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. 2007 Mar;1(1):63-8.
doi: 10.1007/s11832-007-0013-9. Epub 2007 Feb 23.

Acute lymphoblastic leukemia in children: correlation of musculoskeletal manifestations and immunophenotypes

Eran Maman  1 , David M Steinberg, Batia Stark, Shai Izraeli, Shlomo Wientroub
Affiliations

Acute lymphoblastic leukemia in children: correlation of musculoskeletal manifestations and immunophenotypes

Eran Maman et al. J Child Orthop. 2007 Mar.

Abstract

Purpose: Studies on musculoskeletal manifestations (MSM) of childhood acute lymphoblastic leukemia (ALL) have yielded variable findings with regard to their clinical impact. We investigated the significance for differential diagnosis, treatment and outcome of musculoskeletal complaints as presenting symptoms of ALL, and their correlation with leukemia immunophenotypes, for which data is lacking.

Methods: Data on 783 children in the national study for childhood ALL between 1984 and 2003 were reviewed retrospectively. Statistical analysis examined possible relationships between MSM at the time of diagnosis and demographic and clinical data, biological features of leukemia (peripheral blood counts, immunophenotype and main cytogenetic aberration), response to initial prednisone treatment, and outcome.

Results: Of 765 children with data on orthopaedic complaints, 240 presented with MSM (31.4%). Among these children, B cell precursor (BCP) was much more common (209/576, 36.3%) than T cell ALL (25/176, 14.2%). Patients with MSM had lower white blood cell counts (WBC) (median of 9 vs. 20 x 10(9)/L, P < 0.001) and percentage of blast cells in the peripheral blood at diagnosis compared to those without (median of 27 vs. 53%, P < 0.001). Hepatomegaly and splenomegaly were less common in MSM group (67 vs. 53% <3 cm, P < 0.001, and 63 vs. 50% <3 cm, P < 0.001, respectively). Poor response to initial treatment with prednisone was recorded in 7.1% of patients with MSM versus 11.5% of those without (P = 0.086). The analysis revealed no independent effect of MSM on event-free survival (EFS), after correcting for differences in EFS related to immunophenotype or initial WBC.

Conclusions: MSM occur mostly in children with BCP ALL who present with less involvement of extramedullary organs, low peripheral blood blasts and white blood cells counts. These findings highlight the importance of including ALL in the differential diagnosis of MSM even in the presence of an apparently normal peripheral blood count. Our study also suggests that MSM are caused by leukemic cells with enhanced biological propensity to remain relatively confined within the intramedullary bone-marrow space.

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Figures

Fig. 1
Fig. 1
Kaplan–Meier event-free survival curves for children with (upper curve) and those without (lower curve) orthopaedic complaints at presentation. The low proportion of T cell leukemia and in the MSM group and the differences in initial WBC between the groups can account for the differences in outcome
See this image and copyright information in PMC

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