia, plasma cells, plasma cell leukemia

How to cite this article:
Alghasham N, Rawas W, Owaidah T, Hanbali A. Hairy-plasma cell leukemia. J Appl Hematol 2015;6:168-71

How to cite this URL:
Alghasham N, Rawas W, Owaidah T, Hanbali A. Hairy-plasma cell leukemia. J Appl Hematol [serial online] 2015 [cited 2023 May 29];6:168-71. Available from: https://www.jahjournal.org/text.asp?2015/6/4/168/171992

  Introduction Top

Plasma cell leukemia (PCL) is defined by the presence of more than 2 × 109/L peripheral blood PCs or plasmacytosis accounting for >20% of the differential white cell count.[1] It may either be primary disease where the patients present at the time of diagnosis in leukemic phase or evolve from previously diagnosed multiple myeloma (MM) (secondary).[2],[3] It is a very rare type of leukemia with incidence of 0.9% of patients with acute leukemia.[4],[5],[6]

Diagnosis of PCL vary in it is difficulties; the presence of circulating mature PCs is the typical finding in more than 60% of PCL patients.[7],[8] However, frequently the circulating PCs may express more primitive atypical presentation makes the diagnosis of such cases more difficult and challenging base on morphology without immunophenotyping.[9]

In this case, we report a PCL patient with unique circulating PCs showing a cytoplasmic hairy projection misleading the diagnosis with hairy cell leukemia (HCL). The difficulties in the diagnosis with this rare peripheral blood presentation and very few numbers of similar reported cases in the literature make the case worth written and published.

  Case Report Top

A 44-year-old Saudi woman presented with recurrent bruising, menorrhagia, fever, night sweating, and weight loss. She had history of stroke 15 years ago complicated by seizure disorders that controlled by anticonvulsion prophylaxis and Plavix.

On examination, the patient had splenic enlargement 6 cm below the left costal margin. Complete blood count and differential showed the following: White blood cell, 13.59 × 109 cells/L (reference range [RR], 5–10 × 109/L); red blood cell, 1.85 × 1012 cells/L (RR, 3.90–4.60 × 1012 cells/L); hemoglobin, 58 g/L (RR, 140–180 g/L); and platelets, 51 × 109 cells/L (RR, 150–450 × 109/L). White blood cells differential reveals: Neutrophils 2.17 × 109 cells/L, band 0.41 × 109 cells/L, lymphocytes 1.09 × 109, monocytes 0.27 × 109, basophils 0.14 × 109, and atypical lymphoid looking cells 8.83 × 109 (65%).

Peripheral blood smear confirmed the presence of atypical lymphoid cells in which many appear small lymphoid cells with hyperchromatic nucleus, moderate amount of pale gray cytoplasm, and characteristic microvillous projections (hairs) [Figure 1].
Figure 1: Peripheral blood smear confirmed the presence of leukemic cells with cytoplasmic hairy or villus projection (May–Grünwald–Giemsa stain, ×50)

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Bone marrow (BM) examination reveals a hypercellular marrow with marked increase in the PCs number (>95%) [Figure 2].
Figure 2: Bone marrow aspirate appeared markedly infiltrated with plasma cells in which some appeared small mature and one multinucleated plasma cell (Wright–Giemsa stain, ×100)

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Immunophenotyping using flow cytometry shows that 44% of the gated events were positive for CD38, CD138, and cytoplasmic lambda and negative for all B-cell markers [Figure 3].
Figure 3: Dot plot of flow cytometry analysis for bone marrow aspirate shows positivity for CD38, CD138, and cytoplasmic lambda

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Conventional cytogenetic study for her using BM aspirates revealed normal karyotype (46, XX), but fluorescent in situ hybridization was positive for monosomy 13 detected in 87% of nuclei.

Investigation for end organ failure shows the following: Calcium of 2.56 mmol/L (RR, 2.1–2.55 mmol/L), urea of 2.4 mmol/L (RR, 2.5–7.5 mmol/L), creatinine of 58 mol/L (RR, 46–115 l mol/L), lactate dehydrogenase of 162 U/L (RR, 135–225 U/L); and β2-microglobulin of 15.6 mg/L (RR, 0.7–1.8 mg/L).

