Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms
Background. Myeloproliferative neoplasms are a heterogeneous group of disorders resulting from the abnormal proliferation of one or more terminal myeloid cells—established complications include thrombosis and haemorrhagic events; however, there is limited evidence to suggest an association with arte...
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2020-01-01
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| Series: | Case Reports in Vascular Medicine |
| Online Access: | http://dx.doi.org/10.1155/2020/8887423 |
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| author | Raunak Rao Spoorthy Kulkarni Ian B. Wilkinson |
| author_facet | Raunak Rao Spoorthy Kulkarni Ian B. Wilkinson |
| author_sort | Raunak Rao |
| collection | DOAJ |
| description | Background. Myeloproliferative neoplasms are a heterogeneous group of disorders resulting from the abnormal proliferation of one or more terminal myeloid cells—established complications include thrombosis and haemorrhagic events; however, there is limited evidence to suggest an association with arterial hypertension. Herein, we report two independent cases of severe hypertension in JAK2 mutation-positive myeloproliferative neoplasms. Case Presentations. Case 1: a 39-year-old male was referred to our specialist hypertension unit with high blood pressure (BP) (200/120 mmHg), erythromelalgia, and headaches. We recorded elevated serum creatinine levels (146 μM) and panmyelosis. Bone marrow biopsy confirmed JAK2-mutation-positive polycythaemia vera. Renal imaging revealed renal artery stenosis. Aspirin, long-acting nifedipine, interferon-alpha 2A, and renal artery angioplasty were employed in management. BP reached below target levels to an average of 119/88 mmHg. Renal parameters normalised gradually alongside BP. Case 2: a 45-year-old male presented with high BP (208/131 mmHg), acrocyanosis, (vasculitic) skin rashes, and nonhealing ulcers. Fundoscopy showed optic disc blurring in the left eye and full blood count revealed thrombocytosis. Bone marrow biopsy confirmed JAK2-mutation-positive essential thrombocytosis. No renal artery stenosis was found. Cardiac output was measured at 5 L/min using an inert gas rebreathing method, providing an estimated peripheral vascular resistance of 1840 dynes/s/cm5. BP was well-controlled (reaching 130/70 mmHg) with CCBs. Conclusions. These presentations highlight the utility of full blood count analysis in patients with severe hypertension. Hyperviscosity and constitutive JAK-STAT activation are amongst the proposed pathophysiology linking myeloproliferative neoplasms and hypertension. Further experimental and clinical research is necessary to identify and understand possible interactions between BP and myeloproliferative neoplasms. |
| format | Article |
| id | doaj-art-b5622cdb9b1440edb94f9d1b6b83fe93 |
| institution | Kabale University |
| issn | 2090-6986 2090-6994 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
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| series | Case Reports in Vascular Medicine |
| spelling | doaj-art-b5622cdb9b1440edb94f9d1b6b83fe932025-02-03T01:03:58ZengWileyCase Reports in Vascular Medicine2090-69862090-69942020-01-01202010.1155/2020/88874238887423Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative NeoplasmsRaunak Rao0Spoorthy Kulkarni1Ian B. Wilkinson2Division of Experimental Medicine and Immunotherapeutics, University of Cambridge, Cambridge, UKDivision of Experimental Medicine and Immunotherapeutics, University of Cambridge, Cambridge, UKDivision of Experimental Medicine and Immunotherapeutics, University of Cambridge, Cambridge, UKBackground. Myeloproliferative neoplasms are a heterogeneous group of disorders resulting from the abnormal proliferation of one or more terminal myeloid cells—established complications include thrombosis and haemorrhagic events; however, there is limited evidence to suggest an association with arterial hypertension. Herein, we report two independent cases of severe hypertension in JAK2 mutation-positive myeloproliferative neoplasms. Case Presentations. Case 1: a 39-year-old male was referred to our specialist hypertension unit with high blood pressure (BP) (200/120 mmHg), erythromelalgia, and headaches. We recorded elevated serum creatinine levels (146 μM) and panmyelosis. Bone marrow biopsy confirmed JAK2-mutation-positive polycythaemia vera. Renal imaging revealed renal artery stenosis. Aspirin, long-acting nifedipine, interferon-alpha 2A, and renal artery angioplasty were employed in management. BP reached below target levels to an average of 119/88 mmHg. Renal parameters normalised gradually alongside BP. Case 2: a 45-year-old male presented with high BP (208/131 mmHg), acrocyanosis, (vasculitic) skin rashes, and nonhealing ulcers. Fundoscopy showed optic disc blurring in the left eye and full blood count revealed thrombocytosis. Bone marrow biopsy confirmed JAK2-mutation-positive essential thrombocytosis. No renal artery stenosis was found. Cardiac output was measured at 5 L/min using an inert gas rebreathing method, providing an estimated peripheral vascular resistance of 1840 dynes/s/cm5. BP was well-controlled (reaching 130/70 mmHg) with CCBs. Conclusions. These presentations highlight the utility of full blood count analysis in patients with severe hypertension. Hyperviscosity and constitutive JAK-STAT activation are amongst the proposed pathophysiology linking myeloproliferative neoplasms and hypertension. Further experimental and clinical research is necessary to identify and understand possible interactions between BP and myeloproliferative neoplasms.http://dx.doi.org/10.1155/2020/8887423 |
| spellingShingle | Raunak Rao Spoorthy Kulkarni Ian B. Wilkinson Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms Case Reports in Vascular Medicine |
| title | Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms |
| title_full | Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms |
| title_fullStr | Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms |
| title_full_unstemmed | Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms |
| title_short | Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms |
| title_sort | two cases of severe hypertension in jak2 mutation positive myeloproliferative neoplasms |
| url | http://dx.doi.org/10.1155/2020/8887423 |
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