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Leukemia in a child with juvenile idiopathic arthritis and relapse of arthritis following chemotherapy completion

1 Department of Pediatrics, Division of Pediatric Haematology and Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
2 Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Date of Submission22-Dec-2021
Date of Acceptance11-Mar-2022
Date of Web Publication06-Jul-2022

Correspondence Address:
Mahesh Janarthanan,
Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai - 600 116, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_284_21

How to cite this URL:
Koneru SH, Janarthanan M, Saminathan V, Scott JX. Leukemia in a child with juvenile idiopathic arthritis and relapse of arthritis following chemotherapy completion. Indian J Rheumatol [Epub ahead of print] [cited 2022 Dec 5]. Available from:

Dear Editor,

A 9-year-old girl presented to us at the age of 1.5 years in 2013 with swelling of the right knee and elbow joint for 2 months. Physical examination revealed right knee and elbow joint swelling. No systemic features were noted. Except for raised inflammatory markers and positive antinuclear antibodies of 1:320, nuclear homogenous pattern (indirect immunofluorescence method) investigations including complete blood count (CBC) [Table 1] were normal. There was no uveitis. She was diagnosed with oligoarticular juvenile idiopathic arthritis (JIA) and treated with intra-articular injection of triamcinolone acetonide and weekly oral methotrexate (15 mg/m2) and folic acid. The disease remained in remission and methotrexate was stopped after 2 years of treatment in 2015.
Table 1: Investigations

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At 5 years of age in 2017, she presented with persistent back pain associated with nocturnal symptoms. On examination, mild hepatosplenomegaly and nonsignificant lymphadenopathy were noted. Investigations revealed white cell count (WCC) 4200/cumm (4000–11,000), neutrophils 45% (45–70), lymphocytes 54% (25–40), and platelets 1.49 lakhs/cumm (1.5–4.5). Bone marrow aspirate revealed hypercellular marrow with significant blast cells, 60% and increased N: C ratio. Flow cytometry revealed bright expression of CD10, CD33, CD22, HLA-DR AND CD13, and dim expression of CD34 and CD19. FISH showed TEL-AML1 rearrangement confirming t (12; 21). Cerebrospinal fluid (CSF) analysis was normal. A definitive diagnosis of pre-B-cell acute lymphoblastic leukemia was made. She was treated with Children's Oncology Group protocol and minimal residual disease evaluated at day 33 of induction and at the end of treatment using flow cytometry showed <0.05%.

A year after completion of chemotherapy in January 2021, she presented with left knee joint swelling. Routine investigations including CBC [Table 1] was normal except for raised erythrocyte sedimentation rate. Bone marrow aspiration showed normocellular marrow with normoblastic maturation. CSF cytology was normal. A whole-body positron emission tomography-computed tomography did not reveal malignancy. The possibility of leukemia relapse was ruled out and JIA relapse was confirmed. She was treated with intra-articular steroid and oral methotrexate was restarted. Currently, she is asymptomatic and is on regular follow-up

Increased risk of lymphoproliferative malignancies in JIA has been observed and is probably related to immune dysregulation that characterizes the disease.[1],[2],[3] Therapeutic agents used in the treatment of JIA have been implicated, although there is no strong evidence to support this theory.[4]

We report this patient with JIA who developed ALL 3 years after the initial diagnosis. JIA disease activity was quiescent when the patient received chemotherapy and relapsed after 1 year of completion of treatment. The treatment regimen for leukemia included steroids and methotrexate, and we believe this combination could have kept the JIA under control. The low normal WCC, borderline platelet count, and new-onset backache in the young child with nocturnal symptoms led to the suspicion of malignancy. Physicians should be aware of the red flag signs to differentiate musculoskeletal manifestations of malignancies when dealing with a child with arthritis.[5] To our knowledge, this is the first report of relapse of the disease in a child with JIA who developed malignancy.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Nordstrom BL, Mines D, Gu Y, Mercaldi C, Aquino P, Harrison MJ. Risk of malignancy in children with juvenile idiopathic arthritis not treated with biologic agents. Arthritis Care Res 2012;64:1357-64.  Back to cited text no. 1
Simard JF, Neovius M, Hagelberg S, Askling J. Juvenile idiopathic arthritis and risk of cancer: A nationwide cohort study. Arthritis Care Res 2010;62:3776-82.  Back to cited text no. 2
Horne A, Delcoigne B, Palmblad K, Askling J. Juvenile idiopathic arthritis and risk of cancer before and after the introduction of biological therapies. RMD Open 2019;5:e001055.  Back to cited text no. 3
Cleary AG, McDowell H, Sills JA. Polyarticular juvenile idiopathic arthritis treated with methotrexate complicated by the development of non-Hodgkin's lymphoma. Arch Dis Child 2002;86:47-9.  Back to cited text no. 4
Zombori L, Kovacs G, Csoka M, Derfalvi B. Rheumatic symptoms in childhood leukaemia and lymphoma – A ten-year retrospective study. Pediatr Rheumatol Online J 2013;11:20.  Back to cited text no. 5


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