Introduction: Anaplastic lymphoma kinase-positive (ALK+) anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma, which has strong expression of cluster of differentiation (CD)-30 and ALK. ALCL sometimes can involve the bone marrow, and in advanced stages, it can produce destructive bone lesions. But ALK+ ALCL with prominent bone involvement is very rare, especially in children. 
Case report: A 13-year-old boy presented with waist pain and low-grade fever for 8 months. The biopsy of soft tissue lesions around the thoracic spine showed that these cells were positive for ALK-1, CD30, leukocyte common antigen, CD3, CD4, and CD8, as well as being negative for epithelial membrane antigen and pan-cytokeratin, which revealed ALCL. After six cycles of a regimen consisting of hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone/methotrexate and cytarabine (hyper-CVAD/MA) and autologous hematopoietic stem cell transplantation, he achieved complete remission (CR). 
Conclusion: It is generally believed that the regimen consisting of cyclophosphamide, hydroxydaunorubicin (doxorubicin), vincristine, and prednisolone (CHOP) is also applicable to ALCL. Because of the tendency of rapid progression and the frequency of B symptoms, ALCL in children and young adults is treated with high-grade chemotherapy such as hyper-CVAD/MA.


Keywords: anaplastic large cell lymphoma, anaplastic lymphoma kinase, bone involvement, hyper-CVAD/MA 



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INTRODUCTION

Anaplastic large cell lymphoma (ALCL) is a distinct clinicopathologic entity of non-Hodgkin lymphoma (NHL), which comprises approximately 15% of all NHL cases in children. ALCL in children commonly presents with advanced systemic disease.1 Although ALCL frequently involves the bone marrow, primary or secondary involvement to bone is rare. Here, we report a case of anaplastic lymphoma kinase positive (ALK+) ALCL with prominent bone involvement in a 13-year-old boy.

CASE REPORT

A 13-year-old boy presenting with waist pain and low-grade fever for 8 months was examined in our hospital. Physical examination revealed lymphadenopathy in the neck and hepatosplenomegaly. Serum lactate dehydrogenase (LDH) was elevated to 600 IU/L (normal: 200–460 IU/L), but other laboratory data were normal. Computed tomography (CT) scan of chest and abdomen revealed hepatosplenomegaly. 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET) scanning showed increased FDG avidity involving the thoracic vertebrae 1 and 8, the left ribs 4 and 6, the thoracic spine, sacrum, bilateral ilium, left pubis, and femur, suggesting lymphoma involvement (Figure 1). PET-CT also showed an FDG-avid neck mass, suggesting lymphadenopathy. A CT-guided biopsy of soft tissue lesions around the thoracic spine revealed ALCL. These cells were positive for ALK-1 (Figure 2A), CD30 (Figure 2B), leukocyte common antigen, CD3, CD4, and CD8, as well as being negative for epithelial membrane antigen and pan-cytokeratin. Bone marrow aspiration and trephine biopsy showed no infiltration. He was diagnosed as having ALK-positive ALCL with prominent bone involvement. After two cycles of chemotherapy with hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone/methotrexate and cytarabine (hyper-CVAD/MA), his condition improved, and the level of serum LDH returned to normal. After four cycles of hyper-CVAD/MA chemotherapy, PET-CT showed no significant uptake of FDG. After six cycles of hyper-CVAD/MA regimen, PET-CT showed no uptake of FDG, suggesting complete remission (CR). Then, he underwent autologous hematopoietic stem cell transplantation (AHSCT) as consolidation therapy. At present, he has remained in the CR stage for 2 years. Maintenance chemotherapy has not been given.

(To view a larger version of Figure 1, click here.)


(To view a larger version of Figure 2, click here.)