Kasabach-Merritt Phenomenon: Diagnosis, Management, and Outcome on Two Cases in a Sub-Saharan Country (Senegal)
Introduction: Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors. In sub-Saharan Africa, KMP-related data are scarce and it poses significant challenges in management, p...
Gespeichert in:
| Veröffentlicht in: | Case reports in dermatology Jg. 17; H. 1; S. 174 - 180 |
|---|---|
| Hauptverfasser: | , , , , , , , , , |
| Format: | Journal Article |
| Sprache: | Englisch |
| Veröffentlicht: |
Switzerland
S. Karger AG
21.04.2025
|
| Schlagworte: | |
| ISSN: | 1662-6567, 1662-6567 |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| Abstract | Introduction: Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors. In sub-Saharan Africa, KMP-related data are scarce and it poses significant challenges in management, particularly due to limited availability of treatment resources. We report 2 cases of KMP observed in a sub-Saharan Africa country (Dakar, Senegal). Case Presentation: Case 1: a 45-day-old male infant was admitted for a tumor lesion located in the axillary fold that has been evolving since birth. The lesion had rapidly become aggressive, inflammatory, and purpuric. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a kaposiform hemangioendothelioma complicated by a KMP was retained. Treatment with betamethasone was initiated, but death occurred 6 days later secondary to cerebral hemorrhage. Case 2: a 2-month-old female infant was admitted for a rapidly aggressive, inflammatory, infiltrating tumor lesion on the face that had been progressing for 1 month. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a KMP was retained. Treatment with corticosteroids, then combined with vincristine, was administered. The outcome was favorable with a considerable regression of the mass and improvement in biological parameters after 6 months. Conclusion: To our knowledge, we report the first case report on KMP in sub-Saharan Africa, particularly in pediatric dermatology in Dakar. Besides their rarity, these cases highlight the challenges in the management of KMP in a country with limited therapeutic resources. |
|---|---|
| AbstractList | Introduction: Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors. In sub-Saharan Africa, KMP-related data are scarce and it poses significant challenges in management, particularly due to limited availability of treatment resources. We report 2 cases of KMP observed in a sub-Saharan Africa country (Dakar, Senegal). Case Presentation: Case 1: a 45-day-old male infant was admitted for a tumor lesion located in the axillary fold that has been evolving since birth. The lesion had rapidly become aggressive, inflammatory, and purpuric. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a kaposiform hemangioendothelioma complicated by a KMP was retained. Treatment with betamethasone was initiated, but death occurred 6 days later secondary to cerebral hemorrhage. Case 2: a 2-month-old female infant was admitted for a rapidly aggressive, inflammatory, infiltrating tumor lesion on the face that had been progressing for 1 month. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a KMP was retained. Treatment with corticosteroids, then combined with vincristine, was administered. The outcome was favorable with a considerable regression of the mass and improvement in biological parameters after 6 months. Conclusion: To our knowledge, we report the first case report on KMP in sub-Saharan Africa, particularly in pediatric dermatology in Dakar. Besides their rarity, these cases highlight the challenges in the management of KMP in a country with limited therapeutic resources. Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors. In sub-Saharan Africa, KMP-related data are scarce and it poses significant challenges in management, particularly due to limited availability of treatment resources. We report 2 cases of KMP observed in a sub-Saharan Africa country (Dakar, Senegal).IntroductionKasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors. In sub-Saharan Africa, KMP-related data are scarce and it poses significant challenges in management, particularly due to limited availability of treatment resources. We report 2 cases of KMP observed in a sub-Saharan Africa country (Dakar, Senegal).Case 1: a 45-day-old male infant was admitted for a tumor lesion located in the axillary fold that has been evolving since birth. The lesion had rapidly become aggressive, inflammatory, and purpuric. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a kaposiform hemangioendothelioma complicated by a KMP was retained. Treatment with betamethasone was initiated, but death occurred 6 days later secondary to cerebral hemorrhage. Case 2: a 2-month-old female infant was admitted for a rapidly aggressive, inflammatory, infiltrating tumor lesion on the face that had been progressing for 1 month. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a KMP was retained. Treatment with corticosteroids, then combined with vincristine, was administered. The outcome was favorable with a considerable regression of the mass and improvement in biological parameters after 6 months.Case PresentationCase 1: a 45-day-old male infant was admitted for a tumor lesion located in the axillary fold that has been evolving since birth. The lesion had rapidly become aggressive, inflammatory, and purpuric. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a kaposiform hemangioendothelioma complicated by a KMP was retained. Treatment with betamethasone was initiated, but death occurred 6 days later secondary to cerebral hemorrhage. Case 2: a 2-month-old female infant was admitted for a rapidly aggressive, inflammatory, infiltrating tumor lesion on the face that had been progressing for 1 month. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a KMP was retained. Treatment with corticosteroids, then combined with vincristine, was administered. The outcome was favorable with a considerable regression of the mass and improvement in biological parameters after 6 months.To our knowledge, we report the first case report on KMP in sub-Saharan Africa, particularly in pediatric dermatology in Dakar. Besides their rarity, these cases highlight the challenges in the management of KMP in a country with limited therapeutic resources.ConclusionTo our knowledge, we report the first case report on KMP in sub-Saharan Africa, particularly in pediatric dermatology in Dakar. Besides their rarity, these cases highlight the challenges in the management of KMP in a country with limited therapeutic resources. Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors. In sub-Saharan Africa, KMP-related data are scarce and it poses significant challenges in management, particularly due to limited availability of treatment resources. We report 2 cases of KMP observed in a sub-Saharan Africa country (Dakar, Senegal). Case 1: a 45-day-old male infant was admitted for a tumor lesion located in the axillary fold that has been evolving since birth. The lesion had rapidly become aggressive, inflammatory, and purpuric. