Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review
Abstract Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder classically presenting with cytopenia and recurrent infections but atypical manifestations such as bone lesions, skin lesions and effusion have been described. We report here an unusual meningeal localization in a 33 years old...
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| Format: | Article |
| Language: | English |
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Wiley
2024-02-01
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| Series: | eJHaem |
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| Online Access: | https://doi.org/10.1002/jha2.841 |
| _version_ | 1797296731316027392 |
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| author | Fabien Claves Sylvain Carras Barbara Burroni Elsa Maitre Jean Boutonnat Xavier Troussard Lysiane Molina |
| author_facet | Fabien Claves Sylvain Carras Barbara Burroni Elsa Maitre Jean Boutonnat Xavier Troussard Lysiane Molina |
| author_sort | Fabien Claves |
| collection | DOAJ |
| description | Abstract Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder classically presenting with cytopenia and recurrent infections but atypical manifestations such as bone lesions, skin lesions and effusion have been described. We report here an unusual meningeal localization in a 33 years old man who presented with headache, hand paresthesia and visual symptoms. Brain magnetic resonance imaging revealed an occipital meningeal lesion. Diagnostic explorations led to the diagnosis of classical HCL with meningeal localization. After treatment by cladribine and rituximab the patient rapidly improved and is still in complete remission 12 months after end of treatment. The literature review identified 9 other cases of HCL with central nervous system localization (CNS) presenting with brain parenchyma and/or meninges localization. Four out of 9 patients presented with hyperleukocytosis. Most patients experienced good responses with various treatments. Cladribine alone or with rituximab led to complete responses similar to our patient. In our patient, molecular biology revealed KLF2 mutations, which implication in the atypical localization could be suspected but would need dedicated studies. In conclusion, CNS localizations of HCL are rare but can be observed and treatment with cladribine alone or with rituximab appears as an effective strategy. |
| first_indexed | 2024-03-07T22:09:02Z |
| format | Article |
| id | doaj.art-548ebd1e0d9840d4ba5f7a287c626a2b |
| institution | Directory Open Access Journal |
| issn | 2688-6146 |
| language | English |
| last_indexed | 2024-03-07T22:09:02Z |
| publishDate | 2024-02-01 |
| publisher | Wiley |
| record_format | Article |
| series | eJHaem |
| spelling | doaj.art-548ebd1e0d9840d4ba5f7a287c626a2b2024-02-23T16:50:43ZengWileyeJHaem2688-61462024-02-015124224610.1002/jha2.841Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature reviewFabien Claves0Sylvain Carras1Barbara Burroni2Elsa Maitre3Jean Boutonnat4Xavier Troussard5Lysiane Molina6Hematology Department University Hospital of Grenoble Alpes Grenoble FranceHematology Department University Hospital of Grenoble Alpes Grenoble FrancePathology Department Cochin University Hospital Paris FranceUniversity Hospital CaenCaenFrancePathology Department University Hospital of Grenoble Alpes Grenoble FranceUniversity Hospital CaenCaenFranceHematology Department University Hospital of Grenoble Alpes Grenoble FranceAbstract Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder classically presenting with cytopenia and recurrent infections but atypical manifestations such as bone lesions, skin lesions and effusion have been described. We report here an unusual meningeal localization in a 33 years old man who presented with headache, hand paresthesia and visual symptoms. Brain magnetic resonance imaging revealed an occipital meningeal lesion. Diagnostic explorations led to the diagnosis of classical HCL with meningeal localization. After treatment by cladribine and rituximab the patient rapidly improved and is still in complete remission 12 months after end of treatment. The literature review identified 9 other cases of HCL with central nervous system localization (CNS) presenting with brain parenchyma and/or meninges localization. Four out of 9 patients presented with hyperleukocytosis. Most patients experienced good responses with various treatments. Cladribine alone or with rituximab led to complete responses similar to our patient. In our patient, molecular biology revealed KLF2 mutations, which implication in the atypical localization could be suspected but would need dedicated studies. In conclusion, CNS localizations of HCL are rare but can be observed and treatment with cladribine alone or with rituximab appears as an effective strategy.https://doi.org/10.1002/jha2.841atypical localizationcentral nervous systemcladribine and rituximabHairy cell leukemia |
| spellingShingle | Fabien Claves Sylvain Carras Barbara Burroni Elsa Maitre Jean Boutonnat Xavier Troussard Lysiane Molina Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review eJHaem atypical localization central nervous system cladribine and rituximab Hairy cell leukemia |
| title | Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review |
| title_full | Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review |
| title_fullStr | Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review |
| title_full_unstemmed | Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review |
| title_short | Atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association. A case report and literature review |
| title_sort | atypical meningeal localization of classical hairy cell leukemia with an impressive response to rituximab and cladribine association a case report and literature review |
| topic | atypical localization central nervous system cladribine and rituximab Hairy cell leukemia |
| url | https://doi.org/10.1002/jha2.841 |
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