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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 11  |  Issue : 1  |  Page : 40-43

Lupus and lymphoma: An intriguing association


1 Department of Medicine, ESI PGIMSR, New Delhi, India
2 Department of Ophthalmology, Dr. B S A Hospital, New Delhi, India

Date of Submission05-Oct-2019
Date of Decision04-Dec-2019
Date of Acceptance13-Dec-2019
Date of Web Publication12-Feb-2020

Correspondence Address:
Dr. Rajesh Chetiwal
Flat No. 63, Munirka Vihar, New Delhi - 110 067
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/INJMS.INJMS_132_19

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  Abstract 


A 21-year-old female patient, diagnosed with systemic lupus erythematosus (SLE) without renal involvement on remission with oral corticosteroids and hydroxychloroquine for the past 6 months. presented again with a history of polyarthralgia, weight loss, high-grade fever, and neck swelling. Histological and radiological studies confirmed non-Hodgkin's lymphoma subtype diffuse large B-cell type. We thus report an infrequent and intriguing association of non-Hodgkin's disease with SLE. The awareness of the association of non-Hodgkin's disease with SLE and its modes of presentation will help in the early diagnosis and management of such patients.

Keywords: Lymphadenopathy, non-Hodgkin's lymphoma, systemic lupus erythematosus


How to cite this article:
Gupta S, Chetiwal R, Gupta P, Rastogi P, Kumar A. Lupus and lymphoma: An intriguing association. Indian J Med Spec 2020;11:40-3

How to cite this URL:
Gupta S, Chetiwal R, Gupta P, Rastogi P, Kumar A. Lupus and lymphoma: An intriguing association. Indian J Med Spec [serial online] 2020 [cited 2020 Sep 23];11:40-3. Available from: http://www.ijms.in/text.asp?2020/11/1/40/278087




  Introduction Top


Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease with variable presentations. In the past few decades, data have accumulated about an increased risk of lymphomas in SLE compared to healthy cohorts, particularly non-Hodgkin's lymphoma (NHL).[1],[2] The reasons for this increased risk are unknown but speculated to be related to the primary disease pathophysiology itself. As there is considerable overlap between the features of SLE and NHL, there can be a great difficulty in diagnosing NHL in SLE patients. In this report, we describe a similar association in a patient in the early stages of her disease course.


  Case Report Top


A 21-year-old woman presented to the outpatient department with complaints of severe joint pains, anemia, and erythematous rash over the cheeks for 6 months. She was diagnosed with SLE according to the American College of Rheumatology criteria, including positive antinuclear antibody titers, fever, oral ulcers, discoid rash, and pancytopenias. Treatment was initiated with oral corticosteroids and hydroxychloroquine, and remission was achieved after 1 month of treatment. On subsequent follow-up post 6 months of treatment, the patient developed recurrent joint pains, weight loss, high-grade fever, and palpable neck mass. Physical examination showed mild pallor with a left indolent, fixed, and elastic cervical adenopathy with hepatosplenomegaly not present on treatment initiation. Other common causes of lymphadenopathy including tuberculosis, sarcoidosis, Epstein–Barr virus (EBV), and HIV infection were ruled out using standard radiological and serological tests. Hematological studies revealed mild anemia and thrombocytopenia with normal liver and kidney function tests.

Computed tomography of the chest and abdomen showed a left basicervical mass expanded to the anterior and superior mediastinum along with enlarged supraclavicular and inguinal lymph nodes and splenomegaly [Figure 1] and [Figure 2]. Lymph node biopsy performed from the left supraclavicular lymph node revealed the classical histology of NHL [Figure 3] with CD3 and CD20 positivity [Figure 4] and [Figure 5], typical of diffuse large B-cell lymphoma (DLBCL). Viral serology (EBV, herpes simplex virus [HSV], and herpes zoster) was negative. The diagnosis of NHL was thus confirmed, and the patient was transferred to the hematology department where she was treated by CHOP (cyclophosphamide, vincristine, prednisolone and doxorubicin) chemotherapy regimen with favorable outcomes.
Figure 1: Axillary and supraclavicular lymph nodes more commonly found in non-Hodgkin's lymphoma

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Figure 2: Abdominal scans depicting hepatosplenomegaly with retroperitoneal lymph nodes

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Figure 3: Large atypical monomorphic cells with round nucleus and eosinophilic cytoplasm

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Figure 4: CD3-positive cells seen on H and E stain

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Figure 5: CD20-positive cells

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  Discussion Top


SLE is a very complex autoimmune disease that presents with any number of different symptoms and can involve multiple organ systems within the body. The relative risk of hematologic malignancy is estimated to be 60% higher in patients with SLE than in the general population, with the reason being unknown. It has been stated that lymphoma is a rare complication of SLE, occurring in <1% of SLE patients.[3] Of all hematologic cases reported in patients with SLE, the most common is NHL, followed by Hodgkin's disease, leukemia, and multiple myeloma.[4] The initial presenting features of SLE and non-Hodgkin's disease are similar, with fever, weight loss, and peripheral lymphadenopathy seen in most cases.[5] Persistent large lymph nodes not responding to conventional therapy in SLE can be evaluated further for other causes of lymphadenopathy such as tuberculosis, sarcoidosis, lymphoma, or viral infections such as EBV, HIV, and HSV and intervened timely.

