Chronic Lymphocytic Leukemia in an Adolescent Girl: A Case Report and Clinico-Pathologic Review

Chronic lymphocytic leukemia (CLL) in pediatric age is rare in the literature. It is a common disease of older adults, characterized by clonal proliferation and progressive accumulation of monoclonal B-cell lymphocytes. The diagnosis is established by immunophenotyping and prognosis is defined by staging system (Rai and Binet), as well as by several biological and genetic markers. We report a case of CLL diagnosed in an adolescent girl presented at 16 years of age. The case is being reported to improve awareness regarding this rare entity in children.

Case Presentation: A 16 year old female presented with fever, weight loss and cervical lymphadenopathy. After baseline workup, lymph node biopsy, bone marrow biopsy along with immunohistochemistry and flowcytometry on peripheral blood was performed to establish the diagnosis. The clinico-pathologic features including extensive immunophenotyping were consistent with CLL.

Discussion: Management guidelines for older adults are very well established but no standardized protocol exists for pediatric age group. We offered her Fludarabine and Cyclophosphamide as first line regimen and she responded well and achieved remission after four cycles. Unfortunately disease relapsed within two years. At this time, determination of optimum therapeutic protocol was a unique challenge as hematopoietic stem cell transplant (HSCT) was not available at our institute.

Conclusion: CLL is an extremely rare malignancy in childhood and adolescence. Therefore age specific treatment protocols are not established. Reporting this case will help in eliciting the high index of suspicion among pathologists and oncologists for this exceptionally unusual and life threatening disease so that delays can be avoided.