今日の臨床サポート

多血症

著者: 樋口敬和 獨協医科大学埼玉医療センター 輸血部

監修: 神田善伸 自治医科大学附属病院 血液科

著者校正/監修レビュー済:2021/03/10
参考ガイドライン:
  1. McMullin MF, Harrison CN, Ali S, et al: A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology Guideline. British Journal of Haematology 2019 Jan;184(2):176-191.
患者向け説明資料

概要・推奨   

  1. 多血症は、わが国では、ヘモグロビン(Hb)濃度が、男性では18g/dl、女性では16 g/dl以上、ヘマトクリット(Ht)では、男性では55%、女性では50%以上である場合をいう。
  1. 循環血液量は、日常臨床では、ヘモグロビン(Hb)とヘマトクリット(Ht)を評価の指標として用いることが勧められる。
  1. Hbg/dl/ヘマトクリットHtが、男性で19.5/60、女性で17.5/55を超えていれば、循環赤血球量が増加していると判断して絶対的赤血球増加症として対応することが勧められる。
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  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必
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  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲 覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧には
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
樋口敬和 : 特に申告事項無し[2021年]
監修:神田善伸 : 未申告[2021年]

改訂のポイント:
  1. 内容について定期レビューを行った。

病態・疫学・診察

疫学情報・病態・注意事項  
  1. 多血症は、わが国では、ヘモグロビン(Hb)濃度が、男性では18g/dl、女性では16g/dl以上、ヘマトクリット(Ht)では、男性では55%、女性では50%以上である場合をいう。
  1. 無症状で他の目的で行った採血や健康診断などの際に発見されることが多いが、ときに、頭痛、頭重感、めまいなどを訴えたり、赤ら顔に気づいて検査されて発見されることもある。
  1. 循環血漿量が低下していることによる見かけ上の多血症(相対的赤血球増加症)と、循環赤血球量が増加している絶対的赤血球増加症がある。
  1. 赤血球増加症(多血症)の鑑別:<図表>
  1. 相対性赤血球増加症は、急性の脱水でも起こるが、よくみられるのはストレス赤血球増加症(Gaisböck症候群)である。
  1. ストレス赤血球増加症は、中年のやや小太りの男性に多く、多くは喫煙者である。治療は禁煙を含めた生活習慣の改善である。
  1. 循環赤血球量の測定は一般的な検査でなく通常行われない。Hb(g/dl)/ヘマトクリット(Ht)(%)が、男性で19.5/60、女性で17.5/55を超えていれば、循環赤血球量が増加していると判断してよく、絶対的赤血球増加症と考えられる。
  1. 絶対性赤血球増加症には、骨髄増殖性腫瘍の1つである真性赤血球増加症(PV)とエリスロポエチン(EPO)産生亢進やHbの酸素親和性の異常による二次性赤血球増加症がある。
  1. 二次性赤血球増加症では、EPO産生を亢進させる基礎疾患を検討する。通常、基礎疾患の症状が主で多血症の症状は目立たない。
  1. 赤血球増加症(多血症)の分類と原因:<図表>
 
