scholarly journals Two Cases of Severe Hypertension in JAK2 Mutation-Positive Myeloproliferative Neoplasms

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Raunak Rao ◽  
Spoorthy Kulkarni ◽  
Ian B. Wilkinson
Keyword(s):  
Bone Marrow ◽  
Renal Artery ◽  
Renal Artery Stenosis ◽  
Bone Marrow Biopsy ◽  
Blood Count ◽  
Severe Hypertension ◽  
Myeloproliferative Neoplasms ◽  
Artery Stenosis ◽  
Full Blood Count ◽  
Jak2 Mutation

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.

scholarly journals Renal artery stenosis in a young female patient with severe hypertension - a case report

2019 ◽  
Vol 13 (3) ◽  
pp. 176-180
Author(s):  
Giuliano De Stefano ◽  
Federica De Pisapia ◽  
Giovanni Albano ◽  
Maria Immacolata Arnone ◽  
Giovanni Esposito ◽  
...  
Keyword(s):  
Case Report ◽  
Renal Artery ◽  
Renal Artery Stenosis ◽  
Severe Hypertension ◽  
Young Female ◽  
Secondary Hypertension ◽  
Artery Stenosis ◽  
Drug Eluting Stent ◽  
Hypertensive Patients ◽  
Drug Eluting

Renal artery stenosis is a frequent cause of secondary hypertension, but the diagnostic and therapeutic management of these hypertensive patients is controversial. We report a case of secondary hypertension due to renal artery stenosis, treated with the implantation of a drug-eluting stent.

scholarly journals CXCL16 regulates renal injury and fibrosis in experimental renal artery stenosis

2016 ◽  
Vol 311 (3) ◽  
pp. H815-H821 ◽  
Author(s):  
Zhiheng Ma ◽  
Xiaogao Jin ◽  
Liqun He ◽  
Yanlin Wang
Keyword(s):  
Blood Pressure ◽  
Bone Marrow ◽  
Renal Artery ◽  
Renal Artery Stenosis ◽  
Knockout Mice ◽  
Renal Injury ◽  
Critical Role ◽  
Artery Stenosis ◽  
Left Renal Artery ◽  
Wild Type

Recent studies have shown that inflammation plays a critical role in the initiation and progression of hypertensive kidney disease, including renal artery stenosis. However, the signaling mechanisms underlying the induction of inflammation are poorly understood. We found that CXCL16 was induced in the kidney in a murine model of renal artery stenosis. To determine whether CXCL16 is involved in renal injury and fibrosis, wild-type and CXCL16 knockout mice were subjected to renal artery stenosis induced by placing a cuff on the left renal artery. Wild-type and CXCL16 knockout mice had comparable blood pressure at baseline. Renal artery stenosis caused an increase in blood pressure that was similar between wild-type and CXCL16 knockout mice. CXCL16 knockout mice were protected from RAS-induced renal injury and fibrosis. CXCL16 deficiency suppressed bone marrow-derived fibroblast accumulation and myofibroblast formation in the stenotic kidneys, which was associated with less expression of extracellular matrix proteins. Furthermore, CXCL16 deficiency inhibited infiltration of F4/80+ macrophages and CD3+ T cells in the stenotic kidneys compared with those of wild-type mice. Taken together, our results indicate that CXCL16 plays a pivotal role in the pathogenesis of renal artery stenosis-induced renal injury and fibrosis through regulation of bone marrow-derived fibroblast accumulation and macrophage and T-cell infiltration.

scholarly journals Characterization of Philadelphia-Negative Myeloproliferative Neoplasms in the Chilean Public Health System: Multicentric Study

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5479-5479
Author(s):  
Ximena Valladares ◽  
Christine Rojas ◽  
Camila Peña ◽  
Claudia Gajardo ◽  
Maria Elena Cabrera ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Biopsy ◽  
Blood Count ◽  
Myeloproliferative Neoplasms ◽  
Cytogenetic Study ◽  
Public Hospitals ◽  
Lower Percentage ◽  
Multicentric Study ◽  
Clinical Records