Serum protein electrophoresis and immunofixation revealed a monoclonal gammopathy (IgG-lambda) of 45, 87 g/L. Skeletal survey reveals lytic lesions of the distal left femur and quite subtle lytic lesions in sacral ala on both sides.

The patient was diagnosed with PCL and started on cycle 1 bortezomib, thalidomide, dexamethasone, cisplatin, doxorubicin, cyclophosphamide, and etoposide (VTD-PACE). The patient was discharged on thalidomide 100 mg, Plavix (dose), and acyclovir 200 mg.

One month later, and prior to starting the second cycle of VTD-PACE, the patient was found to have facial palsy with inability to walk because of bilateral lower extremities weakness. Chemotherapy was put on hold and magnetic resonance imaging of brain and spine was done and showed caudal equine enhancement. She was diagnosed with Guillain–Barré syndrome, which treated with intravenous immunoglobulin for 5 days. Chemotherapy was switched to bortezomib, cyclophosphamide, and dexamethasone (VCD) regimen with dose reduction of subcutaneous bortezomib to 1 mg/m 2 once a week. The patient's neurologic manifestations improved, and the patient tolerated five cycles of VCD and she achieved very good partial remission with drop of IgG lambda to 4.0 g/L. Her stem cells were mobilized using cyclophosphamide and granulocyte-colony stimulating factor, and she underwent autologous stem cell transplantation (auto-SCT) using melphalan 200 mg/m 2 as a conditioning regimen. One month after transplant, IgG lambda continued to be at 4.0 g/L level. The patient received two cycles of lenalidomide and dexamethasone consolidation and her IgG lambda disappeared. She was started on maintenance lenalidomide thereafter.

  Discussion Top

Presence of more than 20% circulating clonal PCs is the rule for diagnosis of PCL.[1],[2],[10] The incidence of PCL ranges between 2% and 4% of patients with MM.[4]

The circulating tumor cells may resemble normal mature PCs with basophilic cytoplasm, prominent Golgi zone, and eccentric nuclei.[11] In some cases, many lymphoplasmacytoid lymphocytes where in the picture with only few characteristic PCs or the patient may host mainly primitive cells with a higher nuclear-cytoplasmic ratio, open chromatin, prominent nucleoli, and a less prominent Golgi zone (plasmablasts).[9] The morphological appearance of those circulating PCs could be misleading and flow cytometry is important to demonstrate clonality of the PCs. For what, immunophenotyping is the only test to prove the cell of origin for those cells and rule out others as lymphoproliferative diseases, including low-grade B-cell or lymphoplasmacytic lymphoma.[4] Typically, leukemic PCs will be restricted to one of the cytoplasmic light chain (kappa or lambda) and positive for the surface CD38, CD138 proteins but were negative for B-cell markers such as CD19 and CD79a.[1],[10]

In our case, the clue to the diagnosis of HCL is the splenomegaly with hairy looking cells in the peripheral blood. However, hairy or villus PCs are well-described in the literature. Four cases reported with hairy or villus cytoplasmic projection in the leukemic PCs.[12],[13],[14],[15] All the three cases together with our case show a phenotype of PCL including positivity for CD38, CD138, lambda, and deficient for HCL markers (CD19, CD25, CD23, and CD103). The flow cytometry of the BM in our case ruled out HCL and confirm the diagnosis of PCL. Further investigation for end organ failure shows only anemia and lytic lesion, but renal profile and calcium level were within normal.

Incidence of monosomy 13 is 70% in patients with the previous diagnosis of monoclonal gammopathy of undetermined significance history.[16] PCL also had a higher incidence of monosomy 13 when compared with MM.[4],[16],[17] It was concluded that chromosome 13 deletions might be involved in initiation of a subset of PCL.[18]

Our patient shows a good response for bortezomib base therapy (VCD) followed by auto-SCT. Chan et al. confirm the good response for bortezomib base therapy in his reported case.[14] This case highlighted the importance of including PCL in the differential diagnosis of HCL and combination of morphology and immunophenotyping in the diagnosis.

