Clinical variability in multifocal lymphangioendotheliomatosis with thrombocytopenia: a review of the literature

Manor, Joshua; Patel, Kalyani R.; Iacobas, Ionela; Margolin, Judith F.; Mahajan, Priya

doi:10.1080/08880018.2020.1871135

Cited by 9 publications

(30 citation statements)

References 32 publications

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“…It might be complicated with thrombocytopenia refractory to blood cell transfusions and life-threatening bleeding, with a subsequent mortality rate of 65% in newborns [ 24 , 27 ]. As this is a newly recognized entity, which was firstly described by North et al, there is no consensus on the proper therapy [ 25 , 28 ]. The rapamycin inhibitors such as sirolimus, combination of vincristine and prednisolone, and association of aminocaproic acid, octreotide drip, and corticosteroids were used in the cases reported in literature [ 25 – 28 ].…”

Section: Vascular Hamartomasmentioning

confidence: 99%

“…As this is a newly recognized entity, which was firstly described by North et al, there is no consensus on the proper therapy [ 25 , 28 ]. The rapamycin inhibitors such as sirolimus, combination of vincristine and prednisolone, and association of aminocaproic acid, octreotide drip, and corticosteroids were used in the cases reported in literature [ 25 – 28 ]. In refractory cases, the antiangiogenic therapy with bevacizumab was suggested to be used [ 27 ].…”

Section: Vascular Hamartomasmentioning

confidence: 99%

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Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Gurzu

Jung

2022

Journal of Oncology

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Purpose. To perform the first systematic report about histological subtypes of nonpolypous hamartomas of the gastrointestinal (GI) tract, from esophagus to anal canal. Design. From over 19,000 studies about hamartomas, most of them published as case series or case presentations, we have selected the most representative ones for the GI tract, excluding polyposis syndromes. To have a whole picture of these hamartomas, all of the data were combined with the personal experience of the authors who are GI pathologists. Results. The examined articles showed predominance of vascular and combined vascular and mesenchymal hamartomas. Arteriovenous hamartomas or Brunner gland hamartomas are mainly diagnosed in the small intestine, with preponderance for duodenum. Other malformations such cavernous hamartomas are more specific for the colorectal segments, whereas chondromatous hamartomas or those derived from the neural ectoderm were mostly reported in the esophagus. As newly recognized entities were admitted in the last years, misdiagnosis is frequent, and the best therapeutic approach is far to be known. Conclusion. Even rare, hamartomas of the GI tract need to be differentiated from tumors and familial polyposis syndromes. Knowing their proper denominations and possible complications is valuable for gastroenterologists, pathologists, and surgeons, to be aware in the differential diagnosis.

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Section: Vascular Hamartomasmentioning

confidence: 99%

Section: Vascular Hamartomasmentioning

confidence: 99%

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Gurzu

Jung

2022

Journal of Oncology

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“…Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a rare disease that was first described in 2004 1. MLT is characterized by multiple cutaneous lesions and bleeding of the gastrointestinal (GI) tract with thrombocytopenia 2. Although no standard diagnostic criteria exist,2 the diagnosis is commonly made based on the clinical phenotypes and histopathological tests 3–7.…”

mentioning

confidence: 99%

“…Although no standard diagnostic criteria exist,2 the diagnosis is commonly made based on the clinical phenotypes and histopathological tests 3–7. To date, ~30 MLT cases have been reported in the literature 2. Although various phenotypes had been described,2 massive bleeding from the GI tract can be life-threatening and the major cause of mortality in the neonatal period 6,7…”

mentioning

confidence: 99%

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Successful Treatment With Everolimus for Multifocal Lymphangioendotheliomatosis With Thrombocytopenia in an Infant

Hoshino¹,

Arai²,

Kato

et al. 2022

Journal of Pediatric Hematology/Oncology

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Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a rare disease characterized by the presence of multiple cutaneous lesions and bleeding from the gastrointestinal tract with thrombocytopenia. Because of the varied phenotypes and rarity of MLT, a treatment strategy has not been standardized thus far. We describe a case of infantile MLT that did not respond to treatment with propranolol, prednisolone, or vincristine. We successfully treated the patient with everolimus, an inhibitor of the mammalian target of rapamycin. Our case provides the first evidence of the effectiveness of everolimus for the treatment of MLT.

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Multifocal lymphangioendotheliomatosis with thrombocytopenia: Report of a case with favorable outcome into adulthood

Kerrouch,

Gouillon,

Duclaux-Loras

et al. 2024

JAAD Case Reports

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Clinical variability in multifocal lymphangioendotheliomatosis with thrombocytopenia: a review of the literature

Cited by 9 publications

References 32 publications

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

Successful Treatment With Everolimus for Multifocal Lymphangioendotheliomatosis With Thrombocytopenia in an Infant

Multifocal lymphangioendotheliomatosis with thrombocytopenia: Report of a case with favorable outcome into adulthood

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