Long-term low-dose sirolimus therapy and successful discontinuation in an adult with kaposiform lymphangiomatosis and disseminated intravascular coagulation: a case report - Takeaways - MDSpire

Long-term low-dose sirolimus therapy and successful discontinuation in an adult with kaposiform lymphangiomatosis and disseminated intravascular coagulation: a case report

  • By

  • Junya Liu

  • Whenshuai Xu

  • Qingwei Jiang

  • Kai-Feng Xu

  • May 25, 2026

  • 0 min

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  • 1

    Kaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly often complicated by disseminated intravascular coagulation (DIC).

  • 2

    A 23-year-old male with KLA and an NRAS p.Q61R mutation was treated with low-dose sirolimus, achieving significant clinical and laboratory improvements over four years.

  • 3

    The patient maintained stable health and remained relapse-free for five years after discontinuing sirolimus, suggesting potential for treatment withdrawal.

  • 4

    Long-term low-dose sirolimus therapy effectively controlled disease progression in this adult patient with KLA, highlighting individualized treatment strategies.

  • 5

    This case emphasizes the need for further research on optimal dosing and monitoring strategies for sirolimus in managing KLA and similar conditions.

Original Source(s)

Long-term low-dose sirolimus therapy and successful discontinuation in an adult with kaposiform lymphangiomatosis and disseminated intravascular coagulation: a case report

  • by Junya Liu, Whenshuai Xu, Qingwei Jiang, Kai-Feng Xu

  • May 25, 2026

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