ALK-positive anaplastic large cell lymphoma with prominent bone involvement in a 13-year-old boy

the ONA take:

A 13-year-old boy with anaplastic lymphoma kinase-positive (ALK+) anaplastic large cell lymphoma (ALCL) was successfully treated with hypofractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone/methotrexate and cytarabine (hyper-CVAD/MA), rather than cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP), followed by autologous hematopoietic cell transplantation (HCT), according to a case report published in the journal OncoTargets and Therapy.

The case report describes a pediatric patient who presented with waist pain and low-grade fever for 8 months. His serum lactate dehydrogenase level was 600 IU/L. Biopsy results of soft tissue lesions around the thoracic spine revealed ALCL. These cells were positive for ALK-1, CD30, leukocyte common antigen, CD3, CD4, and CD8, and were negative for epithelial membrane antigen and pan-cytokeratin. The boy was diagnosed with ALK+ ALCL with prominent bone involvement.

Because rapid progression is common in ALCL and B symptoms are frequent, physicians decided to initiate treatment with hyper-CVAD/MVA high-grade chemotherapy. After 2 cycles of chemotherapy, the patient’s condition improved and his LDH normalized. After 6 cycles, PET-CT findings suggested a complete remission. He then underwent consolidation therapy with autologous HCT and has remained in complete remission for 2 years.

Although chemotherapy regimens like CHOP and hyper-CVAD/MVA have demonstrated efficacy in children and young adults with ALCL, novel agents like brentuximab vedotin may drastically alter the approach to treating this disease.

OncoTargets and Therapy
OncoTargets and Therapy

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 

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.)  

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