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a kaposiform hemangioendothelioma complicated by a KMP was retained. Treatment with betamethasone was initiated, but death occurred 6 days later secondary to cerebral hemorrhage. Case 2: a 2-month-old female infant was admitted for a rapidly aggressive, inflammatory, infiltrating tumor lesion on the face that had been progressing for 1 month. Blood tests showed normocytic normochromic anemia with severe thrombocytopenia and high D-dimer levels. The diagnosis of a KMP was retained. Treatment with corticosteroids, then combined with vincristine, was administered. The outcome was favorable with a considerable regression of the mass and improvement in biological parameters after 6 months. To our knowledge, we report the first case report on KMP in sub-Saharan Africa, particularly in pediatric dermatology in Dakar. Besides their rarity, these cases highlight the challenges in the management of KMP in a country with limited therapeutic resources. |
| Author | Tall, Fatime Ndiaye, Maodo Diop, Ibrahima Ba, Idrissa Demba Ndiaye Diop, Mame Téné Mejri, Ines Ndiaye, Ousmane Diassé, Fatou Diop, Khadim Yassine, Lana |
| Author_xml | – sequence: 1 givenname: Khadim surname: Diop fullname: Diop, Khadim – sequence: 2 givenname: Mame Téné surname: Ndiaye Diop fullname: Ndiaye Diop, Mame Téné – sequence: 3 givenname: Ibrahima surname: Diop fullname: Diop, Ibrahima – sequence: 4 givenname: Ines surname: Mejri fullname: Mejri, Ines – sequence: 5 givenname: Lana surname: Yassine fullname: Yassine, Lana – sequence: 6 givenname: Fatou surname: Diassé fullname: Diassé, Fatou – sequence: 7 givenname: Fatime surname: Tall fullname: Tall, Fatime – sequence: 8 givenname: Idrissa Demba surname: Ba fullname: Ba, Idrissa Demba – sequence: 9 givenname: Maodo surname: Ndiaye fullname: Ndiaye, Maodo – sequence: 10 givenname: Ousmane surname: Ndiaye fullname: Ndiaye, Ousmane |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/40438153$$D View this record in MEDLINE/PubMed |
| BookMark | eNpVkUtv1DAQxy1URB9w4AsgH1upgfEzDRdULQWqtirS7t2aJOPdoKxd7KSo375GLatyGM1o5qf_vA7ZXoiBGHsv4KMQpvkEAEZbkPCKHQhrZWWNrfdexPvsMOdfALYxVr1h-xq0OhNGHbB4hRlb7DbVDaU0TBP_uaEQt8XCZ_51wHWIecin_AYDrqnkp1OOoee389QVjMfAV38iX2CmzIfAkS_ntlriBhMGvohzmNIDP15SoDWOJ2_Za49jpnfP_oitvl2sFj-q69vvl4vz66qTRkHlfa9AmOJaL31vRWsMkkbvkRD7sq31pq1JAFnfAGotGyVrLbzSDUh1xL48yd7N7Zb6roydcHR3adhienARB_d_JQwbt473TkghGqhFUTh-Vkjx90x5ctshdzSOGCjO2SkplIWz2uqCfnjZbNfl35ULcPIEdCnmnMjvEAHu7wfd7oPqEeHUjQQ |
| Cites_doi | 10.1186/s13023-020-1320-1 10.1007/s12519-020-00379-9 10.47391/JPMA.9185 10.3389/fped.2021.788120 10.3389/fped.2022.949950 10.3857/roj.2021.00983 10.1002/ccr3.7859 10.1016/j.jvs.2019.06.217 10.1002/pbc.30779 10.7499/j.issn.1008-8830.2103146 10.1007/s12098-021-03817-2 10.1136/bcr-2020-235065 10.1080/08880018.2023.2300485 10.3892/etm.2022.11558 10.1016/j.jpeds.2012.06.044 |
| ContentType | Journal Article |
| Copyright | 2025 The Author(s). Published by S. Karger AG, Basel. 2025 The Author(s). Published by S. Karger AG, Basel 2025 |
| Copyright_xml | – notice: 2025 The Author(s). Published by S. Karger AG, Basel. – notice: 2025 The Author(s). Published by S. Karger AG, Basel 2025 |
| DBID | AAYXX CITATION NPM 7X8 5PM |
| DOI | 10.1159/000546020 |
| DatabaseName | CrossRef PubMed MEDLINE - Academic PubMed Central (Full Participant titles) |
| DatabaseTitle | CrossRef PubMed MEDLINE - Academic |
| DatabaseTitleList | CrossRef MEDLINE - Academic PubMed |
| Database_xml | – sequence: 1 dbid: NPM name: PubMed url: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 2 dbid: 7X8 name: MEDLINE - Academic url: https://search.proquest.com/medline sourceTypes: Aggregation Database |
| DeliveryMethod | fulltext_linktorsrc |
| Discipline | Medicine |
