Discrimination of acute lymphoblastic leukemia from systemic-onset juvenile idiopathic arthritis at disease onset.
OBJECTIVE
To assess clinical and laboratory features that differentiate acute lymphoblastic leukemia from systemic juvenile idiopathic arthritis at disease onset.


METHODS
Fifty-seven leukemia patients with musculoskeletal involvement, without blasts on peripheral blood and without glucocorticoid therapy at disease onset and 102 systemic juvenile idiopathic arthritis patients (International League of Associations for Rheumatology criteria) were retrospectively evaluated. The following features were examined: fever, rheumatoid rash, arthritis, limb pain, hepatomegaly, splenomegaly, pericarditis, myocarditis, pleuritis, weight loss, bleeding, anemia, leukopenia, neutropenia, thrombocytopenia, erythrocyte sedimentation rate, and lactic dehydrogenase levels.


RESULTS
The median age at disease onset was significantly higher in leukemia patients than in those with systemic-onset juvenile idiopathic arthritis (5.8 vs. 3.8 years). In addition, the frequencies of limb pain, hepatomegaly, weight loss and hemorrhagic manifestations were significantly higher in leukemia patients than in systemic-onset juvenile idiopathic arthritis patients (70% vs. 1%, 54% vs. 32%, 30% vs. 8%, and 9% vs. 0%, respectively). Likewise, the frequencies of anemia, leukopenia, neutropenia, thrombocytopenia and high lactic dehydrogenase levels were statistically higher in leukemia patients than in patients with systemic-onset juvenile idiopathic arthritis (88% vs. 57%, 39% vs. 1%, 60% vs. 1%, 77% vs. 1%, and 56% vs. 14%, respectively). Remarkably, multivariate analysis revealed that limb pain (OR = 553; 95% CI = 46.48-6580.42) and thrombocytopenia (OR = 754.13; 95% CI = 64.57-8806.72) were significant independent variables that differentiated leukemia from systemic-onset juvenile idiopathic arthritis. The R2 of the Nagelkerke test was 0.91, and the Kaplan-Meier survival curves were similar for acute lymphoblastic leukemia patients with and without limb pain.


CONCLUSIONS
Our study emphasizes the importance of investigating leukemia in patients presenting with musculoskeletal manifestations and, in particular, limb pain associated with thrombocytopenia.
Pediatric Rheumatology Unit, Faculdade de Medicina, Instituto da Criança, Universidade de São Paulo, São Paulo, Brazil.
Mirian S
MS
Tamashiro
Nádia Emi
NE
Aikawa
Lucia Maria A
LM
Campos
Lílian Maria
LM
Cristofani
Vicente
V
Odone-Filho
Clovis A
CA
Silva
D000293:Adolescent; D001171:Arthritis, Juvenile / Q000097:blood / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004812:Epidemiologic Methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007970:Leukopenia / Q000097:blood; D008297:Male; D059352:Musculoskeletal Pain / Q000209:etiology; D009503:Neutropenia / Q000097:blood; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood / Q000175:diagnosis*; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000097:blood