Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels

Janet Crane; Jackie Manfredo; Elisa Boscolo; Mara Coyan; Clifford Takemoto; Maxim Itkin; Denise M. Adams; Timothy D. Le Cras

doi:10.1002/pbc.28529

Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels

Janet Crane, Jackie Manfredo, Elisa Boscolo, Mara Coyan, Clifford Takemoto, Maxim Itkin, Denise M. Adams, Timothy D. Le Cras

School of Medicine

Research output: Contribution to journal › Article › peer-review

2 Scopus citations

Abstract

Kaposiform lymphangiomatosis (KLA) is a rare, life-threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin-2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status. We report a 7-year-old male child with easy bruising, inguinal swelling, and consumptive coagulopathy, diagnosed with KLA. A multimodal treatment regimen of prednisone, sirolimus, vincristine, and adjunctive zoledronate was used. Plasma ANG2 levels were highly elevated at diagnosis but decreased during treatment. The patient showed significant clinical improvement over a 38-month period and normalization of ANG2 levels correlated with resolution of the coagulopathy.

Original language	English (US)
Article number	e28529
Journal	Pediatric Blood and Cancer
Volume	67
Issue number	9
DOIs	https://doi.org/10.1002/pbc.28529
State	Published - Sep 1 2020

Keywords

angiogenesis
vascular malformations
vincristine

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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10.1002/pbc.28529

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title = "Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels",

abstract = "Kaposiform lymphangiomatosis (KLA) is a rare, life-threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin-2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status. We report a 7-year-old male child with easy bruising, inguinal swelling, and consumptive coagulopathy, diagnosed with KLA. A multimodal treatment regimen of prednisone, sirolimus, vincristine, and adjunctive zoledronate was used. Plasma ANG2 levels were highly elevated at diagnosis but decreased during treatment. The patient showed significant clinical improvement over a 38-month period and normalization of ANG2 levels correlated with resolution of the coagulopathy.",

keywords = "angiogenesis, vascular malformations, vincristine",

author = "Janet Crane and Jackie Manfredo and Elisa Boscolo and Mara Coyan and Clifford Takemoto and Maxim Itkin and Adams, {Denise M.} and {Le Cras}, {Timothy D.}",

note = "Funding Information: The authors would like to thank the patient and the patient's family for their consent to participate in the study and supporting publication of this report. The proactive approach by the family in seeking out interdisciplinary expertise led to this synergistic collaboration between physicians and scientists at five major medical centers. They also thank Tricia Pastura for assistance with the biomarker assays. Funding from the Lymphatic Malformation Institute is also acknowledged. Informed consent was obtained from the patient and family to use photographs of the patient. Publisher Copyright: {\textcopyright} 2020 Wiley Periodicals, Inc.",

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AU - Crane, Janet

AU - Manfredo, Jackie

AU - Boscolo, Elisa

AU - Coyan, Mara

AU - Takemoto, Clifford

AU - Itkin, Maxim

AU - Adams, Denise M.

AU - Le Cras, Timothy D.

N1 - Funding Information: The authors would like to thank the patient and the patient's family for their consent to participate in the study and supporting publication of this report. The proactive approach by the family in seeking out interdisciplinary expertise led to this synergistic collaboration between physicians and scientists at five major medical centers. They also thank Tricia Pastura for assistance with the biomarker assays. Funding from the Lymphatic Malformation Institute is also acknowledged. Informed consent was obtained from the patient and family to use photographs of the patient. Publisher Copyright: © 2020 Wiley Periodicals, Inc.

PY - 2020/9/1

Y1 - 2020/9/1

N2 - Kaposiform lymphangiomatosis (KLA) is a rare, life-threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin-2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status. We report a 7-year-old male child with easy bruising, inguinal swelling, and consumptive coagulopathy, diagnosed with KLA. A multimodal treatment regimen of prednisone, sirolimus, vincristine, and adjunctive zoledronate was used. Plasma ANG2 levels were highly elevated at diagnosis but decreased during treatment. The patient showed significant clinical improvement over a 38-month period and normalization of ANG2 levels correlated with resolution of the coagulopathy.

AB - Kaposiform lymphangiomatosis (KLA) is a rare, life-threatening congenital lymphatic malformation. Diagnosis is often delayed due to complex indistinct symptoms. Blood angiopoietin-2 (ANG2) levels are elevated in KLA and may be useful as a biomarker to monitor disease status. We report a 7-year-old male child with easy bruising, inguinal swelling, and consumptive coagulopathy, diagnosed with KLA. A multimodal treatment regimen of prednisone, sirolimus, vincristine, and adjunctive zoledronate was used. Plasma ANG2 levels were highly elevated at diagnosis but decreased during treatment. The patient showed significant clinical improvement over a 38-month period and normalization of ANG2 levels correlated with resolution of the coagulopathy.

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KW - vascular malformations

KW - vincristine

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Kaposiform lymphangiomatosis treated with multimodal therapy improves coagulopathy and reduces blood angiopoietin-2 levels

Abstract

Keywords

ASJC Scopus subject areas

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