| DocumentTitleAlternate | Kasabach-Merritt Phenomenon |
| EISSN | 1662-6567 |
| EndPage | 180 |
| ExternalDocumentID | PMC12119071 40438153 10_1159_000546020 |
| Genre | Journal Article Case Reports |
| GroupedDBID | --- 0~B 3O. 4.4 53G 5VS 6J9 7X7 8FI 8FJ AAYXX ABBTS ABDBF ABPAZ ABUWG ABWCG ACGFS ACUHS ADBBV AEGXH AEYAO AFKRA AHFRZ AHMBA ALMA_UNASSIGNED_HOLDINGS AOIJS AZPMC BAWUL BCNDV BENPR BPHCQ BVXVI CCPQU CITATION DIK E0A EBS F5P FB. FYUFA GROUPED_DOAJ GX1 HMCUK HYE IAO IHR ITC KQ8 M-- M~E O5R O5S OK1 PHGZM PQQKQ PROAC RNS RPM TR2 UKHRP ADRAZ ALIPV C1A CAG COF CYUIP EJD IPNFZ M48 NPM RIG 7X8 5PM |
| ID | FETCH-LOGICAL-c2530-ffd3015ffdbf2fd61b55ae4affaeaad5466f5b7e10e6f90a442932741f349023 |
| ISSN | 1662-6567 |
| IngestDate | Tue Sep 30 17:04:40 EDT 2025 Thu Oct 02 22:42:57 EDT 2025 Sun Jun 01 01:35:25 EDT 2025 Sat Nov 29 07:49:19 EST 2025 |
| IsDoiOpenAccess | true |
| IsOpenAccess | true |
| IsPeerReviewed | true |
| IsScholarly | true |
| Issue | 1 |
| Keywords | Kasabach-Merritt phenomenon Vascular tumors Limited resources Sub-Saharan Africa Pediatric dermatology |
| Language | English |
| License | https://creativecommons.org/licenses/by-nc/4.0 2025 The Author(s). Published by S. Karger AG, Basel. This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission. |
| LinkModel | OpenURL |
| MergedId | FETCHMERGED-LOGICAL-c2530-ffd3015ffdbf2fd61b55ae4affaeaad5466f5b7e10e6f90a442932741f349023 |
| Notes | ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 |
| OpenAccessLink | http://dx.doi.org/10.1159/000546020 |
| PMID | 40438153 |
| PQID | 3213608764 |
| PQPubID | 23479 |
| PageCount | 7 |
| ParticipantIDs | pubmedcentral_primary_oai_pubmedcentral_nih_gov_12119071 proquest_miscellaneous_3213608764 pubmed_primary_40438153 crossref_primary_10_1159_000546020 |
| PublicationCentury | 2000 |
| PublicationDate | 2025-04-21 |
| PublicationDateYYYYMMDD | 2025-04-21 |
| PublicationDate_xml | – month: 04 year: 2025 text: 2025-04-21 day: 21 |
| PublicationDecade | 2020 |
| PublicationPlace | Switzerland |
| PublicationPlace_xml | – name: Switzerland – name: Basel, Switzerland |
| PublicationTitle | Case reports in dermatology |
| PublicationTitleAlternate | Case Rep Dermatol |
| PublicationYear | 2025 |
| Publisher | S. Karger AG |
| Publisher_xml | – name: S. Karger AG |
| References | ref13 ref12 ref15 ref14 ref11 ref10 ref2 ref1 ref8 ref7 ref9 ref4 ref3 ref6 ref5 |
| References_xml | – ident: ref8 doi: 10.1186/s13023-020-1320-1 – ident: ref6 doi: 10.1007/s12519-020-00379-9 – ident: ref9 doi: 10.47391/JPMA.9185 – ident: ref12 doi: 10.3389/fped.2021.788120 – ident: ref7 doi: 10.3389/fped.2022.949950 – ident: ref4 doi: 10.3857/roj.2021.00983 – ident: ref11 doi: 10.1002/ccr3.7859 – ident: ref13 doi: 10.1016/j.jvs.2019.06.217 – ident: ref14 doi: 10.1002/pbc.30779 – ident: ref10 doi: 10.7499/j.issn.1008-8830.2103146 – ident: ref2 doi: 10.1007/s12098-021-03817-2 – ident: ref1 doi: 10.1136/bcr-2020-235065 – ident: ref15 doi: 10.1080/08880018.2023.2300485 – ident: ref5 doi: 10.3892/etm.2022.11558 – ident: ref3 doi: 10.1016/j.jpeds.2012.06.044 |
| SSID | ssj0069563 |
| Score | 2.3166614 |
| Snippet | Introduction: Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive... Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease, characterized by the profound thrombocytopenia and consumptive coagulopathy... |
| SourceID | pubmedcentral proquest pubmed crossref |
| SourceType | Open Access Repository Aggregation Database Index Database |
| StartPage | 174 |
| SubjectTerms | Case Series |
| Title | Kasabach-Merritt Phenomenon: Diagnosis, Management, and Outcome on Two Cases in a Sub-Saharan Country (Senegal) |
