High Dose Cladribine in Combination with High Dose Cytarabine as A Salvage Therapy for Adults with Refractory Relapsed Langerhans Cell Histiocytosis Volume 4 - Issue 4

Naif I AlJohani*, Nada AlHarbi, Jalil Ur Rehman, Binyam Usman

• Adult Hematology and Bone marrow transplant section, Oncology Department, King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia

Received:June 11, 2021   Published: June 22, 2021

Corresponding author: Naif I AlJohani, Section Head & Consultant, Adult Hematology and Bone Marrow Transplant. Oncology Department, King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia

DOI: 10.32474/OAJOM.2021.04.000194

 

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Abstract

Introduction

Langerhans cell histiocytosis (LCH) is a rare condition with extremely variable manifestations ranging from single-organ involvement to multisystemic dysfunction that can be refractory or relapse. Currently, no international standard care guidelines exist for treating adult patients with refractory, multisystem LCH. This case series describes three patients with refractory multisystem LCH who failed to respond to multiple lines of chemotherapy or radiotherapy but remarkably achieved sustained complete metabolic remission after treatment with a modified “pediatric-inspired” cladribine-based intensive induction (CLAG) protocol.

Presentation of Cases

Case 1 was a 29-year-old female who presented with chronic left thigh pain and an abnormal gait. Case 2 was a 39-year-old male who presented with a 2-year history of generalized body and back pain. Case 3 was a 21-year-old female who presented with increasing maxillary pain, night sweats, and weight loss. In all three cases, the initial radiological imaging showed osteolytic lesions and tissue biopsies confirmed the diagnosis of LCH. Case 1 received standard induction chemotherapy and two lines of salvage therapy before the CLAG protocol was initiated. Case 2 was treated with standard induction chemotherapy, and the CLAG protocol was used as first line salvage therapy. Case 3 was treated with standard induction chemotherapy and the CLAG protocol used as second line salvage therapy. In Case 1, maintenance therapy was stopped at 6 months due to recurrent infection and prolonged cytopenia. In Case 2, significant changes were made to the maintenance phase protocol because of side effects. The CLAG protocol resulted in complete metabolic remission in all patients based on follow-up PET/CT examinations.

Conclusion

We report 3 cases of refractory/relapsed LCH in adult patients who experienced complete metabolic remission following salvage chemotherapy using a modified CLAG protocol after failure of at least one line of standard induction therapy. Keywords: Cladribine; Cytarabine; Langerhans Cell Histiocytosis; Osteolytic Lesions

Abstract| Introduction| Clag Protocol| Case Reports| Discussion| Conclusion| Acknowledgment| Disclosure| Ethical Approval and Informed Consent| Consent for Publication| Funding| Authors’ contributions| References|