Conference Proceeding


Dr. Nandhini ,
Sri Ramachandra University, Chennai, India

Introduction: Anaplastic large cell lymphoma (ALCL) is a rare large cell, malignant non Hodgkin lymphoma, accounts for 10-15% of pediatric NHL, characterized by expansion of pleomorphic large lymphoid cells, expressing the CD30 antigen. Systemic ALCL manifesting as isolated skin ulcer with no hepatosplenomegaly, lymphadenpathy in pediatric age group is rare. We present a case of 4 year old child who had non healing progressive ulcer and fever spikes with no other symptoms. Biographypsy of the ulcer revealed ALCL. Metastatic evaluation revealed a disseminated disease.
Case Report: A 4 year old developmentally normal male child presented with intermittent high grade fever for three months. He developed an abscess in left gluteal region two months later, following IM injection. Child underwent incision & drainage and had multiple courses of oral and iv antiBiographytics. The ulcer was non healing and progressive, and had repeated debridements. He was brought to our centre for further management. On examination, he had no pallor, hepatosplenomegaly or lymphadenopathy. Multiple hard nodules were noted bilaterally over both extremities. He also had cellullitis over right calf, ultrasound of which revealed pus collection of around 20-25ml. A 4cmsX 5cms X 2 cms necrotic ulcer was seen over left gluteal region. Biographypsy of the ulcer revealed highly mitotic malignant cells. Immunohistochemistry was confirmatory of anaplastic large cell lymphoma. Staging investigations revealed disseminated disease. After counseling the diagnosis and prognosis, he was started on chemotherapy. Fever subsided after 72 hours of chemotherapy and there was complete healing of ulcer after 2 cycles of chemotherapy.
Discussion: ALCL has 2 forms, primary cutaneous and systemic. Primary cutaneous ALCL accounts for 9 % of cutaneous lymphomas and arises denovo in the skin. Systemic ALCL is more frequent than primary cutaneous form. Systemic form involves lymph nodes in 90% of cases, extra nodal sites such as skin (25%), lung (10%), bone (17%) and liver (8%) are also involved. Systemic type with cutaneous spread must be distinguished from primary cutaneous ALCL as the systemic type is an aggressive disease and needs multi agent systemic chemotherapy. Further, systemic ALCL may be divided based on the expression of anaplastic lymphoma kinase (ALK) protein. ALK positive systemic ALCL is seen in children and younger population with male predominance. Systemic ALK+ ALCLs have improved prognosis compared with ALK-negative ALCL. In Systemic ALCL, B symptoms are present in 75% of cases and bone marrow involvement is seen in 11% of cases. Approximately two-thirds of patients with systemic ALCL are known to have advanced-stage disease at presentation. The rate of misdiagnosis is high due to variety of non specific clinical manifestations. Immunohistochemistry and translocation studies help in the identification of this rarer entity.
Conclusion: In an immunocompetent child, a non healing ulcer could be a manifestation of a underlying malignancy and hence through systemic evaluation should be done to identify the primary cause.

Published: 11 May 2017