| URI | https://www.ncbi.nlm.nih.gov/pubmed/40438153 https://www.proquest.com/docview/3213608764 https://pubmed.ncbi.nlm.nih.gov/PMC12119071 |
| Volume | 17 |
| hasFullText | 1 |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| journalDatabaseRights | – providerCode: PRVAON databaseName: DOAJ Directory of Open Access Journals customDbUrl: eissn: 1662-6567 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0069563 issn: 1662-6567 databaseCode: DOA dateStart: 20090101 isFulltext: true titleUrlDefault: https://www.doaj.org/ providerName: Directory of Open Access Journals – providerCode: PRVHPJ databaseName: ROAD: Directory of Open Access Scholarly Resources customDbUrl: eissn: 1662-6567 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0069563 issn: 1662-6567 databaseCode: M~E dateStart: 20090101 isFulltext: true titleUrlDefault: https://road.issn.org providerName: ISSN International Centre – providerCode: PRVKGR databaseName: Karger Open Access Journals customDbUrl: eissn: 1662-6567 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0069563 issn: 1662-6567 databaseCode: M-- dateStart: 20090101 isFulltext: true titleUrlDefault: https://www.karger.com/OpenAccess providerName: Karger AG – providerCode: PRVPQU databaseName: AUTh Library subscriptions: ProQuest Central customDbUrl: eissn: 1662-6567 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0069563 issn: 1662-6567 databaseCode: BENPR dateStart: 20170101 isFulltext: true titleUrlDefault: https://www.proquest.com/central providerName: ProQuest – providerCode: PRVPQU databaseName: Health Medical collection customDbUrl: eissn: 1662-6567 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0069563 issn: 1662-6567 databaseCode: 7X7 dateStart: 20170101 isFulltext: true titleUrlDefault: https://search.proquest.com/healthcomplete providerName: ProQuest |
| link | http://cvtisr.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwtV1Jj9MwFLY6MwhxQeyUpTKIA6gTETuxE3ODmSJGQ0vF9NBb5SQOCYJ01KbDcOG_8c94trN04TAcuCSVU0eW35e32O99RuhFnEiVejFx4oBAgBJS3xHSd52UB0qb_4hGplD4YzAahdOpGHc6v-tamItvQVGEl5fi_L-KGtpA2Lp09h_E3bwUGuA3CB2uIHa4Xknwp3IpIxlnzlCTLpZlf5ypQvMs2CSOY5tbZ6kF2uSXOovz06qEQSm9hzD5Me8fgZEzGbNSqxjnTGp-58JUspcLkyhwBsryiz41QKz7ubpfsx9hEm61a7yxhH-cz83ZeKeZTPLvzaI04PWn6tcPhxLGMrG7-YW9bfc_gXg_y1vzMlRfbfX8SaE2FjUo0_sztlK60sOcUwdcTWuL1V_aauUd7IDUamJiD_-pjDqxx0Xt2gsmbIIl87lL3dYo1okAW7ayyWA0sRMTs6brHjqgARNasQ5_DWpngEP4aWo86rFX5FbQ9XXTddMl2olzttN11_yfyS10swpc8FsLuNuoo4o76PqwSs24i-bbuMMt7t7gBnWHuMXcIQbE4QpxeF5gQBw2iMN5gSVeQxyuEIdfVnh7dQ9N3g8mRx-c6jQPJ6bMg68_TcCYMLhFKU0TTiLGpPJlmkolZQJTwVMWgYZwFU-FK33wlDxNrpR6vgDP8j7ahwGrhwirJCIxT0KXJ9QXMRGEJVFMiAyEEjShXfS8ns_ZueVsme3Iq4ue1TM9A42qt8lkoear5cyjxOOaqdHvogd25pvX-IYSj3ldFG7IpPmDZmvffFLkmWFtN1yK4NA_usroHqMb7WfxBO2Xi5V6iq7FF2W-XPTQXjANzDXsoYN3g9H4c88sJvUM-P4ATua0aw |
| linkProvider | ISSN International Centre |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Kasabach-Merritt+Phenomenon%3A+Diagnosis%2C+Management%2C+and+Outcome+on+Two+Cases+in+a+Sub-Saharan+Country+%28Senegal%29&rft.jtitle=Case+reports+in+dermatology&rft.au=Diop%2C+Khadim&rft.au=Ndiaye+Diop%2C+Mame+T%C3%A9n%C3%A9&rft.au=Diop%2C+Ibrahima&rft.au=Mejri%2C+Ines&rft.date=2025-04-21&rft.issn=1662-6567&rft.eissn=1662-6567&rft.volume=17&rft.issue=1&rft.spage=174&rft.epage=180&rft_id=info:doi/10.1159%2F000546020&rft.externalDBID=n%2Fa&rft.externalDocID=10_1159_000546020 |
| thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=1662-6567&client=summon |
| thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=1662-6567&client=summon |
| thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=1662-6567&client=summon |