Little is known about the frequency of NHL subtypes in SLE. This is unfortunate because determining subtype predominance in SLE might be an important step in exploring pathogenic mechanisms for the association of NHL and SLE. A study conducted by Bernatsky et al. examined the demographic factors of patients with SLE who developed NHL, revealing a median patient age at diagnosis of 57 years, with DLBCL being the most commonly identified (11 out of 21 cases).[6] DLBCL subtype of NHL is derived from activated B-cells, suggesting that an increased incidence of lymphoma may be a secondary effect of chronic inflammation in this subgroup.[7]

The exact pathogenic mechanisms of oncogenesis in SLE remain unclear, but a variety of factors including genetic, environmental, and infections are likely to contribute to its development.[3],[4],[5] Complex biological pathways including an overproliferation of lymphocytes from prolonged exposure to chronic inflammation have been proposed, leading to a sequence of events that result in lymphoma. SLE patients are also more prone to viral infections, which can increase their cancer risk. One such virus, EBV, has been linked to SLE as well as to B-cell lymphoma. It has been observed that EBV antigens share structural similarities with certain SLE antigens.[8]

Concerns have been raised against the plausible role of immunosuppressive agents commonly used to treat SLE and other autoimmune diseases.[5],[7] The plausible association between cyclophosphamide and azathioprine use and lymphoma in SLE has been widely studied in previous literature. In the studies of Sultan et al.[9] and Abu-Shakra et al.,[10] the use of cytotoxic agents was not related to the occurrence of malignancy. Mostly, evidence suggests that immunosuppressives do not increase the incidence of lymphoma in SLE patients.[11],[12],[13] In our case, the patient received only oral corticosteroids and hydroxychloroquine, of which limited data are available. It may be thus the disease itself that confers an increased risk.

Large multicentric studies are required to adequately address the risk of developing malignancies in large cohorts of patients with SLE and to address issues such as associated risk factors and additional confounding factors, such as deprivation and exposure to therapy. The chance of detection of malignancy may vary in our country due to factors such as access to health services, which vary widely and are not uniformly available, and may therefore underestimate the risk of malignancy.


  Conclusion Top


SLE has been associated with increased frequency of neoplasm including lymphomas. Non-Hodgkin's disease has been occasionally associated with SLE in adults. Prognosis relies on early detection, thus unexplained fever, weight loss, night sweats, and lymphadenopathy should raise suspicion for lymphoma and warrant an immediate treatment. An awareness of the association of non-Hodgkin's disease with SLE and the overlapping presenting features will help in the early diagnosis and clinical management of such patients. Continuing work should provide further insight into the patterns of presentation, prognosis, and etiology of NHL in SLE.

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

None.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bernatsky S, Boivin JF, Joseph L, Rajan R, Zoma A, Manzi S, et al. An international cohort study of cancer in systemic lupus erythematosus. Arthritis Rheum 2005;52:1481-90.  Back to cited text no. 1
    
2.
Mellemkjaer L, Andersen V, Linet MS, Gridley G, Hoover R, Olsen JH. Non-Hodgkin's lymphoma and other cancers among a cohort of patients with systemic lupus erythematosus. Arthritis Rheum 1997;40:761-8.  Back to cited text no. 2
    
3.
Freedmann AS, Nadler LM. Non-Hodgkin's lymphomas. In: Bast RC Jr., editor. Holland-Frei Cancer Medicine. Ontario, Canada: Hamilton, Decker; 2000. p. 2034-58.  Back to cited text no. 3
    
4.
Engels EA, Cerhan JR, Linet MS, Cozen W, Colt JS, Davis S, et al. Immune-related conditions and immune-modulating medications as risk factors for non-Hodgkin's lymphoma: A case-control study. Am J Epidemiol 2005;162:1153-61.  Back to cited text no. 4
    
5.
King JK, Costenbader KH. Characteristics of patients with systemic lupus erythematosus (SLE) and non-Hodgkin's lymphoma (NHL). Clin Rheumatol 2007;26:1491-4.  Back to cited text no. 5
    
6.
Bernatsky S, Ramsey-Goldman R, Rajan R, Boivin JF, Joseph L, Lachance S, et al. Non-Hodgkin's lymphoma in systemic lupus erythematosus. Ann Rheum Dis 2005;64:1507-9.  Back to cited text no. 6
    
7.
Jaffe ES, editor. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. larc: International Agency for Research on Cancer; 2001.  Back to cited text no. 7
    
8.
Draborg AH, Duus K, Houen G. Epstein-Barr virus and systemic lupus erythematosus. Clin Dev Immunol 2012 Jul 3;2012.  Back to cited text no. 8
    
9.
Sultan SM, Ioannou Y, Isenberg DA. Is there an associan of malignancy with systemic lupus erythematosus? An analysis of 276 patients under long-term review. Rheumatology (Oxford) 2000;39:1147-52.  Back to cited text no. 9
    
10.
Abu-Shakra M, Gladman DD, Urowitz MB. Malignancy in systemic lupus erythematosus. Arthritis Rheum 1996;39:1050-4.  Back to cited text no. 10
    
11.
Kang KY, Kim HO, Yoon HS, Lee J, Lee WC, Ko HJ, et al. Incidence of cancer among female patients with systemic lupus erythematosus in Korea. Clin Rheumatol 2010;29:381-8.  Back to cited text no. 11
    
12.
Tarr T, Gyorfy B, Szekanecz E, Bhattoa HP, Zeher M, Szegedi G, et al. Occurrence of malignancies in Hungarian patients with systemic lupus erythematosus: Results from a single center. Ann N Y Acad Sci 2007;1108:76-82.  Back to cited text no. 12
    
13.
Sweeney DM, Manzi S, Janosky J, Selvaggi KJ, Ferri W, Medsger TA Jr., et al. Risk of malignancy in women with systemic lupus erythematosus. J Rheumatol 1995;22:1478-82.  Back to cited text no. 13
    


    Figures

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



 

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