Blood Res 2014; 49(4):
Published online December 31, 2014
https://doi.org/10.5045/br.2014.49.4.283
© The Korean Society of Hematology
Division of Hematology-Oncology, Department of Internal Medicine, Yeouido St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, Korea.
Correspondence to : Jae Young Kim. Division of Hematology-Oncology, Department of Internal Medicine, Yeouido St. Mary's Hospital, 10, 63-ro, Yeongdeungpo-gu, Seoul 150-713, Korea. kjykjj04@naver.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
A 30-year-old woman presented with headache, hypertension, and abnormal laboratory findings on routine health examination. Her initial blood pressure was 190/140 mmHg, and pulse rate was 84 beats/min. The laboratory testing yielded a white blood cell count of 22.5×109/L hemoglobin at 22.5 g/dL, hematocrit level at 66.9%, and platelet count of 400.0×109/L. Physical examination revealed mild splenomegaly. She did not have hypoxemia and had never smoked. Sequentially performed bone marrow aspiration and biopsy revealed panmyelosis consistent with PV, and molecular testing revealed the presence of
To date, only a few cases of PV combined with renal artery stenosis (RAS) have been reported, and we do not know whether these two disease entities have a causal relationship. Although RAS is known to impair renal perfusion and subsequently increases EPO secretion and leads to secondary erythrocytosis, it is not obvious whether the elevated EPO level caused by RAS could induce an acquired mutation. In 1965, Luke et al. [1] reported the first case of RAS combined with PV and severe hypertension in a 54-year-old man. However,
PV is known to be associated with thrombotic risks. Theoretically, the increase in red blood cell mass is known to displace platelets toward the vessel wall and facilitate shear-induced platelet activation. Moreover, in patients with PV or ET, biochemical changes in the cell membrane and intracellular contents of red blood cell can increase red blood cell aggregation and thrombogenesis [5]. On the other hand, damage to the vascular structure itself or atherosclerosis has not been recognized as a major complication of PV because vascular effects by PV are mostly limited to the endothelium, which is insufficient for vascular stenosis, especially in large vessels. However, a few studies revealed that hyperproliferation of erythrocytes, leukocytes, or platelets could damage vascular endothelial cells, and this damage could cause fibromuscular intimal proliferation of the artery without thrombotic occlusion [6].
RAS is caused chiefly by atherosclerosis or fibromuscular dysplasia (FMD). Atherosclerosis commonly occurs at the origin or the proximal portion of the artery in older patients with typical cardiovascular risk factors. In contrast, FMD occurs in the middle or distal arterial segments in younger patients without cardiovascular risk factors. FMD is a non-atherosclerotic, non-inflammatory vascular disease that most commonly affects the renal artery. Although a variety of genetic, mechanical, and hormonal factors have been proposed, the principal pathogenetic mechanism of FMD remains uncertain [7]. As mentioned above, we do not know the exact underlying mechanism that was predominant in our case because we did not perform a pathologic evaluation of the patient's renal artery. Although we presume FMD to be the main mechanism because of the patient's young age and the location of renal arterial stenosis, both mechanisms can be explained in connection with the secondary effect of PV or ET. A review of related literature revealed conflicting reports. First, Hua et al. [4] who reported a PV patient with multiple intracranial and extracranial steno-occlusive diseases, insisted that the large vessel steno-occlusion is due to hyperplasia of smooth muscle cells and luminal thrombosis rather than atherosclerosis or inflammation. Furthermore, they presented a few similar cases of sickle cell anemia or ET for reference with the explanation that progressive intimal and medial wall proliferation can be caused by endothelial irritation and edema due to repeated microvascular thrombosis [4]. However, Gavriilaki et al. [8] insisted that PV may lead to atherosclerosis and subsequent atherosclerotic RAS based on their case report and supported that theory by thrombotic restenosis, which occurred following a period of high hematocrit levels after stenting. Notably, Cucuianu et al. [9] performed arterial Doppler ultrasonography in 37 patients with PV (29) and ET (8); they found significant arterial stenosis in 23 patients (62.1%) including 10 patients with peripheral arterial stenosis. The possible relevance was explained by hyperactivation of
In summary, our case and a few previous reports demonstrate that there is a possible mutual relationship between PV and RAS. Therefore, elevated EPO levels and hypertension in patients with
Computed tomography (CT) angiogram shows bilateral renal artery stenosis. Rt. kidney is atrophied with multifocal renal artery stenosis.
Conventional angiogram of renal artery demonstrates bilateral renal artery stenosis
Blood Res 2014; 49(4): 283-285
Published online December 31, 2014 https://doi.org/10.5045/br.2014.49.4.283
Copyright © The Korean Society of Hematology.
