Refractory kaposiform lymphangiomatosis relieved by splenectomy
IntroductionKaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly with a poor prognosis. There is no standard treatment, and drug therapies are the most common therapeutic method. However, some patients' symptoms become gradually aggravated despite medical treatment. Splene...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2023-08-01
|
| Series: | Frontiers in Pediatrics |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2023.1203336/full |
| _version_ | 1797741390998798336 |
|---|---|
| author | Yuru Lan Jiangyuan Zhou Tong Qiu Xue Gong Yi Ji |
| author_facet | Yuru Lan Jiangyuan Zhou Tong Qiu Xue Gong Yi Ji |
| author_sort | Yuru Lan |
| collection | DOAJ |
| description | IntroductionKaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly with a poor prognosis. There is no standard treatment, and drug therapies are the most common therapeutic method. However, some patients' symptoms become gradually aggravated despite medical treatment. Splenectomy may be an alternative option when pharmacological therapies are ineffective.Materials and MethodsWe reviewed and evaluated the cases of 3 patients with KLA who ultimately underwent splenectomy. Results: The lesions were diffusely distributed and involved the lungs and spleens of the 3 patients. Laboratory examinations revealed that all three patients had thrombocytopenia and reduced fibrinogen levels. All patients underwent symptomatic splenectomy after the medication failed. Surprisingly, their symptoms greatly improved. Histopathological investigation of the splenic lesions of the three patients confirmed the diagnosis of KLA. Immunohistochemical staining showed positivity for CD31, CD34, podoplanin, Prox-1 and angiopoietin 2 (Ang-2).DiscussionThis study aimed to review the features of KLA patients treated by splenectomy and explore the underlying link between splenectomy and prognosis. The reason for the improvement after splenectomy may be related to increased Ang-2 levels and platelet activation in patients with KLA. Future research should seek to develop more targeted drugs based on molecular findings, which may give new hope for the treatment of KLA. |
| first_indexed | 2024-03-12T14:26:01Z |
| format | Article |
| id | doaj.art-1f4379cb2e464552906afb467beb7344 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-03-12T14:26:01Z |
| publishDate | 2023-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-1f4379cb2e464552906afb467beb73442023-08-18T04:56:56ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-08-011110.3389/fped.2023.12033361203336Refractory kaposiform lymphangiomatosis relieved by splenectomyYuru LanJiangyuan ZhouTong QiuXue GongYi JiIntroductionKaposiform lymphangiomatosis (KLA) is a rare and complex lymphatic anomaly with a poor prognosis. There is no standard treatment, and drug therapies are the most common therapeutic method. However, some patients' symptoms become gradually aggravated despite medical treatment. Splenectomy may be an alternative option when pharmacological therapies are ineffective.Materials and MethodsWe reviewed and evaluated the cases of 3 patients with KLA who ultimately underwent splenectomy. Results: The lesions were diffusely distributed and involved the lungs and spleens of the 3 patients. Laboratory examinations revealed that all three patients had thrombocytopenia and reduced fibrinogen levels. All patients underwent symptomatic splenectomy after the medication failed. Surprisingly, their symptoms greatly improved. Histopathological investigation of the splenic lesions of the three patients confirmed the diagnosis of KLA. Immunohistochemical staining showed positivity for CD31, CD34, podoplanin, Prox-1 and angiopoietin 2 (Ang-2).DiscussionThis study aimed to review the features of KLA patients treated by splenectomy and explore the underlying link between splenectomy and prognosis. The reason for the improvement after splenectomy may be related to increased Ang-2 levels and platelet activation in patients with KLA. Future research should seek to develop more targeted drugs based on molecular findings, which may give new hope for the treatment of KLA.https://www.frontiersin.org/articles/10.3389/fped.2023.1203336/fullkaposiform lymphangiomatosissplenectomyangiopoietin 2plateletangiopoietin 1 |
| spellingShingle | Yuru Lan Jiangyuan Zhou Tong Qiu Xue Gong Yi Ji Refractory kaposiform lymphangiomatosis relieved by splenectomy Frontiers in Pediatrics kaposiform lymphangiomatosis splenectomy angiopoietin 2 platelet angiopoietin 1 |
| title | Refractory kaposiform lymphangiomatosis relieved by splenectomy |
| title_full | Refractory kaposiform lymphangiomatosis relieved by splenectomy |
| title_fullStr | Refractory kaposiform lymphangiomatosis relieved by splenectomy |
| title_full_unstemmed | Refractory kaposiform lymphangiomatosis relieved by splenectomy |
| title_short | Refractory kaposiform lymphangiomatosis relieved by splenectomy |
| title_sort | refractory kaposiform lymphangiomatosis relieved by splenectomy |
| topic | kaposiform lymphangiomatosis splenectomy angiopoietin 2 platelet angiopoietin 1 |
| url | https://www.frontiersin.org/articles/10.3389/fped.2023.1203336/full |
| work_keys_str_mv | AT yurulan refractorykaposiformlymphangiomatosisrelievedbysplenectomy AT jiangyuanzhou refractorykaposiformlymphangiomatosisrelievedbysplenectomy AT tongqiu refractorykaposiformlymphangiomatosisrelievedbysplenectomy AT xuegong refractorykaposiformlymphangiomatosisrelievedbysplenectomy AT yiji refractorykaposiformlymphangiomatosisrelievedbysplenectomy |