Therapeutic effect of sirolimus for lymphangioleiomyomatosis remaining in the abdominopelvic region after lung transplantation: A case report

T. Ito, Manabu Suno, K. Sakamoto, Y. Yoshizaki, K. Yamamoto, R. Nakanishi, Y. Hirano, M. Irie, Takeshi Kurosaki, Shinji Otani, Masaomi Yamane, Seiichiro Sugimoto, K. Miyoshi, Takahiro Oto

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Abstract

Purpose Sirolimus (SRL) is used to treat pulmonary lymphangioleiomyomatosis (P-LAM). There is limited evidence that SRL has systemic efficacy for the patients with extrapulmonary lymphangioleiomyomatosis (E-LAM) remaining after lung transplantation (LT) for P-LAM. This report examines the efficacy of SRL treatment for the patient with E-LAM remaining after an LT for P-LAM. Case Summary The course of the patient's recovery from an LT for P-LAM was complicated by lymphedema in the left femoral region that was caused by two E-LAM lesions remaining in the left pelvic cavity and in the retroperitoneal area. After the LT was performed, the patient started SRL treatment to reduce the E-LAM lesions. The daily SRL dose, selected based on the standard SRL dose for P-LAM, was initiated at 1 mg/d and was maintained at 2 mg/d. The remaining E-LAM lesions and lymphedema in the left femoral region improved in approximately 9 months after the LT with the administration of both SRL and the standard immunosuppressive therapy used by Okayama University Hospital, including tacrolimus, mycophenolate mofetil, and prednisolone. The SRL and tacrolimus trough concentrations in whole blood were maintained within the therapeutic window for the next 1.5 years after initiation of SRL treatment. The patient experienced no severe adverse events that required discontinuation of the SRL treatment during this time. Conclusion The patients with remaining E-LAM lesions may receive SRL treatment to improve the quality of life after LT for P-LAM as effective therapy in cases where the patient's recovery is complicated by E-LAM lesions.

Original languageEnglish
Pages (from-to)271-274
Number of pages4
JournalTransplantation Proceedings
Volume48
Issue number1
DOIs
Publication statusPublished - Jan 1 2016

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Lymphangioleiomyomatosis
Lung Transplantation
Therapeutic Uses
Sirolimus
Lung
Lymphedema
Tacrolimus
Thigh
Therapeutics
Mycophenolic Acid
Immunosuppressive Agents
Prednisolone

ASJC Scopus subject areas

  • Surgery
  • Transplantation

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Therapeutic effect of sirolimus for lymphangioleiomyomatosis remaining in the abdominopelvic region after lung transplantation : A case report. / Ito, T.; Suno, Manabu; Sakamoto, K.; Yoshizaki, Y.; Yamamoto, K.; Nakanishi, R.; Hirano, Y.; Irie, M.; Kurosaki, Takeshi; Otani, Shinji; Yamane, Masaomi; Sugimoto, Seiichiro; Miyoshi, K.; Oto, Takahiro.

In: Transplantation Proceedings, Vol. 48, No. 1, 01.01.2016, p. 271-274.

Research output: Contribution to journalArticle

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abstract = "Purpose Sirolimus (SRL) is used to treat pulmonary lymphangioleiomyomatosis (P-LAM). There is limited evidence that SRL has systemic efficacy for the patients with extrapulmonary lymphangioleiomyomatosis (E-LAM) remaining after lung transplantation (LT) for P-LAM. This report examines the efficacy of SRL treatment for the patient with E-LAM remaining after an LT for P-LAM. Case Summary The course of the patient's recovery from an LT for P-LAM was complicated by lymphedema in the left femoral region that was caused by two E-LAM lesions remaining in the left pelvic cavity and in the retroperitoneal area. After the LT was performed, the patient started SRL treatment to reduce the E-LAM lesions. The daily SRL dose, selected based on the standard SRL dose for P-LAM, was initiated at 1 mg/d and was maintained at 2 mg/d. The remaining E-LAM lesions and lymphedema in the left femoral region improved in approximately 9 months after the LT with the administration of both SRL and the standard immunosuppressive therapy used by Okayama University Hospital, including tacrolimus, mycophenolate mofetil, and prednisolone. The SRL and tacrolimus trough concentrations in whole blood were maintained within the therapeutic window for the next 1.5 years after initiation of SRL treatment. The patient experienced no severe adverse events that required discontinuation of the SRL treatment during this time. Conclusion The patients with remaining E-LAM lesions may receive SRL treatment to improve the quality of life after LT for P-LAM as effective therapy in cases where the patient's recovery is complicated by E-LAM lesions.",
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T1 - Therapeutic effect of sirolimus for lymphangioleiomyomatosis remaining in the abdominopelvic region after lung transplantation