Jae Young Kim*, In Sook Woo, Sang Hoon Yoo, Kang Nam Bae, Gi Jun Kim, and Yun Hwa Jung
Division of Hematology-Oncology, Department of Internal Medicine, Yeouido St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, Korea.
Correspondence to: Jae Young Kim. Division of Hematology-Oncology, Department of Internal Medicine, Yeouido St. Mary's Hospital, 10, 63-ro, Yeongdeungpo-gu, Seoul 150-713, Korea. kjykjj04@naver.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
A 30-year-old woman presented with headache, hypertension, and abnormal laboratory findings on routine health examination. Her initial blood pressure was 190/140 mmHg, and pulse rate was 84 beats/min. The laboratory testing yielded a white blood cell count of 22.5×109/L hemoglobin at 22.5 g/dL, hematocrit level at 66.9%, and platelet count of 400.0×109/L. Physical examination revealed mild splenomegaly. She did not have hypoxemia and had never smoked. Sequentially performed bone marrow aspiration and biopsy revealed panmyelosis consistent with PV, and molecular testing revealed the presence of
To date, only a few cases of PV combined with renal artery stenosis (RAS) have been reported, and we do not know whether these two disease entities have a causal relationship. Although RAS is known to impair renal perfusion and subsequently increases EPO secretion and leads to secondary erythrocytosis, it is not obvious whether the elevated EPO level caused by RAS could induce an acquired mutation. In 1965, Luke et al. [1] reported the first case of RAS combined with PV and severe hypertension in a 54-year-old man. However,
PV is known to be associated with thrombotic risks. Theoretically, the increase in red blood cell mass is known to displace platelets toward the vessel wall and facilitate shear-induced platelet activation. Moreover, in patients with PV or ET, biochemical changes in the cell membrane and intracellular contents of red blood cell can increase red blood cell aggregation and thrombogenesis [5]. On the other hand, damage to the vascular structure itself or atherosclerosis has not been recognized as a major complication of PV because vascular effects by PV are mostly limited to the endothelium, which is insufficient for vascular stenosis, especially in large vessels. However, a few studies revealed that hyperproliferation of erythrocytes, leukocytes, or platelets could damage vascular endothelial cells, and this damage could cause fibromuscular intimal proliferation of the artery without thrombotic occlusion [6].
RAS is caused chiefly by atherosclerosis or fibromuscular dysplasia (FMD). Atherosclerosis commonly occurs at the origin or the proximal portion of the artery in older patients with typical cardiovascular risk factors. In contrast, FMD occurs in the middle or distal arterial segments in younger patients without cardiovascular risk factors. FMD is a non-atherosclerotic, non-inflammatory vascular disease that most commonly affects the renal artery. Although a variety of genetic, mechanical, and hormonal factors have been proposed, the principal pathogenetic mechanism of FMD remains uncertain [7]. As mentioned above, we do not know the exact underlying mechanism that was predominant in our case because we did not perform a pathologic evaluation of the patient's renal artery. Although we presume FMD to be the main mechanism because of the patient's young age and the location of renal arterial stenosis, both mechanisms can be explained in connection with the secondary effect of PV or ET. A review of related literature revealed conflicting reports. First, Hua et al. [4] who reported a PV patient with multiple intracranial and extracranial steno-occlusive diseases, insisted that the large vessel steno-occlusion is due to hyperplasia of smooth muscle cells and luminal thrombosis rather than atherosclerosis or inflammation. Furthermore, they presented a few similar cases of sickle cell anemia or ET for reference with the explanation that progressive intimal and medial wall proliferation can be caused by endothelial irritation and edema due to repeated microvascular thrombosis [4]. However, Gavriilaki et al. [8] insisted that PV may lead to atherosclerosis and subsequent atherosclerotic RAS based on their case report and supported that theory by thrombotic restenosis, which occurred following a period of high hematocrit levels after stenting. Notably, Cucuianu et al. [9] performed arterial Doppler ultrasonography in 37 patients with PV (29) and ET (8); they found significant arterial stenosis in 23 patients (62.1%) including 10 patients with peripheral arterial stenosis. The possible relevance was explained by hyperactivation of
In summary, our case and a few previous reports demonstrate that there is a possible mutual relationship between PV and RAS. Therefore, elevated EPO levels and hypertension in patients with
Computed tomography (CT) angiogram shows bilateral renal artery stenosis. Rt. kidney is atrophied with multifocal renal artery stenosis.
Conventional angiogram of renal artery demonstrates bilateral renal artery stenosis
Computed tomography (CT) angiogram shows bilateral renal artery stenosis. Rt. kidney is atrophied with multifocal renal artery stenosis.
|@|~(^,^)~|@|Conventional angiogram of renal artery demonstrates bilateral renal artery stenosis