  1. 循環血液量の測定は日常臨床で行える検査でないが、ヘモグロビン(Hb)とヘマトクリット(Ht)で代用可能であるので、これらを参考にすることが勧められるO
  1. 循環赤血球量と血漿量を測定してある真性赤血球増加症(PV)患者77例と相対的赤血球増加症(AP)患者66例で、HbあるいはHtにより循環赤血球量が増加していることを評価できるか検討した。男性ではPV患者の35%、AP患者の14%でHb値が18.5 g/dlを超えており、女性でもPV患者の63%、AP患者の35%がHb>16.5 g/dlで、Hb値だけでは循環赤血球量の指標として用いることはできなかった。Htの範囲は、PV患者の男性では50~73%、女性で44~68%であったが、AP患者では59%を超えた症例はなかった。Ht値が60%以上であれば循環赤血球量が増加していると判断していい[1]
  1. 循環血漿量と赤血球量を測定した105例の多血症患者(PV 25例、二次性多血症35例、相対的多血症28例、本態性血小板血症 7例)でHbとHtについて検討した。循環赤血球量増加はPVの診断において76%の感度で、相対的多血症との鑑別の特異度は79%であった。循環赤血球量はHb、Htレベルと相関し、PVの診断において追加的な情報とはならなかった。Hb/Ht値が、男性で19.5/58、女性で17.5/53を超えていた症例のほとんどで循環赤血球量が増加していた[2]
  1. 追記:循環赤血球量の増加は、真性赤血球増加症、二次性赤血球増加症でみられ、ストレス赤血球増加症ではみられないことから、これらの鑑別において重要であるが、日常臨床で通常行われる検査ではない。Hb値とHt値を循環赤血球量を測定する代わりに循環赤血球量増加を判断する指標として用いることができる。Hb/Htが、男性で19.5/58、女性で17.5/53を超える多血症では循環赤血球量が増加していると考えてよく、男性でHt≧60%、女性でHt≧55%であれば、循環赤血球量が増加していると判断していい。
  1. なお、WHO分類第4版(2016年改訂)の真性赤血球増加症(PV)の診断基準では、Hb/Htが、男性で16.5/49、女性で16.0/48とより低い基準を採用しているが、この基準を超えているだけではPVと診断できない。
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問診:
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文献 

著者: Peter L Johansson, Soodabeh Safai-Kutti, Jack Kutti
雑誌名: Br J Haematol. 2005 Jun;129(5):701-5. doi: 10.1111/j.1365-2141.2005.05517.x.
Abstract/Text The diagnosis of polycythaemia vera (PV) has been established upon sets of clinical criteria, which require the presence of absolute erythrocytosis (AE). The most recent clinical criteria for PV, published by the World Health Organization (WHO) in 2001, also required AE, and stated that the measured red cell mass (RCM) could be replaced by a surrogate marker for AE; a haemoglobin (Hb) value of >18.5 g/dl in males and >16.5 g/dl in females. The present study evaluated the potential of venous haematocrit (Hct) and Hb values as possible surrogate markers for AE in a series of 77 consecutive patients with PV and 66 patients with apparent polycythaemia (AP), in all of whom the RCM had been previously determined. In only 35% of the male PV patients would Hb values >18.5 g/dl indicate the presence of AE. Conversely, 14% of male AP patients would be misdiagnosed as having AE. A Hb > 16.5 g/dl would predict the presence of AE in 63% of the female PV patients, but 35% of female AP cases would be misdiagnosed as having AE. However, when the Hct was > or =0.60 an AE was always present, and this was true for both male and female subjects.

PMID 15916693  Br J Haematol. 2005 Jun;129(5):701-5. doi: 10.1111/j.13・・・
著者: Shireen Sirhan, Virgil F Fairbanks, Ayalew Tefferi
雑誌名: Cancer. 2005 Jul 1;104(1):213-5. doi: 10.1002/cncr.21105.
Abstract/Text BACKGROUND: Despite the absence of any systematic evidence for diagnostic utility, red cell mass (RCM) measurement has been endorsed as a major diagnostic criterion for polycythemia vera (PV) based on a set of eligibility criteria for a clinical trial formulated by an International PV Study Group in 1967.
METHODS: A retrospective analysis was performed on a consecutive series of 105 patients who underwent blood volume measurements for evaluation of polycythemia. In a previous study, the authors had systematically compared RCM measurement by 51Cr-labeled erythrocytes and 125I-labeled human serum albumin and demonstrated equivalence between the two methods. In the current study, they used the latter method and applied the International Committee for Standardization in Haematology recommendations for result interpretation in order to evaluate test performance and practical utility.
RESULTS: RCM exceeded the 98-99% limits of the reference range in 76%, 20%, 22%, and 57% of patients with PV (n = 25), secondary polycythemia (n = 35), spurious or apparent polycythemia (n = 38), and essential thrombocythemia (n = 7), respectively. Decreased plasma volume was rarely seen in any of the disease categories. In all instances of PV, the diagnosis was readily apparent, based on alternative clinical and laboratory tests, and did not require the additional information from blood volume measurement. Furthermore, alternative methods of PV diagnosis, based on disease-specific biological markers as well as bone marrow histology, are now available.
CONCLUSIONS: The continued use of RCM and plasma volume measurements for the diagnosis of PV is no longer warranted.

