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CASE REPORT
Year : 2013  |  Volume : 40  |  Issue : 1  |  Page : 44-46

Migratory polyarthritis in aleukemic lymphoblastic leukemia: An undesignated paraneoplastic syndrome


Department of Pediatrics, KLE University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India

Date of Web Publication28-Mar-2013

Correspondence Address:
G P Prashanth
Department of Pediatrics, Jawaharlal Nehru Medical College, Nehru Nagar, Belgaum - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-5009.109704

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  Abstract 

Arthritic presentation of childhood acute leukemias is well known. A subset of these children may have peripheral blast cells undetectable (aleukemic leukemia). We report classical asymmetric, fleeting type of polyarthritis involving the large weight-bearing joints along with remittent fever and normal leukocyte counts as a presenting feature of acute lymphoblastic aleukemic leukemia, deliberated as a 'paraneoplastic syndrome' of childhood.

Keywords: Acute leukemia, children, paraneoplastic syndrome, polyarthritis


How to cite this article:
Jali S, Prashanth G P, Amarkhed P. Migratory polyarthritis in aleukemic lymphoblastic leukemia: An undesignated paraneoplastic syndrome. J Sci Soc 2013;40:44-6

How to cite this URL:
Jali S, Prashanth G P, Amarkhed P. Migratory polyarthritis in aleukemic lymphoblastic leukemia: An undesignated paraneoplastic syndrome. J Sci Soc [serial online] 2013 [cited 2019 Jul 18];40:44-6. Available from: http://www.jscisociety.com/text.asp?2013/40/1/44/109704


  Introduction Top


Acute leukemia can present with varied manifestations in children including arthritic presentation which is known for over 75 years. [1] Several studies in the past have analyzed the orthopedic manifestations at presentation in leukemia and its differentiation from other conditions. [2],[3],[4] Here we report a case series of three children who presented with fever and migratory polyarthritis along with normal blood counts. They were treated for presumed rheumatic fever, with immobilization of the joints with cast in one case; subsequent evaluation revealing the diagnosis of acute lymphoblastic leukemia (ALL). Arthritis resolved with the initiation of chemotherapy. We highlight the strong association of fever, classical migratory polyarthritis of large joints, and blast-negative leukemia as an evident 'paraneoplastic syndrome' in ALL.


  Case Reports Top


Case 1

An 8-year-old boy presented to us with a history of fever and migratory polyarthritis of 2-month duration. He had pain and swelling in the left ankle initially; subsequently, there was serial involvement of other large joints of upper and lower limbs, each lasting for a brief time. Blood counts and peripheral smear were normal. Multiple x-rays of affected joints were normal. The child was seen by two pediatricians who managed on the lines of acute rheumatic fever. In view of severity of pain, swelling, and inability to bear weight on the joint, an immobilizing cast was applied to the left leg before referral. When we examined, the child had pallor, and moderate hepatosplenomegaly. Investigations showed hemoglobin 4 g/dL, total leukocytes 48 × 10 9 /L, and a platelet count of 93 × 10 9 /L. Peripheral smear showed numerous lymphoblasts containing moderate amount of basophilic cytoplasm and hyperchromic indented nuclei. Bone marrow showed sheets of lymphoblasts of varying sizes; blasts were 90% with 5% erythroid and myeloid cells each, suggestive of ALL-L2. Flow cytometry showed CD10 positivity in 90% cells. Arthritis resolved with initiation of chemotherapy.

Case 2

A 12-year-old healthy boy presented with fever and acute-onset arthritis of 20-day duration. Right knee was first involved, which subsided spontaneously within a week. Ankle joint of the same limb was affected later. Left knee and ankle joint were involved over the next 12 days. There was no history of bony pain, myalgia, night sweats, loss of appetite, or recent significant weight loss. Small joints, axial joints, and joints of the upper limb were not affected. There was no preceding trauma, sore throat, skin rash, or bowel disturbances. The child had no pallor, lymphadenopathy, organomegaly, or bony tenderness. Examination of the eyes was normal. Laboratory investigations revealed hemoglobin 10.4 g/dL, leukocytes 9 × 10 9 /L, and platelets 240 × 10 9 /L. Peripheral blood smear was normal. X-ray of the affected joints, and synovial fluid analysis showed no abnormality. Hepatic and renal function tests were normal. Antistreptolysin-O (ASO) titer, C-reactive protein, rheumatoid factor, antinuclear antibody, creatine kinase, and hepatitis-B serological tests were all negative. Bone marrow aspiration done later confirmed the diagnosis of ALL-L2. Symptoms resolved with initiation of chemotherapy.

