Uncommon Presentation and Therapeutic Strategies in Pediatric Relapsed/Refractory ALK-Positive ALCL

Area of Science:

• Hematology
• Oncology
• Pediatric Oncology

Background:

• Anaplastic large cell lymphoma (ALCL) is an aggressive T-cell malignancy.
• Secondary haemophagocytic lymphohistiocytosis (HLH) is a rare but serious complication of ALCL.

Purpose of the Study:

• To describe a challenging case of ALK-positive ALCL with concurrent HLH in a pediatric patient.
• To highlight the role of targeted therapies and multidisciplinary treatment in achieving remission.

Main Methods:

• Case report of a 6-year-old boy diagnosed with ALK-positive ALCL and HLH.
• Treatment involved intensive care, chemotherapy, targeted agents (crizotinib, ruxolitinib), dexamethasone, and allogeneic stem cell transplant.
• Management of multiple relapses was required.

Main Results:

• The patient experienced multiple relapses requiring intensive treatment.
• Successful remission was ultimately achieved with a combination of targeted therapies and allogeneic stem cell transplant.
• This case underscores the complexity of managing ALCL-HLH.

Conclusions:

• ALK-positive ALCL with concurrent HLH poses significant clinical challenges.
• Targeted therapies, such as crizotinib and ruxolitinib, play a crucial role in managing relapsed or refractory disease.
• Advances in molecular understanding and integration of novel agents are vital for improving outcomes in high-risk ALCL.