Treatment of Langerhans Cell Histiocytosis With Pamidronate

Abstract

A 14-year-old boy with longstanding multisystem Langerhans cell histiocytosis (LCH) had multifocal bone pain unresponsive to chemotherapy, corticosteroids, anti-inflammatories, and narcotic analgesics. He responded to two cycles of intravenous pamidronate, each 90 mg per day on three consecutive days. Two subsequent episodes of deterioration also responded to treatment. The pathophysiology of his bone pain and the likely mechanisms of biphosphonate action are discussed, with emphasis on the molecular and cellular basis of LCH.

Elevated Serum Levels of the Decoy Receptor Osteoprotegerin in Children with Langerhans Cell Histiocytosis

“…Indeed, we and others have recently shown that various osteoclastogenic factors are highly expressed in the serum and/or in lesions in LCH patients (8,24). The aforementioned observations provide a rationale for the use of bisphosphonates (potent inhibitors of bone resorption) in LCH patients with osteolytic lesions and bone pain (25,26). Moreover, the recent demonstration that pamidronate stimulates OPG production by human osteoblasts (27) further supports the notion that the RANKL-OPG axis may play an important role in this context.…”

Strahlentherapie ossärer Manifestationen der Langerhans-Zell-Histiozytose beim Erwachsenen. Review und Vorstellung eines internationalen Registers

“…Radiotherapy (RT) in patients with uni-or multifocal bone involvement in multi-system disease in LCH: studies with more than ten patients. [3,6,15]. In contrast to unifocal osseous manifestations, CT is indicated, if rapid osseous progression or imminent transformation in a multi-system disease is observed [4,5,9,13,25,50,51,54].…”

“…Many studies do not differentiate the outcome by the applied dosage, and use different dosages in the same age group. Dose recommendations for children (6 Gy) and adults (15)(16)(17)(18)(19)(20) are explained by only one study, in which the dose recommendation for children was based on two historical articles with a total of only 37 patients [8]; dose recommendation for adults was derived by higher relapse rate in adults who had received a low-dose concept.…”

Langerhans cell histiocytosis

“…Includes patients with reactivation of multifocal bone disease or lowrisk multisystem disease (without risk organ involvement); disease reactivations occur in approximately one-third of patients and they usually respond well to second-line therapy. Many regimens have been described, including oral 6-mercaptopurine and methotrexate [82], indomethacin [83], bisphosphonates [84] and cladribine [85]. (b) Patients with "high-risk" disease.…”