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Conflicts of Interest

There are no conflicts of interest

  References Top

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. 1
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. 2
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. 3
Fernández de Larrea C, Kyle RA, Durie BG, Ludwig H, Usmani S, Vesole DH, et al. Plasma cell leukemia: Consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia 2013;27:780-91.  Back to cited text no. 4
Eddou H, Mahtat el M, Zahid H, Maaroufi HE, Jennane S, Messaoudi N, et al. Plasma cell leukemia: Three case-reports and review of literature. Ann Biol Clin (Paris) 2013;71:698-702.  Back to cited text no. 5
Alghasham N, Alnounou R, Alzahrani H, Alsharif F. Plasma cell leukemia: Clinicopathologic, immunophenotypic and cytogenetic characteristics of 4 cases. Hematol Oncol Stem Cell Ther 2015;8:71-7.  Back to cited text no. 6
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
Greipp PR, Kyle RA. Clinical, morphological, and cell kinetic differences among multiple myeloma, monoclonal gammopathy of undetermined significance, and smoldering multiple myeloma. Blood 1983;62:166-71.  Back to cited text no. 8
Johnson MR, Del Carpio-Jayo D, Lin P, Giralt S, Anderlini P, Champlin RE, 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;10:263-8.  Back to cited text no. 9
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. 10
Swerdlow SH, Campo E, Harrie NL, Jaffe ES, Pilers SA, Stein H, Thiele J, Vardiman JW. WHO Classification of tumors of hematopoietic and lymphoid tissues. 4th edition. Lyon: IARC Press; 2008.  Back to cited text no. 11
Tanioka F, Tamashima S, Shimizu S, Kobayashi H, Kobayashi Y, Sugimura H. A case of primary plasma cell leukemia with hairy-cell morphology and lambda-type Bence-Jones protein. Immunohistochemical and molecular analysis. Jpn J Clin Oncol 2003;33:232-7.  Back to cited text no. 12
Kumar TN, Krishnamani K, Gandhi LV, Sadashivudu G, Raghunadharao D. Plasma cell leukaemia masquerading as hairy cell leukaemia: A case report. Indian J Hematol Blood Transfus 2014;30 Suppl 1:33-5.  Back to cited text no. 13
Chan SM, George T, Cherry AM, Medeiros BC. Complete remission of primary plasma cell leukemia with bortezomib, doxorubicin, and dexamethasone: A case report. Cases J 2009;2:121.  Back to cited text no. 14
Loo SY, Bhagavan NV, Scottolini AG. Double IgA bands in serum from a patient with lymphoplasmacytoid leukemia with hairy-cell morphology. Clin Chem 1987;33:2317-9.  Back to cited text no. 15
Avet-Loiseau H, Daviet A, Brigaudeau C, Callet-Bauchu E, Terré C, Lafage-Pochitaloff M, et al. Cytogenetic, interphase, and multicolor fluorescence in situ hybridization analyses in primary plasma cell leukemia: A study of 40 patients at diagnosis, on behalf of the Intergroupe Francophone du Myélome and the Groupe Français de Cytogénétique Hématologique. Blood 2001;97:822-5.  Back to cited text no. 16
Chiecchio L, Dagrada GP, White HE, Towsend MR, Protheroe RK, Cheung KL, et al. Frequent upregulation of MYC in plasma cell leukemia. Genes Chromosomes Cancer 2009;48:624-36.  Back to cited text no. 17
Xu W, Li JY, Fan L, Chen LJ, Qiu HR, Wang R, et al. Detection of chromosome 13 deletion in plasma cell leukemia by dual-color fluorescence in situ hybridization. Zhongguo Shi Yan Xue Ye Xue Za Zhi 2008;16:1261-4.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3]

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