Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis
BACKGROUND: Langerhans cell histiocytosis is a rare disorder leading to destruction of tissues as well as causing lesions in the body. LCH can be self-limiting to disseminated life threatening. OBJECTIVES: We wanted to review data on presentation and outcome of our treatment efforts on patients with...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2022-01-01
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| Series: | Journal of Applied Hematology |
| Subjects: | |
| Online Access: | http://www.jahjournal.org/article.asp?issn=1658-5127;year=2022;volume=13;issue=4;spage=249;epage=254;aulast=AlFawaz |
| _version_ | 1811220778918608896 |
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| author | Ibrahim AlFawaz Amal Alhejaili Khawar Siddiqui Mouhab Ayas |
| author_facet | Ibrahim AlFawaz Amal Alhejaili Khawar Siddiqui Mouhab Ayas |
| author_sort | Ibrahim AlFawaz |
| collection | DOAJ |
| description | BACKGROUND: Langerhans cell histiocytosis is a rare disorder leading to destruction of tissues as well as causing lesions in the body. LCH can be self-limiting to disseminated life threatening.
OBJECTIVES: We wanted to review data on presentation and outcome of our treatment efforts on patients with LCH diagnosed and treated at our institution.
MATERIAL AND METHODS: In this retrospective study involving 75 pediatric patients with LCH diagnosed between 1998 and 2018, we evaluated clinicopathological features, laboratory findings, treatment modalities and outcome.
RESULTS: In males median age at the time of diagnosis was 2.4 years. The median follow up was 63+/- 7.9 months. The most common presenting symptom was a bone lesion-related symptom. 57.3% were above 2 years of age at presentation while 38.7% below 2 years of age. 53% of cases had multisystem disease at diagnosis. Liver found to be most common organ involved. Treatment consisted of observation, surgery or chemotherapy. Vinblastine with steroid was the most common used chemotherapy regimen. 3 patients with progressive refractory disease underwent stem cell transplantation.
CONCLUSION: Overall survival was significantly lower in patients with liver involvement. |
| first_indexed | 2024-04-12T07:48:44Z |
| format | Article |
| id | doaj.art-214c7741e3904adcbb7674430f502e20 |
| institution | Directory Open Access Journal |
| issn | 1658-5127 |
| language | English |
| last_indexed | 2024-04-12T07:48:44Z |
| publishDate | 2022-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Journal of Applied Hematology |
| spelling | doaj.art-214c7741e3904adcbb7674430f502e202022-12-22T03:41:39ZengWolters Kluwer Medknow PublicationsJournal of Applied Hematology1658-51272022-01-0113424925410.4103/joah.joah_223_20Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosisIbrahim AlFawazAmal AlhejailiKhawar SiddiquiMouhab AyasBACKGROUND: Langerhans cell histiocytosis is a rare disorder leading to destruction of tissues as well as causing lesions in the body. LCH can be self-limiting to disseminated life threatening. OBJECTIVES: We wanted to review data on presentation and outcome of our treatment efforts on patients with LCH diagnosed and treated at our institution. MATERIAL AND METHODS: In this retrospective study involving 75 pediatric patients with LCH diagnosed between 1998 and 2018, we evaluated clinicopathological features, laboratory findings, treatment modalities and outcome. RESULTS: In males median age at the time of diagnosis was 2.4 years. The median follow up was 63+/- 7.9 months. The most common presenting symptom was a bone lesion-related symptom. 57.3% were above 2 years of age at presentation while 38.7% below 2 years of age. 53% of cases had multisystem disease at diagnosis. Liver found to be most common organ involved. Treatment consisted of observation, surgery or chemotherapy. Vinblastine with steroid was the most common used chemotherapy regimen. 3 patients with progressive refractory disease underwent stem cell transplantation. CONCLUSION: Overall survival was significantly lower in patients with liver involvement.http://www.jahjournal.org/article.asp?issn=1658-5127;year=2022;volume=13;issue=4;spage=249;epage=254;aulast=AlFawazlangerhans cell histiocytosispediatricstem cell transplantation |
| spellingShingle | Ibrahim AlFawaz Amal Alhejaili Khawar Siddiqui Mouhab Ayas Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis Journal of Applied Hematology langerhans cell histiocytosis pediatric stem cell transplantation |
| title | Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis |
| title_full | Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis |
| title_fullStr | Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis |
| title_full_unstemmed | Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis |
| title_short | Stem cell transplantation: A promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis |
| title_sort | stem cell transplantation a promising approach for pediatric patients with refractory multistage langerhans cell histiocytosis |
| topic | langerhans cell histiocytosis pediatric stem cell transplantation |
| url | http://www.jahjournal.org/article.asp?issn=1658-5127;year=2022;volume=13;issue=4;spage=249;epage=254;aulast=AlFawaz |
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