PMID 15889449  Cancer. 2005 Jul 1;104(1):213-5. doi: 10.1002/cncr.2110・・・
著者: D Nordenberg, R Yip, N J Binkin
雑誌名: JAMA. 1990 Sep 26;264(12):1556-9.
Abstract/Text The relationships among cigarette smoking, hemoglobin concentration, and carboxyhemoglobin concentration were examined using data from the Second National Health and Nutrition Examination Survey. Among women, smokers had a mean (+/- SE) hemoglobin level of 137 +/- 0.4 g/L, significantly higher than the mean hemoglobin level of 133 +/- 0.5 g/L for never-smokers. Among men, the mean hemoglobin levels for smokers and never-smokers were 156 +/- 0.4 and 152 +/- 0.5 g/L, respectively. No significant difference in mean hemoglobin was noted between ex-smokers and never-smokers. Mean hemoglobin levels and carboxyhemoglobin levels increased progressively with the number of cigarettes consumed per day. Cigarette smoking seems to cause a generalized upward shift of the hemoglobin distribution curve, which reduces the utility of hemoglobin level to detect anemia. Among women of comparable socioeconomic status, the prevalence of anemia was 4.8% +/- 0.6% among smokers, compared with 8.5% +/- 1.2% among never-smokers. This study suggests that minimum hemoglobin cutoff values should be adjusted for smokers to compensate for the masking effect of smoking on the detection of anemia.

PMID 2395196  JAMA. 1990 Sep 26;264(12):1556-9.
著者: J C Moore-Gillon, D F Treacher, E J Gaminara, T C Pearson, I R Cameron
雑誌名: Br Med J (Clin Res Ed). 1986 Sep 6;293(6547):588-90.
Abstract/Text The aetiology of polycythaemia is unclear in up to 30% of patients. Twenty patients with unexplained polycythaemia were investigated to see whether they had an intermittent hypoxic stimulus to erythropoiesis that was undetected by conventional investigations for hypoxic secondary polycythaemia. Overnight polygraphic sleep studies showed that five patients had prolonged nocturnal hypoxaemia. Their arterial oxygen saturation was below 92%, the level at which appreciable hypoxic stimulation of erythropoiesis occurs, for 26-68% of the time for which they were studied. Considerable evidence is accumulating that intermittent hypoxia is a potent stimulus to erythropoiesis, and clinicians should consider the possibility of nocturnal hypoxia in patients with unexplained polycythaemia. Appropriate investigation will lead to the correct diagnosis of polycythaemia secondary to hypoxia in some cases previously regarded as idiopathic, and treatment may then be planned accordingly.

