Extensive skeletal lesions in childhood acute lymphoblastic leukemia.
ABSTRACT A 2 years old male child was admitted in Dhaka Shishu Hospital with one month history of fever, swelling and pain in joints of right leg. Hematological and microbiological investigation revealed normal except radiological findings when there were destructive lesions of affected bones and thought to be Langerhan's cell histiocytosis but did not respond to treatment. Subsequently, he was transfer to Pediatric Hematology and Oncology, Bangabandhu Sheikh Mujib Medical University (BSMMU) for proper management. On examination, the boy was mildly pale, severe bone pain, no organomegaly but few occipital group of lymphnode were palpable. He had extensive red tender swelling over the hands and legs. Hematological values showed high WBC, low platelet count and lymphoblasts. Biochemical values were high serum LDH and serum uric acid. Skeletal survey showed diffuse osteolytic lesion and osteoporosis with evidence of transverse metaphyseal radiolucent bands (leukemic line), lamellar periosteal reactions and cortical erosions widespread throughout the skeleton with subperiosteal new bone formation. Radioisotop of skeleton showed increase uptake at the site of lesions. Bone marrow aspiration findings were suggestive of ALL-L1 and cytochemistry of aspirate showed PAS positive and Sudan black negative. Immunophenotype confirmed as pre-B ALL, Then, protocol based induction had given for 4 weeks. Thereafter, a short course of intensification followed by maintenance therapy had started with significant improvement of physical, hematological and radiological findings. So, an awareness of varied clinical and radiological manifestations of childhood ALL in bone marrow and skeletal system are needed in order to establish a correct diagnosis when the presenting signs and symptoms are enigmatic. It is also important to keep in mind the possibility of extensive skeletal involvement in ALL, especially when a child present with pain, swelling of limb with walking difficulties. The curability of ALL in contrast to metastatic malignancies makes accurate diagnosis of paramount importance.
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ABSTRACT: Interference with the molecular mechanisms that generate tumor supportive niches in the bone microenvironment is a rational approach to inhibit the growth of hematological malignancies. However, the advancement of knowledge in this area has been slowed down by the lack of in vitro models to facilitate the screening of potential candidate agents. The rare cases of acute lymphoblastic leukemia (ALL) in children presenting with extensive bone involvement may represent an exaggerated form of some aspects of the normal tumor-bone interactions. Thus, these cases can provide insight into processes that are otherwise challenging to uncover. The authors describe the case of a 6-year-old child who presented with severe osteopenia that resolved at the time of leukemic remission. Compared to control sera, serum taken at disease presentation contained increased levels of a group of osteolytic cytokines and was effective in activating preosteoclast cells in culture. Based on these findings, the authors describe an experimental model to identify agents that would interfere with leukemia mediated osteolytic process.Pediatric Hematology and Oncology 04/2010; 27(3):212-27. DOI:10.3109/08880011003663382 · 0.96 Impact Factor
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ABSTRACT: Carcinogenic agents such as N-methyl-N-nitrosourea can cause tumors. The aims of the present study were to evaluate and classify a subtype of AML (acute myeloid leukemia) that was induced by NMU. According to previous publications, NMU induces not only mammary cancer but also leukemia in Sprague-Dawley (S-D) rats. However, the subtype of leukemia involved in NMU-treated rats is unknown. We found that both organ weight and relative organ weights were significantly higher in NMU-exposed rats than in controls. Morphological changes of rat livers and spleens were assessed by histological evaluation (H&E staining), which found that these tissues were abnormal in appearance. Also, cytological examination of the blood showed immature white blood cells in a smear using Liu's and Papanicolaou stains, indicating that gross abnormalities and histopathological changes were pathologically observed. NMU leukemia incidence was 97.1%. In this study, immunohistochemical (IHC) analysis was valuable in classifying the leukemia of poorly differentiated blasts induced by NMU. Paraffin blocks were stained for MPO, CD3, CD15, CD20, and CD34 markers. The NMU-induced group was positive for MPO, but negative for CD3, CD15, CD20, and CD34. These CD markers suggest that they are useful in helping diagnose APL (M3) leukemia. The model of NMU-induced leukemogenesis in an S-D rat suggests a more definite way to classify APL. This APL will provide an important tool for chemical carcinogenesis and leukemia studies.Archives of Toxicology 10/2011; 86(2):315-27. DOI:10.1007/s00204-011-0753-7 · 5.08 Impact Factor