T2 - A case report

AU - Ito, T.

AU - Suno, Manabu

AU - Sakamoto, K.

AU - Yoshizaki, Y.

AU - Yamamoto, K.

AU - Nakanishi, R.

AU - Hirano, Y.

AU - Irie, M.

AU - Kurosaki, Takeshi

AU - Otani, Shinji

AU - Yamane, Masaomi

AU - Sugimoto, Seiichiro

AU - Miyoshi, K.

AU - Oto, Takahiro

PY - 2016/1/1

Y1 - 2016/1/1

N2 - Purpose Sirolimus (SRL) is used to treat pulmonary lymphangioleiomyomatosis (P-LAM). There is limited evidence that SRL has systemic efficacy for the patients with extrapulmonary lymphangioleiomyomatosis (E-LAM) remaining after lung transplantation (LT) for P-LAM. This report examines the efficacy of SRL treatment for the patient with E-LAM remaining after an LT for P-LAM. Case Summary The course of the patient's recovery from an LT for P-LAM was complicated by lymphedema in the left femoral region that was caused by two E-LAM lesions remaining in the left pelvic cavity and in the retroperitoneal area. After the LT was performed, the patient started SRL treatment to reduce the E-LAM lesions. The daily SRL dose, selected based on the standard SRL dose for P-LAM, was initiated at 1 mg/d and was maintained at 2 mg/d. The remaining E-LAM lesions and lymphedema in the left femoral region improved in approximately 9 months after the LT with the administration of both SRL and the standard immunosuppressive therapy used by Okayama University Hospital, including tacrolimus, mycophenolate mofetil, and prednisolone. The SRL and tacrolimus trough concentrations in whole blood were maintained within the therapeutic window for the next 1.5 years after initiation of SRL treatment. The patient experienced no severe adverse events that required discontinuation of the SRL treatment during this time. Conclusion The patients with remaining E-LAM lesions may receive SRL treatment to improve the quality of life after LT for P-LAM as effective therapy in cases where the patient's recovery is complicated by E-LAM lesions.

AB - Purpose Sirolimus (SRL) is used to treat pulmonary lymphangioleiomyomatosis (P-LAM). There is limited evidence that SRL has systemic efficacy for the patients with extrapulmonary lymphangioleiomyomatosis (E-LAM) remaining after lung transplantation (LT) for P-LAM. This report examines the efficacy of SRL treatment for the patient with E-LAM remaining after an LT for P-LAM. Case Summary The course of the patient's recovery from an LT for P-LAM was complicated by lymphedema in the left femoral region that was caused by two E-LAM lesions remaining in the left pelvic cavity and in the retroperitoneal area. After the LT was performed, the patient started SRL treatment to reduce the E-LAM lesions. The daily SRL dose, selected based on the standard SRL dose for P-LAM, was initiated at 1 mg/d and was maintained at 2 mg/d. The remaining E-LAM lesions and lymphedema in the left femoral region improved in approximately 9 months after the LT with the administration of both SRL and the standard immunosuppressive therapy used by Okayama University Hospital, including tacrolimus, mycophenolate mofetil, and prednisolone. The SRL and tacrolimus trough concentrations in whole blood were maintained within the therapeutic window for the next 1.5 years after initiation of SRL treatment. The patient experienced no severe adverse events that required discontinuation of the SRL treatment during this time. Conclusion The patients with remaining E-LAM lesions may receive SRL treatment to improve the quality of life after LT for P-LAM as effective therapy in cases where the patient's recovery is complicated by E-LAM lesions.

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