PMID 3092936  Br Med J (Clin Res Ed). 1986 Sep 6;293(6547):588-90.
著者: Christopher D Nguyen, Jon-Erik C Holty
雑誌名: Respir Med. 2017 Sep;130:27-34. doi: 10.1016/j.rmed.2017.07.003. Epub 2017 Jul 6.
Abstract/Text BACKGROUND: The current literature suggests a relationship between obstructive sleep apnea (OSA) severity and hematocrit. However, the degree that OSA contributes to clinically significant erythrocytosis is uncertain. The aim of this study is to evaluate this association in a large study sample controlling for multiple confounders.
METHODS: We evaluated consecutive subjects with suspected untreated OSA using multivariate analysis to test the associations between apnea-hypopnea index (AHI) and hematocrit. Subjects were evaluated with sleep studies, comprehensive sleep questionnaires, and detailed electronic medical record reviews to document their medical comorbidities, and demographic and laboratory information.
RESULTS: 1604 consecutive veterans (age 57.6 ± 13.4 years, 92% male) were included in the analysis with 77.4% diagnosed with OSA. However, few included subjects (1.6%) had clinical erythrocytosis. OSA severity defined by AHI was not associated with hematocrit or clinically significant erythrocytosis. Rather, awake oxygen saturation (-0.17 points, p < 0.001) and mean nocturnal oxygen saturation (-0.08 points, p = 0.04) were inversely proportional to hematocrit (per standardized Z-score). Other factors including active tobacco, increased alcohol ingestion and exogenous testosterone therapy were associated with higher hematocrit. Although AHI was not predictive of erythrocytosis, having severe OSA was predictive of nocturnal hypoxemia (adjusted OR 7.4, p < 0.001).
CONCLUSIONS: Hematocrit levels and presence of erythrocytosis appear not associated with OSA severity, but rather with hypoxemia as measured by awake and to a lesser extent mean nocturnal oxygen saturation. Nocturnal oximetry may provide diagnostic utility in the evaluation of unexplained secondary polycythemia and polysomongraphy may be warranted in those with unexplained nocturnal hypoxemia and erythrocytosis.

Published by Elsevier Ltd.
PMID 29206630  Respir Med. 2017 Sep;130:27-34. doi: 10.1016/j.rmed.201・・・
著者: Samuel J Ohlander, Bibin Varghese, Alexander W Pastuszak
雑誌名: Sex Med Rev. 2018 Jan;6(1):77-85. doi: 10.1016/j.sxmr.2017.04.001. Epub 2017 May 16.
Abstract/Text INTRODUCTION: A rapid increase in awareness of androgen deficiency has led to substantial increases in prescribing of testosterone therapy (TTh), with benefits of improvements in mood, libido, bone density, muscle mass, body composition, energy, and cognition. However, TTh can be limited by its side effects, particularly erythrocytosis. This review examines the literature on testosterone-induced erythrocytosis and polycythemia.
AIM: To review the available literature on testosterone-induced erythrocytosis, discuss possible mechanisms for pathophysiology, determine the significance of formulation, and elucidate potential thromboembolic risk.
METHODS: A literature review was performed using PubMed for articles addressing TTh, erythrocytosis, and polycythemia.
MAIN OUTCOME MEASURES: Mechanism, pharmacologic contribution, and risk of testosterone-induced erythrocytosis.
RESULTS: For men undergoing TTh, the risk of developing erythrocytosis compared with controls is well established, with short-acting injectable formulations having the highest associated incidence. Potential mechanisms explaining the relation between TTh and erythrocytosis include the role of hepcidin, iron sequestration and turnover, erythropoietin production, bone marrow stimulation, and genetic factors. High blood viscosity increases the risk for potential vascular complications involving the coronary, cerebrovascular, and peripheral vascular circulations, although there is limited evidence supporting a relation between TTh and vascular complications.
CONCLUSION: Short-acting injectable testosterone is associated with greater risk of erythrocytosis compared with other formulations. The mechanism of the pathophysiology and its role on thromboembolic events remain unclear, although some data support an increased risk of cardiovascular events resulting from testosterone-induced erythrocytosis. Ohlander SJ, Varghese B, Pastuszak AW. Erythrocytosis Following Testosterone Therapy. Sex Med Rev 2018;6:77-85.

