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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 18  |  Issue : 1  |  Page : 37-40

Massive splenomegaly as the presenting feature of multiple myeloma in an elderly man


1 Department of General Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
2 Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
3 Department of Geriatric Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Submission20-Mar-2022
Date of Decision23-Mar-2022
Date of Acceptance23-Mar-2022
Date of Web Publication21-Apr-2022

Correspondence Address:
Dr. Sankha Shubhra Chakrabarti
Department of Geriatric Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiag.jiag_15_22

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  Abstract 


A 70-year-old man presented with generalized weakness, easy fatigability, and early satiety of 2-month duration. On examination, he had severe pallor and massive splenomegaly. Hematological investigations revealed bicytopenia with hypergammaglobulinemia and acute kidney injury. Bone marrow aspiration cytology was suggestive of plasma cell dyscrasia. Monoclonal protein peak (due to heavy chain of IgG type) was found on serum protein electrophoresis, and lambda light chains and IgG heavy chains were elevated on immunofixation. The patient was diagnosed as a case of multiple myeloma and was started on bortezomib–lenalidomide–dexamethasone regimen. After 7 months of chemotherapy, his spleen had regressed, and the patient had become asymptomatic. Presentation with massive splenomegaly is usually a feature of Waldenstrom's macroglobulinemia. However, rarely multiple myeloma may have extramedullary manifestations such as splenomegaly as the primary presenting feature.

Keywords: Chemotherapy, extramedullary, plasma cell infiltration, Waldenstrom's


How to cite this article:
Mishra V, Pandey A, Goyal D, Chakrabarti SS. Massive splenomegaly as the presenting feature of multiple myeloma in an elderly man. J Indian Acad Geriatr 2022;18:37-40

How to cite this URL:
Mishra V, Pandey A, Goyal D, Chakrabarti SS. Massive splenomegaly as the presenting feature of multiple myeloma in an elderly man. J Indian Acad Geriatr [serial online] 2022 [cited 2022 May 19];18:37-40. Available from: http://www.jiag.com/text.asp?2022/18/1/37/343681




  Introduction Top


Multiple myeloma usually affects persons above 50 years and accounts for about 10% of all hematological malignancies. It commonly presents with anemia, bony lytic lesions, and renal involvement. Although extramedullary, extraosseous disease is well known in the course of multiple myeloma, initial presentation with splenomegaly or extramedullary disease (EMD) is rare. In contrast, splenomegaly is present in 15% of cases of Waldenstrom's macroglobulinemia at presentation.[1] In myeloma patients, an extramedullary presentation may represent poor biology or disease with a worse prognosis. Here, we report the case of a 70-year-old man who presented with splenomegaly, generalized weakness, and easy fatigability.


  Case Report Top


A 70-year-old man with a history of alcohol use presented with generalized weakness and easy fatigability of 2-month duration. He had received four units of packed red blood cell transfusion before presenting to our outpatient department. He also complained of heaviness in the abdomen with a dragging sensation and had early satiety. On examination, there was severe pallor, and the spleen was palpable 8 cm below the left costal margin. Other findings were inconspicuous.

Biochemical and hematological investigations are detailed in [Table 1]. The general blood picture revealed 15% plasma cells with few microcytes and a corrected reticulocyte count of 0.3%. No hemoparasites were observed. Bone marrow aspiration cytology revealed a hypocellular picture, with 20% plasma cells. A plain radiograph of the skull showed multiple lytic lesions, and a color Doppler ultrasonography of the abdomen revealed a spleen of size 19.6 cm along its long axis, with a normal portal vein diameter. Considering plasma cell dyscrasia as a probable diagnosis, serum electrophoresis, and immunofixation were performed. M-peak was observed with an IgG concentration of 5383 mg/dL, β2-microglobulin concentration of 7.28 mg/L, and a serum kappa/lambda ratio of 0.010 (κ free light chains-16.0 mg/L; λ free light chains-1580 mg/L).
Table 1: Hematological and biochemical investigations of the patient

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We made a provisional diagnosis of multiple myeloma with extramedullary (splenic) involvement. There may be several causes for splenomegaly in multiple myelomas, such as plasmacytoma, splenic infiltration by plasma cells, amyloidosis, splenic hematoma, or an underlying unrelated tropical splenomegaly. Waldenstrom's macroglobulinemia is a plasma cell disorder that more commonly presents with splenomegaly as an initial feature. In our patient, the serum immunofixation electrophoresis picture ruled out Waldenstrom's macroglobulinemia. Ultrasound of the abdomen did not show any well-demarcated encased mass in the spleen, which made plasmacytoma unlikely. Splenomegaly was nontender, and the coagulation profile was normal in our patient, making a splenic hematoma less likely. Splenic amyloidosis and direct infiltration by plasma cells were close differentials, but our patient responded well to the bortezomib-based regimen, making direct splenic infiltration more likely.

The patient was started on bortezomib (2 mg administered subcutaneously twice weekly for the first 4 cycles and then weekly for the next 5–7 cycles), lenalidomide (10 mg once a day orally as per renal modification), and dexamethasone (40 mg orally with every dose of bortezomib). He was also started on aspirin 75 mg/day prophylaxis to prevent deep vein thrombosis and acyclovir 200 mg orally once daily as herpes prophylaxis. Once the patient was in remission, the maintenance phase comprised lenalidomide monotherapy.

The patient responded well to bortezomib-based chemotherapy. His follow-up hematological and biochemical investigations are shown in [Table 1]. After 7 months of chemotherapy, bone marrow aspiration revealed 3% plasma cells, and the spleen regressed to 15.6 cm size on Doppler ultrasound imaging of the abdomen. No M-peak could be visualized on serum protein electrophoresis after 10 months of chemotherapy. The patient was shifted to lenalidomide-based maintenance therapy and was doing well after nearly 1.5 years of chemotherapy.


