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| CASE REPORT |
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| Year : 2015 | Volume
: 6
| Issue : 4 | Page : 168-171 |
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Hairy-plasma cell leukemia
Nahlah Alghasham1, Wedian Rawas2, Tarek Owaidah3, Amr Hanbali4
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 Publication | 16-Dec-2015 |
Correspondence Address:
Amr Hanbali
Department of Hematology, King Faisal Specialist Hospital and Research Center, Riyadh
Saudi Arabia
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1658-5127.171992
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 |
| Introduction | |  |
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 | | |
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 | | |
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.
Financial Support and Sponsorship
Nil.
Conflicts of Interest
There are no conflicts of interest
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[Figure 1], [Figure 2], [Figure 3]
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