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Treatment of Pediatric Idiopathic Pulmonary Hemosiderosis with Low-Dose Cyclophosphamide

Pediatric Critical Care Pharmacist, Department of Pharmacy, Chang-Gung Memorial Hospital, Kaohsiung, Taiwan

Ping-Yu Lee, MSc

Director, Department of Pharmacy, Chang-Gung Memorial Hospital

Chen-Kuang Niu, MD

Chief, Division of Pediatric Pulmonology, Chang-Gung Children's Hospital, Kaohsiung

Reprints: Chen-Kuang Niu MD, Division of Pediatric Pulmonology, Chang-Gung Children's Hospital, No. 123, Dabi Rd., Niausung Shiang, 833, Kaohsiung, Taiwan, FAX 886 7 7338009, niusnet{at}ms10.hinet.net

OBJECTIVE: To report the safety and efficacy of long-term, low-dose cyclophosphamide therapy in a child with idiopathic pulmonary hemosiderosis (IPH).

CASE SUMMARY: A 7-year-old boy diagnosed with IPH 4 years previously was initially prescribed prednisolone. Because he only had a transient response to prednisolone, oral cyclophosphamide 2 mg/kg/d was later added. A dramatic improvement was noted during the subsequent follow-up. One year after cyclophosphamide therapy, the patient suddenly developed thrombocytopenia (platelet count 75 x 10³/mm³), with the platelet count decreasing to 10 x 10³/mm³ over the following 10 months. Cyclophosphamide was tapered to an alternating daily dosage of 1 mg/kg. The tapering resulted in a subsequent increase in the platelet count, which was maintained between 20 and 50 x 10³/mm³ without occurrence of petechiae or spontaneous bleeding. Under this reduced dosing regimen, the disease has remained in remission for >1 year.

DISCUSSION: Due to the low prevalence of IPH, only limited data document the safety and efficacy of immunosuppressive therapy in treating this disease. Although our patient showed a good response to low-dose cyclophosphamide, he developed thrombocytopenia with its use. The mechanism is unclear, but it may be similar to that of high-dose cyclophosphamide-induced myelosuppression. Due to the development of thrombocytopenia, the use of cyclophosphamide was maintained under a reduced dosing regimen. The benefit of long-term immunosuppressive therapy is controversial, and more clinical evidence is required to support its continued usage.

CONCLUSIONS: Long-term, low-dose cyclophosphamide is effective in treating childhood IPH, but caution should be exercised due to the possible development of thrombocytopenia. Periodic monitoring of the platelet count in long-term treatment is recommended.

Key Words: cyclophosphamide, low-dose, idiopathic pulmonary hemosiderosis, pediatrics, thrombocytopenia

Published Online, October 1, 2003. www.theannals.com, DOI 10.1345/aph.1D042

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