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Igm Myeloma with Plasma Cell Leukemia: Case Report and Literature Review

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Igm Myeloma with Plasma Cell Leukemia: Case Report and Literature Review Available online at www.annclinlabsci.org Annals of Clinical & Laboratory Science, vol. 47, no. 5, 2017 611 IgM Myeloma with Plasma Cell Leukemia: Case Report and Literature Review Saurabh Chhabra1, Sandeep Jain2, Amanda Fowler3,Valeriy Sedov3, Amarendra K Neppalli3, Cynthia A. Schandl4, and John Lazarchick4 1Division of Hematology/Oncology, Medical College of Wisconsin, Milwaukee, WI, 2Division of Hematology/ Oncology, Mayo Clinic, Rochester, MN, 3Division of Hematology/Oncology, Medical University of South Carolina, Charleston, SC, and 4Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston, SC, USA Abstract. IgM multiple myeloma (MM) is a rare entity representing approximately 0.5% of all MM. It should be distinguished from malignant neoplasms of B cells with plasmacytic differentiation such as Waldenstrom macroglobulinemia (WM) and marginal zone lymphoma with plasmacytic differentiation. Plasma cell leukemia (PCL) is a rare and aggressive variant of MM characterized by the presence of circulat- ing plasma cells. We present a case report of a patient who presented with IgM MM in primary PCL phase with high-risk cytogenetics. To our knowledge, this is the first reported case of IgM MM with primarily leukemic presentation in the era of novel drugs. We demonstrate that it is important to distinguish IgM MM from WM and review the data from clinical trials that was used to devise a treatment strategy for this high-risk patient. This case adds to the understanding of the diagnosis and management of IgM MM in leukemic phase. Key words: IgM multiple myeloma, plasma cell leukemia, high-risk, deletion 17p, deletion TP53. Introduction 13.2 mg/dL, hemoglobin was 12 g/dL, and creatinine was 1.1 mg/dL with an estimated glomerular filtration IgM multiple myeloma (MM) is a rare and poorly rate of 57 mL/min. Serum protein electrophoresis (PEP) characterized disorder estimated to account for ap- with immunofixation (IFE) showing M-protein of IgM proximately 0.5% of all cases of myeloma. IgM kappa at 5.85 g/dL and quantitative IgM of 6537 mg/dL were suggestive of an M-protein producing lymphopro- MM presenting with plasma cell leukemia (PCL) is liferative neoplasm. In addition, peripheral blood flow extremely rare. This report details the clinical and cytometry (FC) revealed 25% plasma cells (PC). Serum laboratory features and course of a patient with free light chain (FLC) ratio was elevated (2100; kappa of IgM MM with PCL with high-risk cytogenetics. 252 mg/dL and lambda of 0.12 mg/dL) and a 24-hour Initial cytogenetic and fluorescence in-situ hybrid- urine PEP with IFE showed monoclonal free kappa at ization (FISH) analyses demonstrated a complex 195 mg. Beta-2 microglobulin was 7.8 mg/L and serum karyotype including deletion of 17p, t(11;14) and albumin was 3.2 g/dL. LDH was normal. No lytic bone deletion of 13q. We believe this to be the first re- lesions were identified on the skeletal survey. ported case of high-risk IgM MM with PCL in the Waldenstrom macroglobulinemia (WM) was a consider- era of novel agents. ation given the IgM and the absence of bone lesions on x-ray and she was given one course of rituximab-benda- mustine. A CT scan of the chest and abdomen (Figure Case Report 1) showed several subtle lucent lesions of the L2 verte- bral body and ill-defined lucencies within the sacrum A 68-year-old Caucasian female presented with a brief and proximal femurs bilaterally, representing regions of history of fatigue and right-sided chest pain. Laboratory osteopenia. There was no evidence of lymphadenopathy. evaluation showed hypercalcemia and IgM monoclonal PET-CT showed a conspicuous hypermetabolic 1.2 cm paraprotein (M-protein). The corrected calcium was left thyroid nodule with SUVmax of 19.3. Increased hy- permetabolic activity in the lytic spine and pelvic lesions Address correspondence to John Lazarchick, MD; Department of Pathology and Laboratory Medicine, Medical University of South was also seen, without any hypermetabolic adenopathy. Carolina, MSC 908, Charleston, SC 29425, USA; phone: 843 792 MRI of the spine showed decreased T1 signal involving 0217; e mail: [email protected] the lateral aspect of the left L2 vertebral body, with no 0091-7370/17/0500-611. © 2017 by the Association of Clinical Scientists, Inc. 612 Annals of Clinical & Laboratory Science, vol. 47, no. 5, 2017 Figure 1. CT scan abdomen/pelvis showing lytic lesions (arrows). post-contrast enhancement, linear T2 hyperintensity Uniform Response criteria [2]) but the response pla- and diffusely decreased marrow signal throughout the teaued after 12 weeks: M-protein