Copyright © 2017 International Society for Sexual Medicine. Published by Elsevier Inc. All rights reserved.
PMID 28526632  Sex Med Rev. 2018 Jan;6(1):77-85. doi: 10.1016/j.sxmr.2・・・
著者: Demetrios V Vlahakos, Katerina P Marathias, Basil Agroyannis, Nicolaos E Madias
雑誌名: Kidney Int. 2003 Apr;63(4):1187-94. doi: 10.1046/j.1523-1755.2003.00850.x.
Abstract/Text Posttransplant erythrocytosis (PTE) is defined as a persistently elevated hematocrit to a level greater than 51% after renal transplantation. It occurs in 10% to 15% of graft recipients and usually develops 8 to 24 months after engraftment. Spontaneous remission of established PTE is observed in one fourth of the patients within 2 years from onset, whereas in the remaining three fourths it persists for several years, only to remit after loss of renal function from rejection. Predisposing factors include male gender, retention of native kidneys, smoking, transplant renal artery stenosis, adequate erythropoiesis prior to transplantation, and rejection-free course with well-functioning renal graft. Just as in other forms of erythrocytosis, a substantial number (approximately 60%) of patients with PTE experience malaise, headache, plethora, lethargy, and dizziness. Thromboembolic events occur in 10% to 30% of the cases; 1% to 2% eventually die of associated complications. Posttransplant erythrocytosis results from the combined trophic effect of multiple and interrelated erythropoietic factors. Among them, endogenous erythropoietin appears to play the central role. Persistent erythropoietin secretion from the diseased and chronically ischemic native kidneys does not conform to the normal feedback regulation, thereby establishing a form of "tertiary hypererythropoietinemia." However, erythropoietin levels in most PTE patients still remain within the "normal range," indicating that erythrocytosis finally ensues by the contributory action of additional growth factors on erythroid progenitors, such as angiotensin II, androgens, and insulin-like growth factor 1 (IGF-1). Inactivation of the renin-angiotensin system (RAS) by an angiotensin-converting enzyme (ACE) inhibitor, or an angiotensin II type 1 AT1 receptor blocker represents the most effective, safe, and well-tolerated therapeutic modality.

PMID 12631334  Kidney Int. 2003 Apr;63(4):1187-94. doi: 10.1046/j.1523・・・
著者: E Joanna Baxter, Linda M Scott, Peter J Campbell, Clare East, Nasios Fourouclas, Soheila Swanton, George S Vassiliou, Anthony J Bench, Elaine M Boyd, Natasha Curtin, Mike A Scott, Wendy N Erber, Anthony R Green, Cancer Genome Project
雑誌名: Lancet. 2005 Mar 19-25;365(9464):1054-61. doi: 10.1016/S0140-6736(05)71142-9.
Abstract/Text BACKGROUND: Human myeloproliferative disorders form a range of clonal haematological malignant diseases, the main members of which are polycythaemia vera, essential thrombocythaemia, and idiopathic myelofibrosis. The molecular pathogenesis of these disorders is unknown, but tyrosine kinases have been implicated in several related disorders. We investigated the role of the cytoplasmic tyrosine kinase JAK2 in patients with a myeloproliferative disorder.
METHODS: We obtained DNA samples from patients with polycythaemia vera, essential thrombocythaemia, or idiopathic myelofibrosis. The coding exons of JAK2 were bidirectionally sequenced from peripheral-blood granulocytes, T cells, or both. Allele-specific PCR, molecular cytogenetic studies, microsatellite PCR, Affymetrix single nucleotide polymorphism array analyses, and colony assays were undertaken on subgroups of patients.
FINDINGS: A single point mutation (Val617Phe) was identified in JAK2 in 71 (97%) of 73 patients with polycythaemia vera, 29 (57%) of 51 with essential thrombocythaemia, and eight (50%) of 16 with idiopathic myelofibrosis. The mutation is acquired, is present in a variable proportion of granulocytes, alters a highly conserved valine present in the negative regulatory JH2 domain, and is predicted to dysregulate kinase activity. It was heterozygous in most patients, homozygous in a subset as a result of mitotic recombination, and arose in a multipotent progenitor capable of giving rise to erythroid and myeloid cells. The mutation was present in all erythropoietin-independent erythroid colonies.
INTERPRETATION: A single acquired mutation of JAK2 was noted in more than half of patients with a myeloproliferative disorder. Its presence in all erythropoietin-independent erythroid colonies demonstrates a link with growth factor hypersensitivity, a key biological feature of these disorders.
RELEVANCE TO PRACTICE: Identification of the Val617Phe JAK2 mutation lays the foundation for new approaches to the diagnosis, classification, and treatment of myeloproliferative disorders.

