Rao Raunak, Kulkarni Spoorthy, Wilkinson Ian B
Division of Experimental Medicine and Immunotherapeutics, University of Cambridge, Cambridge, UK.
Case Rep Vasc Med. 2020 Nov 9;2020:8887423. doi: 10.1155/2020/8887423. eCollection 2020.
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 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 M) 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/cm. BP was well-controlled (reaching 130/70 mmHg) with CCBs.
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.
骨髓增殖性肿瘤是一组异质性疾病，由一种或多种终末髓系细胞异常增殖引起，已确定的并发症包括血栓形成和出血事件；然而，仅有有限证据表明其与动脉高血压有关。在此，我们报告两例JAK2突变阳性骨髓增殖性肿瘤合并严重高血压的独立病例。病例1：一名39岁男性因高血压（血压200/120 mmHg）、红斑性肢痛症和头痛被转诊至我们的专科高血压病房。我们记录到血清肌酐水平升高（146 μmol/L）和全髓增殖。骨髓活检证实为JAK2突变阳性真性红细胞增多症。肾脏成像显示肾动脉狭窄。治疗采用了阿司匹林、长效硝苯地平、α-2A干扰素和肾动脉血管成形术。血压降至目标水平以下，平均为119/88 mmHg。随着血压下降，肾脏参数逐渐恢复正常。病例2：一名45岁男性出现高血压（208/131 mmHg）、手足发绀、（血管炎性）皮疹和不愈合溃疡。眼底检查显示左眼视盘模糊，全血细胞计数显示血小板增多。骨髓活检证实为JAK2突变阳性原发性血小板增多症。未发现肾动脉狭窄。采用惰性气体再呼吸法测得心输出量为5 L/min，估计外周血管阻力为1840达因/秒/平方厘米。使用钙通道阻滞剂可使血压得到良好控制（降至130/70 mmHg）。
这些病例表明全血细胞计数分析在严重高血压患者中的应用价值。血液高黏滞度和组成性JAK-STAT激活是骨髓增殖性肿瘤与高血压之间可能的病理生理联系。有必要开展进一步的实验和临床研究，以确定并了解血压与骨髓增殖性肿瘤之间可能存在的相互作用。
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