Case 3

A 12-year-old girl had a limp in her right lower limb. The symptoms subsided after 8 days with use of anti-inflammatory drugs. Later, she was admitted with fever and pain in both legs (knee and ankle). Her complete blood count was normal. ASO titers, rheumatoid factor, and antinuclear antibodies were negative. Symptoms were unresponsive to aspirin and naproxen. Radiographs of joints were normal. Cast was put by the treating physician to immobilize the painful joints before referral to our center. At admission, she had sternal tenderness, mild pallor, and petechiae over extremities. Despite a normal peripheral blood smear, bone marrow aspiration was done in view of the strong suspicion of leukemia. The diagnosis was later confirmed as ALL subtype L2. Fever and joint symptoms subsided with initiation of chemotherapy.


  Discussion Top


About 10-30% children with leukemia may experience osteoarticular symptoms at presentation. [5] Arthritis in leukemia may occur at any stage of the disease and joint symptoms may last for several months before leukemia is diagnosed. A subset of children with ALL may have normal leukocyte count at presentation wherein peripheral blast cell may not be detectable (aleukemic leukemia) for several months. The characteristics predictive of leukemia in these cases are as follows: A clinical history of night-time pain, the laboratory findings of leucopenia, low-normal thrombocytopenia, and elevated serum lactate dehydrogenase. [4],[6] Fever, lymphadenopathy, organomegaly, and increased erythrocyte sedimentation rate have limited value in distinguishing leukemia from other conditions. [2] Radiological findings of lytic/sclerotic bone lesions, presence of radiolucent bands and periosteal new bone formation may help in picking up malignancy early. [7] The utility of a thorough history, physical examination, and basic laboratory tests in recognizing ALL has been statistically validated. [4] None of the three cases reported here had the above mentioned features at presentation.

Paraneoplastic syndromes refer to the disorders that accompany benign or malignant conditions, present during the course of and/or preceding the diagnosis of malignancy which respond to the treatment of underlying neoplasm. [8] All the cases reported here satisfied these criteria. Migratory polyarthritis with associated polymyositis, hypercalcemia has been previously identified as a paraneoplastic syndrome in adults. [9],[10],[11] Well-known paraneoplastic rheumatic diseases in adults include palmar fasciitis and polyarthritis syndrome, hypertrophic osteoarthropathy, and remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome reported in ovarian, gastric, and breast carcinomas. [12] Leukemic arthritis is a well-known clinical entity in pediatric medicine; although in many instances it is not reported whether arthritis was classical fleeting-type polyarthritis. Leukemic infiltration of synovium and gout may also cause joint symptoms in leukemia. [1],[13]

The emphasis in our report is on asymmetric and fleeting-type polyarthritis involving the large joints as a presenting feature of acute lymphoblastic aleukemic leukemia, so far not deliberated as a paraneoplastic syndrome in children. Nevertheless, a constellation of polyarthritis (90%) and pyrexia (80%), and normal blood counts (30%) has been evident in previous reports of osteoarticluar manifestations of ALL. [5],[7],[14],[15] The specific mechanism for these paraneoplastic symptoms remains to be elucidated. [16]


  Conclusion Top


ALL is a challenging disease both to diagnose and treat. The clinicians must be aware of the constellation of remittent fever and migratory polyarthritis, and normal blood counts in aleukemic leukemia. A high index of suspicion along with the awareness of this paraneoplastic syndrome may lead to prompt bone marrow examination to establish the diagnosis and avoidance of deleterious 'blind' treatment with immunosuppressant. Perhaps, further investigation of pathogenesis and outcome of ALL subgroup presenting with this syndrome is justified. [4],[5]