PMID 15781101  Lancet. 2005 Mar 19-25;365(9464):1054-61. doi: 10.1016/・・・
著者: Eric Lippert, Marjorie Boissinot, Robert Kralovics, François Girodon, Irène Dobo, Vincent Praloran, Nathalie Boiret-Dupré, Radek C Skoda, Sylvie Hermouet
雑誌名: Blood. 2006 Sep 15;108(6):1865-7. doi: 10.1182/blood-2006-01-013540. Epub 2006 May 25.
Abstract/Text We determined the allelic frequency of the JAK2-V617F mutation in DNA and assessed the expression levels of the mutant and wild-type JAK2 mRNA in granulocytes from 60 patients with essential thrombocythemia (ET) and 62 patients with polycythemia vera (PV) at the time of diagnosis. Using allele-specific quantitative polymerase chain reaction (qPCR), we detected JAK2-V617F in 75% of ET and 97% of PV at diagnosis. The total JAK2 mRNA levels were elevated in ET, PV, and secondary and idiopathic erythrocytosis, suggesting that hyperactive hematopoiesis alters JAK2 expression. The expression levels of JAK2-V617F mRNA were variable but strongly correlated with the allelic ratio of JAK2-V617F determined in DNA. Thus, differences in JAK2-V617F expression, markedly lower in ET than in PV, reflected different percentages of granulocytes carrying the mutation. Moreover, allelic ratios higher than 50% JAK2-V617F, indicating the presence of granulocytes homozygous for JAK2-V617F, were found in 70% of PV at diagnosis but never in ET.

PMID 16728702  Blood. 2006 Sep 15;108(6):1865-7. doi: 10.1182/blood-20・・・
著者: Tiziano Barbui, Jürgen Thiele, Heinz Gisslinger, Guido Finazzi, Alessandra Carobbio, Elisa Rumi, Maria Luigia Randi, Irene Betozzi, Alessandro M Vannucchi, Lisa Pieri, Valentina Carrai, Bettina Gisslinger, Leonhard Müllauer, Marco Ruggeri, Alessandro Rambaldi, Ayalew Tefferi
雑誌名: Am J Hematol. 2014 Jan;89(1):52-4. doi: 10.1002/ajh.23585. Epub 2013 Sep 19.
Abstract/Text We examined the baseline features and clinical outcomes of 140 patients presenting with JAK2V617F positivity and a bone marrow morphology conforming with WHO criteria of polycythemia vera (PV), but a hemoglobin level of <18.5 g/dL in males (range 16.0-18.4) and <16.5 g/dL in females (range 15.0-16.4). This cohort operationally referred to as masked PV (mPV) was compared with 257 patients with overt PV and displayed male predominance, a more frequent history of arterial thrombosis and thrombocytosis. Incidence of thrombosis was similar between the two groups but mPV displayed significantly higher rates of progression to myelofibrosis and acute leukemia and inferior survival. In multivariable analysis mPV diagnosis was an independent predictor of poor survival along with age >65 years and leukocyte count >10 × 10(9) /L. Our data suggest that mPV is a heterogeneous myeloproliferative neoplasia and not necessarily an early/ pre-polycythemic form of classical PV that at onset in a small fraction of patients clinically may mimic essential thrombocythemia. On the other hand, the majority mPV may have a longer prodrome of undiagnosed PV or a disease biology akin to primary myelofibrosis-post PV myelofibrosis that could explain the worsening of outcome in comparison to overt/classical manifestations.