decreased from 5.85 to spine reflecting marrow infiltration. Thyroid ultrasound 2.76 g/dL, but the kappa FLC only decreased by 10%. showed a hypodense nodule in the left thyroid lobe Concerned about the impending loss of response with (1.8x1.1x0.5 cm) with increased vascularity. continued RVD, treatment was changed to the combi- nation of carfilzomib (K), cyclophosphamide (Cy) and Bone marrow examination (BM) (Figures 2a&b) dexamethasone (D) q28 days: Cy 300 mg/m2 po days 1, showed kappa-restricted PC involving >90% of marrow 8, 15; d 40 mg po days 1, 8, 15, 22; and K iv over 30 cellularity and hypercellular marrow with minimal he- minutes days 1, 2, 8, 9, 15, 16 (20 mg/m2 on days 1 and matopoiesis. Immunohistochemistry (IHC) (Figure 2c) 2 of cycle 1 and 36 mg/m2 thereafter) [3]. She achieved showed sheets of CD138+ kappa-restricted PC (also by very good partial response (VGPR: Figure 3) after two FC) and only rare CD20+ B cells (polyclonal by FC). cycles of KCyD and proceeded to growth factor-based Congo red stain was negative for amyloid deposition. peripheral blood progenitor cell mobilization and collec- FISH analysis of CD138-separated cells showed tion followed by autologous hematopoietic cell trans- t(11;14), deletion of TP53 and deletion 13 or 13q14. plantation (autoHCT) using high-dose melphalan (200 Karyotype was complex: 40-48,X,-X,t(1;8) mg/m2 iv) conditioning. BM immediately prior to mo- (q32;q24.1),der(3)t(3;11)(q26;q13),del(4)(q28),del(6) bilization showed 60-70% kappa-PC in a 60% cellular (q15q23),del(6)(q13q23),t(11;14)(q13;q32),+der(11) marrow. Chromosomal microarray analysis revealed at t(11;14)-13,add(16)(q21),add(17)(p12),add(17) least three abnormal clones in 60%, 40% and 25% of (p11.2),-18,-22, +mar[cp14]/ 46,XX[6]. MYD88 muta- cells. Abnormalities included gain of 3q, 4q, 5p, 6p, 11q, tion was undetectable by bi-directional Sanger sequenc- and 18q; loss of 2p, 3q, 4q, 8p, 13, 14q, 16q, 17p (in- ing (expected sensitivity 10-15%). These data resulted in cluding TP53), 22, and X; and loss of heterozygosity amendment of the diagnosis to IgM MM with PCL. (LOH) of 14q (Figure 4 & Table 1). Six weeks post- autoHCT, KCyD was resumed as consolidation and For treatment of International Staging System (ISS) VGPR was maintained after 4 cycles. After the 1st cycle stage III IgM MM with pPCL, a triple-drug regimen of of consolidation, the patient underwent a total thyroid- RVD q21days (bortezomib 1.3 mg/m2 sq on days 1, 4, ectomy with central neck dissection and parathyroid au- 8, 11, lenalidomide 25 mg daily for days 1-14 and dexa- totransplantation. Pathology revealed papillary micro- methasone 40 mg po weekly) was started. Her Revised- carcinoma (0.2 cm) with no lymphovascular/perineural ISS stage was III, based on the cytogenetics and ISS stage invasion and negative margins, and no nodal involve- [1]. She had partial response (PR by International ment (pT1N0). After consolidation, a restaging BM IgM myeloma with PCL 613 Figure 2. Bone marrow aspirate shows an extensive plasma cell infiltrate with scattered normal hematopoietic elements (A). Sheet of plasma cells, many with prominent nucleoli, is noted on the biopsy (B). All these cells are CD138 positive on im- munohistochemical staining (C). showed 10-20% involvement by kappa-PC, while SPEP over a 10-year period (1995-2005) [13]. In addi- and FLC confirmed VGPR. Gene-expression profiling tion, CIBMTR reported outcomes of 147 pPCL (GEP) risk score (70-gene Myeloma Prognostic Risk patients (unknown if there were any IgM PCL in Score; MyPRS®) [4] was 63.06 (high risk; subtype CD- the group) receiving autologous (n=97) or alloge- + 1) and 10.42% cells on FC were CD138 . For mainte- neic (n=50) HCT from 1995 to 2006 [14]. PFS nance therapy, K was continued and Cy was substituted and OS at 3 years were 34% and 64% with auto- by pomalidomide (POM). About 7-month post-auto- HCT, FLC but not M-protein, showed progressive dis- HCT and 20% and 39% with alloHCT, respec- ease (PD), indicative of light-chain escape. BM showed tively. The OS after autoHCT was encouraging, MM involving >80% of the cellularity. She then received whereas alloHCT showed no OS benefit due to one cycle of V-DCEP regimen (bortezomib 1.3 mg/m2 high non-relapse mortality (NRM; 41% at 3 years) iv days 1 and 4, and dexamethasone 40 mg po, Cy 400 and significant relapse rate of 39%. In 2009, a mg/m2 iv, etoposide 40 mg/m2 iv and cisplatin 10 mg/ Surveillance, Epidemiology, and End Results m2 iv on days 1-4 [5,6]), and had stable disease. (SEER) study reported outcomes of 291 patients Subsequently, a combination of iv daratumumab with PCL (1973-2004): median OS was 4 months; (DARA) and POM-D was used, leading to VGPR in 5 1-year, 2-year and 5-year OS were 28%, 14%, and weeks; BM showed 10% MM by IHC.
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