 
  References Top

1.Vining CW, Thomson JG. Gout and aleukaemic leukaemia in a boy aged five. Arch Dis Child 1934;9:277-84.  Back to cited text no. 1
    
2.Jonsson OG, Sartain P, Ducore JM, Buchanan GR. Bone pain as an initial symptom of childhood acute lymphoblastic leukemia: Association with nearly normal hematologic indexes. J Pediatr 1990;117:233-7.  Back to cited text no. 2
    
3.Jones OY, Spencer CH, Bowyer SL, Dent PB, Gottlieb BS, Rabinovich CE. A multicenter case-control study on predictive factors distinguishing childhood leukemia from juvenile rheumatoid arthritis. Pediatrics 2006;117:e840-4.  Back to cited text no. 3
    
4.Marwaha RK, Kulkarni KP, Bansal D, Trehan A. Acute lymphoblastic leukemia masquerading as juvenile rheumatoid arthritis: Diagnostic pitfall and association with survival. Ann Hematol 2010;89:249-54.  Back to cited text no. 4
    
5.Spasova MI, Stoyanova AA, Moumdjiev IN, Dimitrov HK. Childhood acute lymphoblastic leukemia presenting with osteoarticular syndrome-characteristics and prognosis. Folia Med (Plovdiv) 2009;51:50-5.  Back to cited text no. 5
    
6.Wallendal M, Stork L, Hollister JR. The discriminating value of serum lactate dehydrogenase levels in children with malignant neoplasms presenting as joint pain. Arch Pediatr Adolesc Med 1996;150:70-3.  Back to cited text no. 6
    
7.Rogalsky RJ, Black GB, Reed MH. Orthopaedic manifestations of leukemia in children. J Bone Joint Surg Am 1986;68:494-501.  Back to cited text no. 7
    
8.Jameson JL, Longo DL. Paraneoplastic syndromes: Endocrinologic/hematologic. In: Longo DL, Fauci AS, Kasper DL, editors. Harrison's Principles of Internal Medicine. 18 th ed. New York: McGraw-Hill Professional; 2012. p. 826-32.  Back to cited text no. 8
    
9.Pfitzenmeyer P, Bielefed P, Tavernier C, Besancenot JF, Gaudet M. Current concepts of paraneoplastic acute polyarthritis. Rev Med Interne 1992;13:195-9.  Back to cited text no. 9
    
10.Zupancic M, Annamalai A, Brenneman J, Ranatunga S. Migratory polyarthritis as a paraneoplastic syndrome. J Gen Intern Med 2008;23:2136-9.  Back to cited text no. 10
    
11.Lima M, Coutinho J, Bernardo L, dos Anjos Teixeira M, Casais C, Canelhas A, et al. Philadelphia-positive T-cell acute lymphoblastic leukemia with polymyositis, migratory polyarthritis and hypercalcemia following a chronic myeloid leukemia. Ann Hematol 2002;81:174-7.  Back to cited text no. 11
    
12.Nagasawa K. Rheumatic manifestations in paraneoplastic syndrome. Internal Med 2000;39:685-6.  Back to cited text no. 12
    
13.Fam AG, Voorneveld C, Robinson JB, Sheridan BL. Synovial fluid immunocytology in the diagnosis of leukemic synovitis. J Rheumatol 1991;18:293-6.  Back to cited text no. 13
    
14.Chell J, Fernandes JA, Bell MJ. The orthopaedic presentation of acute leukaemia in childhood. Ann R Coll Surg Engl 2001;83:186-9.  Back to cited text no. 14
    
15.Suri D, Ahluwalia J, Sachdeva MS, Das R, Varma N, Singh S. Arthritic presentation of childhood malignancy: Beware of normal blood counts. Rheumatol Int 2011;31:827-9.  Back to cited text no. 15
    
16.Weinberger A, Schumacher HR, Schimmer BM, Myers AR, Brogadir SP. Arthritis in acute leukemia. Clinical and histopathological observations. Arch Intern Med 1981;141:1183-7.  Back to cited text no. 16
    




 

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Introduction
Case Reports
Discussion
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