Copyright © 2013 Wiley Periodicals, Inc.
PMID 23996471  Am J Hematol. 2014 Jan;89(1):52-4. doi: 10.1002/ajh.235・・・
著者: Pascal Mossuz, François Girodon, Magali Donnard, Véronique Latger-Cannard, Irène Dobo, Nathalie Boiret, Jean Claude Lecron, Christine Binquet, Claire Barro, Sylvie Hermouet, Vincent Praloran
雑誌名: Haematologica. 2004 Oct;89(10):1194-8.
Abstract/Text BACKGROUND AND OBJECTIVES: The diagnosis of polycythemia vera (PV) is based on clinical and biological criteria defined by either the Polycythemia Vera Study Group (PVSG) or the World Health Organization (WHO). Both the PVSG and WHO PV criteria have proved helpful and are extensively used, yet diagnostic strategies and scheduling of biological investigations vary. We assessed the value of measuring serum erythropoietin (Epo) as a first intention diagnostic test in patients with absolute erythrocytosis (AE).
DESIGN AND METHODS: Serum and bone marrow (BM) samples of 241 patients with a suspicion of erythrocytosis were collected in 8 hospital centers. One hundred and ninety had an absolute erythrocytosis (116 had PV, 66 had secondary erythrocytosis and 4 had idiopathic erythrocytosis). Serum Epo was assayed (ELISA) in 186. Statistical analysis (ROC curves) was used to define serum Epo thresholds that were specific for PV and secondary erythrocytosis and to analyze the diagnostic value of a low or high serum Epo level.
RESULTS: A large majority of PV patients (87% or 101/116) had a serum Epo level below the normal range in healthy patients (3.3 IU/L), giving this value a specificity of 97% with a 97.8% positive predictive value for the diagnosis of PV. Statistical analysis (ROC curves) defined two thresholds allowing a specific and direct diagnosis of 65.6% (65/99) of untreated PV (Epo < 1.4 IU/L) and 19.7% (13/66) of those with secondary erythrocytosis (Epo > 13.7 IU/L).
INTERPRETATION AND CONCLUSIONS: Based on these data, we propose that measurement of serum Epo level, a simple, reliable and inexpensive test, should be considered as a first intention diagnostic test for patients with absolute erythrocytosis.

PMID 15477203  Haematologica. 2004 Oct;89(10):1194-8.
著者: A F Remacha, I Montserrat, A Santamaria, A Oliver, M J Barceló, M Parellada
雑誌名: Haematologica. 1997 Jul-Aug;82(4):406-10.
Abstract/Text BACKGROUND AND OBJECTIVE: It has been suggested that the determination of serum erythropoietin (sEpo) may be useful in distinguishing between polycythemia vera (PV), relative polycythemia and secondary polycythemia (SP), but no conclusive evidence has yet been provided for this. In the present work, we evaluated the role of sEpo in the differential diagnosis of polycythemia vera and its usefulness in the follow-up of PV patients.
METHODS: sEpo was assessed in 190 patients with polycythemia of different etiologies. A follow-up study was carried out in some of these patients (27 with secondary polycythemia and 17 with polycythemia vera).
RESULTS: sEpo levels were higher in SP than in PV and relative polycythemia. There were, however, differences with regard to the various etiologies of SP. Polycythemia related to congenital heart disorders showed the highest levels of sEpo of the SP. When a study was conducted, sEpo alone as a diagnostic parameter displayed an efficiency of more than 90% and the most discriminating value was 5 U/L. Using lower levels (below 2 U/L) and higher levels (above 12 U/L), it was possible to distinguish between SP and PV, although an important overlap was detected between these limits (approximately 50% of cases). The follow-up study showed that in half the patients with SP the levels of sEpo were at times < 12 U/L and at other times greater than this value. At least three determinations were necessary to detect an elevated reading. In PV after venesection there was an increase in sEpo in some cases, although most of the time there was no change.
INTERPRETATION AND CONCLUSIONS: Using sEpo, it was possible to differentiate between PV and SP, despite an important overlap. A follow-up study demonstrated that the increase in sEpo was intermittent in SP and that in many of these cases more than one determination could be helpful.

PMID 9299851  Haematologica. 1997 Jul-Aug;82(4):406-10.

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