Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinib
Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterised by sustained thrombocytosis and frequently associated with JAK2 V617F mutations. It is primarily known for its thrombotic and haemorrhagic complications, which dominate its clinical presentation. However, inflammat...
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SMC MEDIA SRL
2025-07-01
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| Series: | European Journal of Case Reports in Internal Medicine |
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| Online Access: | https://www.ejcrim.com/index.php/EJCRIM/article/view/5615 |
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| author | Murad Aldarayseh Leonardo Marmolejos Dalia Al Zoubi Michelle Cholankeril |
| author_facet | Murad Aldarayseh Leonardo Marmolejos Dalia Al Zoubi Michelle Cholankeril |
| author_sort | Murad Aldarayseh |
| collection | DOAJ |
| description | Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterised by sustained thrombocytosis and frequently associated with JAK2 V617F mutations. It is primarily known for its thrombotic and haemorrhagic complications, which dominate its clinical presentation. However, inflammatory and dermatologic manifestations are uncommon and less well recognised. Among these, cutaneous vasculitis – particularly leukocytoclastic vasculitis (LCV) – is a rare and underreported feature. We present the case of a 54-year-old woman with JAK2-positive ET who developed a persistent urticarial rash that coincided with worsening thrombocytosis. Bone marrow biopsy revealed megakaryocytic hyperplasia, confirming the diagnosis of ET. Initially, the patient responded to hydroxyurea, but her disease eventually became refractory, and anagrelide failed to control either her platelet count or symptoms. Her skin rash progressed in parallel with uncontrolled thrombocytosis, and biopsy confirmed LCV. Initiation of ruxolitinib, a JAK1/2 inhibitor, led to haematologic improvement and complete resolution of the vasculitis. This case highlights a rare but clinically significant association between ET and cutaneous vasculitis and suggests a potential role for JAK inhibition in managing such refractory inflammatory manifestations. |
| format | Article |
| id | doaj-art-9c12046574bc4ee6848b9f4a6ff0aeeb |
| institution | Kabale University |
| issn | 2284-2594 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | SMC MEDIA SRL |
| record_format | Article |
| series | European Journal of Case Reports in Internal Medicine |
| spelling | doaj-art-9c12046574bc4ee6848b9f4a6ff0aeeb2025-08-20T03:38:59ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942025-07-0110.12890/2025_0056155150Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinibMurad Aldarayseh0Leonardo Marmolejos1Dalia Al Zoubi2Michelle Cholankeril3Internal Medicine Department, RWJBarnabas Health/Trinitas Regional Medical Center, Elizabeth, USAInternal Medicine Department, RWJBarnabas Health/Trinitas Regional Medical Center, Elizabeth, USACollege of Medicine, Jordan University of Science and Technology, Irbid, JordanInternal Medicine Department, RWJBarnabas Health/Trinitas Regional Medical Center, Elizabeth, USAEssential thrombocythemia (ET) is a chronic myeloproliferative neoplasm characterised by sustained thrombocytosis and frequently associated with JAK2 V617F mutations. It is primarily known for its thrombotic and haemorrhagic complications, which dominate its clinical presentation. However, inflammatory and dermatologic manifestations are uncommon and less well recognised. Among these, cutaneous vasculitis – particularly leukocytoclastic vasculitis (LCV) – is a rare and underreported feature. We present the case of a 54-year-old woman with JAK2-positive ET who developed a persistent urticarial rash that coincided with worsening thrombocytosis. Bone marrow biopsy revealed megakaryocytic hyperplasia, confirming the diagnosis of ET. Initially, the patient responded to hydroxyurea, but her disease eventually became refractory, and anagrelide failed to control either her platelet count or symptoms. Her skin rash progressed in parallel with uncontrolled thrombocytosis, and biopsy confirmed LCV. Initiation of ruxolitinib, a JAK1/2 inhibitor, led to haematologic improvement and complete resolution of the vasculitis. This case highlights a rare but clinically significant association between ET and cutaneous vasculitis and suggests a potential role for JAK inhibition in managing such refractory inflammatory manifestations.https://www.ejcrim.com/index.php/EJCRIM/article/view/5615essential thrombocythaemialeukocytoclastic vasculitis (lcv)jak2 mutationruxolitiniburticarial rash |
| spellingShingle | Murad Aldarayseh Leonardo Marmolejos Dalia Al Zoubi Michelle Cholankeril Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinib European Journal of Case Reports in Internal Medicine essential thrombocythaemia leukocytoclastic vasculitis (lcv) jak2 mutation ruxolitinib urticarial rash |
| title | Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinib |
| title_full | Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinib |
| title_fullStr | Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinib |
| title_full_unstemmed | Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinib |
| title_short | Leukocytoclastic vasculitis in JAK2-positive essential thrombocythemia: a case of cytokine-driven urticarial rash responsive to ruxolitinib |
| title_sort | leukocytoclastic vasculitis in jak2 positive essential thrombocythemia a case of cytokine driven urticarial rash responsive to ruxolitinib |
| topic | essential thrombocythaemia leukocytoclastic vasculitis (lcv) jak2 mutation ruxolitinib urticarial rash |
| url | https://www.ejcrim.com/index.php/EJCRIM/article/view/5615 |
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