Dr. Nandhini

Dr. Nandhini  |Clyto Access

CRRI, Sri Ramachandra University

Poster Presentation






Ana plastic  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. Biopsy of the ulcer revealed ALCL. Metastatic evaluation revealed a disseminated disease.
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 helps in the identification of this rarer entity.
In a 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.


Related Conferences :

International Conference on Cancer Care and Cure