  Discussion Top


In multiple myeloma, clonal proliferation of plasma cells is usually confined to the bone marrow; however, the proliferation of plasma cells outside the bone marrow may happen uncommonly. Relapsed and refractory myeloma have a higher incidence of EMD.[2],[3],[4],[5]

The EMD can be due to bone-related plasmacytoma or distant infiltration of tumor cells. In bone-related plasmacytoma myeloma, cells infiltrate from the bone to the surrounding soft tissues.[6],[7] The common sites of EMD in decreasing frequency include the kidneys (27.3%), skin (23%), lymph nodes (17.3%), central nervous system (10.1%), lungs and respiratory tract (6.5%), gastrointestinal tract and liver (5.8%), pleura and heart (5.0%), and spleen, ovaries, and testicles (5.3%).[5],[8],[9] Splenic EMD is an uncommon feature, more so as the first presenting symptom.

Morphologically, plasma cells in multiple myeloma have immature/plasmablastic appearance in patients with EMD and have mature/plasmacytic appearance in bone-related plasmacytoma. These differences may point toward the different pathogenesis of these two clinical conditions. Fifty percent of patients with myeloma and EMD have a clonal mutation of TP53, RB1, FAK, and RAS genes.[3],[5],[6],[10],[11] It may be postulated that these mutations cause “bone marrow escape” of clonal plasma cells due to decreased cell adhesion.[12]

Although there are no differences in response to therapy in patients of myeloma with EMD, more studies are needed on the extramedullary presentation of multiple myeloma. The EMD may be considered a high-risk disease feature. It should be managed intensively although a good response to therapy may be seen in cases such as the current one. For non-transplant candidates such as our patient, standard of care with a high response rate is triplet therapy with an alkylating agent and proteasome inhibitor (bortezomib plus melphalan) with prednisone or continuous lenalidomide with dexamethasone.[13]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Rahar S, Kumar V, Kishore M, Marwah S, Kumar S, Nigam AS. Waldenstrom's macroglobinemia presenting in a hypertensive patient. Ann Pathol Lab Med 2018;5:C100-3. [doi: 10.21276/APALM.1811].  Back to cited text no. 1
    
2.
Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol 2014;15:e538-48.  Back to cited text no. 2
    
3.
Bladé J, Fernández de Larrea C, Rosiñol L, Cibeira MT, Jiménez R, Powles R. Soft-tissue plasmacytomas in multiple myeloma: Incidence, mechanisms of extramedullary spread, and treatment approach. J Clin Oncol 2011;29:3805-12.  Back to cited text no. 3
    
4.
Touzeau C, Moreau P. How I treat extramedullary myeloma. Blood 2016;127:971-6.  Back to cited text no. 4
    
5.
Morales-Chacón K, Bourlon MT, Martínez-Baños D, Delgado-de-la-Mora J, Bourlon C. Multiple Myeloma With Extramedullary Disease: A Challenging Clinical Dilemma. Oncology 2019;33:149-51.  Back to cited text no. 5
    
6.
Short KD, Rajkumar SV, Larson D, Buadi F, Hayman S, Dispenzieri A, et al. Incidence of extramedullary disease in patients with multiple myeloma in the era of novel therapy, and the activity of pomalidomide on extramedullary myeloma. Leukemia 2011;25:906-8.  Back to cited text no. 6
    
7.
Varettoni M, Corso A, Pica G, Mangiacavalli S, Pascutto C, Lazzarino M. Incidence, presenting features and outcome of extramedullary disease in multiple myeloma: A longitudinal study on 1003 consecutive patients. Ann Oncol 2010;21:325-30.  Back to cited text no. 7
    
8.
Weinstock M, Aljawai Y, Morgan EA, Laubach J, Gannon M, Roccaro AM, et al. Incidence and clinical features of extramedullary multiple myeloma in patients who underwent stem cell transplantation. Br J Haematol 2015;169:851-8.  Back to cited text no. 8
    
9.
Gagelmann N, Eikema DJ, Iacobelli S, Koster L, Nahi H, Stoppa AM, et al. Impact of extramedullary disease in patients with newly diagnosed multiple myeloma undergoing autologous stem cell transplantation: A study from the Chronic Malignancies Working Party of the EBMT. Haematologica 2018;103:890-7.  Back to cited text no. 9
    
10.
Weinstock M, Ghobrial IM. Extramedullary multiple myeloma. Leuk Lymphoma 2013;54:1135-41.  Back to cited text no. 10
    
11.
Rasche L, Bernard C, Topp MS, Kapp M, Duell J, Wesemeier C, et al. Features of extramedullary myeloma relapse: High proliferation, minimal marrow involvement, adverse cytogenetics: A retrospective single-center study of 24 cases. Ann Hematol 2012;91:1031-7.  Back to cited text no. 11
    
12.
Leblay N, Maity R, Hasan F, Neri P. Deregulation of adaptive T cell immunity in multiple myeloma: Insights into mechanisms and therapeutic opportunities. Front Oncol 2020;10:636.  Back to cited text no. 12
    
13.
Mateos MV, Oriol A, Martínez-López J, Teruel AI, Bengoechea E, Palomera L, et al. Outcomes with two different schedules of bortezomib, melphalan, and prednisone (VMP) for previously untreated multiple myeloma: Matched pair analysis using long-term follow-up data from the phase 3 VISTA and PETHEMA/GEM05 trials. Ann Hematol 2016;95:2033-41.  Back to cited text no. 13
    



 
 
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