Abstract Background: Philadelphia-negative Myeloproliferative Neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are Polycythemia Vera (PV), Essential Thrombocytopenia (ET) and Myelofibrosis (MF). The diagnosis includes clinical, histological and molecular features. There are not data from Chile. The aim of this study is to determinate epidemiological, clinical, diagnostic and therapeutic characteristics of Ph-MPN in our country. Methods: Descriptive and retrospective study. We reviewed the database of the Molecular Biology Laboratory at the Hospital del Salvador, a national reference laboratory, from 2012 to 2017. All patients referred as Ph-MPN were included. We reviewed the clinical records to obtain clinical information. Results: Clinical data was obtained from 468 cases from 12 public hospitals in Chile. Median age at diagnosis was 70 years. Female to Male ratio= 1,15:1, without significant differences between Ph-MPNs. ET was the most frequently Ph-MNP found, accounting for 49,4% of all Ph-MPN, followed by PV (37%) and MF (10,4%). A 66,2% of ET was JAK2 V617F+. Bone marrow biopsy was performed in 35% of ET cases. Only 7,8% had cytogenetic study. Splenomegaly was found in 8%. Thrombosis was observed in 23,8%. The median platelet count was 842x109/L. All patients received hydrea +/- aspirin or oral anticoagulation. Of the total of PV, 86,6% was JAK2+. Bone marrow biopsy was performed in a quarter of the cases. Thrombosis frequency was 14,5%. A 29% had splenomegaly. Median hemoglobin level was 18 gr/dl. All patients were treated with aspirin +/- phlebotomy and about half of them required cytoreduction. Two patients were refractory to hydrea and used ruxolitinib as second line treatment. A 63,3% of the MF were JAK-2+. Bone marrow biopsy was performed in 59% and 20% had a cytogenetic study. Only one fifth of patients had LDH measurement at diagnosis. Splenomegaly was observed in 75,5% of cases. Thrombosis frequency was 13%. Anemia was the most frequent finding in complete blood count. The treatments were heterogeneous, including hydrea, EPO, thalidomide/prednisone, danazol and ruxolitinib. Discussion: TE was the most common Ph-MPN. The epidemiological and blood count findings were similar to the data reported in the literature. It is important to note that with the 2016 WHO classification new criteria, some of patients diagnosed with ET, now will be in PV cathegory (21 patients in our serie). The distribution of JAK2V617F+ in Ph-MPN was similar to the published data, except for PV, in which we found a lower percentage of JAK2+. Thrombosis were lower than the data reported for PV. It is worrisome that bone marrow biopsy and cytogenetic study were performed only in a low percentage of the patients. The treatment strategies were heterogeneous and not standardized among the participating centers. These findings reveal a lack in the use of the diagnostic tools for Ph-MPN. It is important to improve clinical and molecular characterization of these patients in order to guide available therapeutic alternatives in our country. Disclosures No relevant conflicts of interest to declare.

scholarly journals Severe hypertension and massive proteinuria in a newborn with renal artery stenosis

2004 ◽  
Vol 19 (5) ◽  
pp. 544-546 ◽  
Author(s):  
Fran�ois Cachat ◽  
Alina Bogaru ◽  
Jean-Leopold Micheli ◽  
Domenico Lepori ◽  
Jean-Pierre Guignard
Keyword(s):  
Renal Artery ◽  
Renal Artery Stenosis ◽  
Severe Hypertension ◽  
Artery Stenosis

Severe hypertension in a patient with unilateral obstructive hydronephrosis and renal artery stenosis

1978 ◽  
Vol 93 (3) ◽  
pp. 458-459 ◽  
Author(s):  
Matti Uhari ◽  
Mikko Remes ◽  
Peter Lanning ◽  
Juhani Seppänen
Keyword(s):  
Renal Artery ◽  
Renal Artery Stenosis ◽  
Severe Hypertension ◽  
Artery Stenosis

scholarly journals Revascularization in a 17-Year-Old Girl with Neurofibromatosis and Severe Hypertension Caused by Renal Artery Stenosis

2017 ◽  
Vol 44 (1) ◽  
pp. 50-54
Author(s):  
Carmen C. Beladan ◽  
Oliviana D. Geavlete ◽  
Simona Botezatu ◽  
Marin Postu ◽  
Bogdan A. Popescu ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Renal Artery ◽  
Renal Artery Stenosis ◽  
Neurofibromatosis Type 1 ◽  
Severe Hypertension ◽  
Neurofibromatosis Type ◽  
Artery Stenosis ◽  
Left Renal Artery ◽  
Renal Angioplasty

Renal artery stenosis caused by neurofibromatosis is a rare cause of renovascular hypertension. This hypertension can develop during childhood and is one of the leading causes of poor outcome. We report the case of a 17-year-old girl who was incidentally diagnosed with severe hypertension. During her examination for secondary hypertension, we reached a diagnosis of neurofibromatosis type 1 on the basis of a cluster of typical findings: optic nerve glioma, café au lait spots, nodular neurofibromas, and axillary freckling. Renal angiograms revealed a hemodynamically significant left renal artery stenosis (70%). Renal angioplasty with a self-expanding stent was performed one month later for rapidly progressive renal artery stenosis (90%) and uncontrolled blood pressure. Excellent blood pressure control resulted immediately and was maintained as of the 2-year follow-up evaluation. We think that percutaneous transluminal renal angioplasty can be effective in select patients who have neurofibromatosis type 1 and refractory hypertension caused by renal artery stenosis.

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