An elevated venous haemoglobin concentration cannot be used as a surrogate marker for absolute erythrocytosis: a study of patients with polycythaemia vera and apparent polycythaemia

Johansson, Peter; Safai‐Kutti, Soodabeh; Kutti, Jack

doi:10.1111/j.1365-2141.2005.05517.x

Cited by 98 publications

(74 citation statements)

References 11 publications

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“…9 It is well known that a proportion of patients suffering from PV do not reach the Hb threshold defined by the WHO. [10][11][12] This is extremely important in those cases with concomitant thrombocytosis. In these cases, if RCM is not measured, the majority of patients may be erroneously classified as ET.…”

Section: Introductionmentioning

confidence: 99%

Red cell mass measurement in patients with clinically suspected diagnosis of polycythemia vera or essential thrombocythemia

et al. 2012

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diagnosis of PV required hemoglobin level over 18.5 g/dL in males and over 16.5 g/dL in females, or an increased red cell volume of over 125% of normal values, the demonstration of a mutation in the JAK2 gene, and one minor criteria (decreased erythropoietin serum level, endogenous erythroid colony formation or compatible bone marrow histology). JAK2-negative cases required the presence of at least two minor criteria to establish PV diagnosis. Bone marrow biopsy was performed in all patients with suspicion of ET but it was not routinely assessed in PV. Patients with early/pre-fibrotic primary myelofibrosis were not included in the present study. Informed consent was obtained for the scientific use of the patients' clinico-hematologic data and this was approved by the institutional review board of the Hospital del Mar.Receiving operating characteristic (ROC) curves were performed to evaluate the diagnostic accuracy of Hb and Hct in order to distinguish between normal and increased RCM measured by the Cr51 method. In ROC curves, the specificity and sensitivity of each Hb and Hct value is calculated. The area under the curve (AUC) of a perfect diagnostic test (sensitivity 100%, specificity 100%) is 1 whereas the AUC of a test without diagnostic accuracy is 0.5 (sensitivity 50%, specificity 50%). The best diagnostic test is that with a higher AUC. A diagnostic test is usually considered to have an acceptable diagnostic accuracy when the sensitivity and specificity is higher than 80%, resulting in an AUC of over 0.8. Since the purpose of the present study was to investigate which Hb or Hct value should indicate measurement of RCM (as opposed to not measuring it), the cut off was selected according to sensitivity prevailing over specificity in order to reduce the number of false negative cases. In the differential diagnosis of ET versus PV, false positives represent cases with an Hct or Hb value over a pre-determined threshold but with normal RCM leading to an erroneous diagnosis of PV if RCM is not measured. False negatives (cases with Hct or Hb values below the pre-determined threshold and an increased red cell mass) would correspond to those patients in whom a mistaken diagnosis of ET would be made if RCM was not measured. Results and DiscussionIsotopic RCM was determined as part of the initial evaluation in 179 patients (88 males, 91 females) with a suspected diagnosis of PV or ET. Main hematologic values at diagnosis are shown in Table 1. The majority of patients showed a Hb level and/or platelet counts over the normal values and the JAK2 mutation (V617F or exon 12) was present in 98% of the cases; a clinical picture compatible with PV or ET. RCM was increased in 114 patients establishing a PV diagnosis, whereas ET was diagnosed in 63 of the 65 remaining cases. Two cases with normal RCM did not fulfill ET nor PV criteria at time of evaluation but were diagnosed with PV later on during follow up.The diagnostic accuracy of the WHO Hb criteria is shown in Table 2. WHO Hb criteria showed a high specificity i...

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Section: Introductionmentioning

confidence: 99%

Red cell mass measurement in patients with clinically suspected diagnosis of polycythemia vera or essential thrombocythemia

et al. 2012

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“…In a series of 77 consecutive patients (31 males and 46 females) with PV in the study of Johansson et al only 35% of male and 63% of female PV patients had Hb values above 18.5 and 16.5 g/dL respectively 21 . Laboratory features at diagnosis of 266 PV and 381 ET patients diagnosed according to Table 7: The Basel data in 13 cases with documented MPN in bone marrow biopsy and 5 case of erythrocytosis: direct comparison and erythrocytes, serum EPO and RCM.…”

Section: Discussionmentioning

confidence: 99%

“…Four studies showed that WHO defined elevated hemoglobin concentration cannot be used as a surrogate marker for absolute erythrocytosis in PV patients indicating the need that RCM is mandatory for patients who do not meet the WHO defined, rather crude hemoglobin and hematocrit value [21][22][23][24]. In a series of 77 consecutive patients (31 males and 46 females) with PV in the study of Johansson et al only 35% of male and 63% of female PV patients had Hb values above 18.5 and 16.5 g/dL respectively 21 .…”

Section: Discussionmentioning

confidence: 99%

Increased Erythrocyte Count on Top of Bone Marrow Histology but not Serum EPO Level or JAK2 Mutation Load Discriminates between JAK2V617F Mutated Essential Thrombocythemia and Polycythemia Vera

Michiels¹,

Medinger²

2015

J Hematol Thrombo Dis

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“…Four studies showed that WHO defined elevated hemoglobin concentration cannot be used as a surrogate marker for absolute erythrocytosis in PV patients indicating the need by the PVSG and WHO investigators that RCM is mandatory for patients who do not meet the WHO defined crude hemoglobin and hematocrit values [20][21][22][23]. In a series of 77 consecutive patients (31 males and 46 females) with PV in the study of Johansson et al, only 35% of male and 63% of female PV patients had Hb values above 18.5 and 16.5 g/dL respectively [20]. The laboratory features at diagnosis of PVSG defined PV and ET patients from a nation-wide survey of 647 patients with MPN disease in Japan are shown in Table 7 [23].…”

Section: Discussionmentioning

confidence: 99%

“…We assessed the relation between RCM, erythrocyte count and bone marrow histology findings at time of diagnosis (Tables 4 and 5) in 12 ET and 14 PV cases with no or minor splenomegaly at time of PV as compared to the 2008 WHO cut-of levels of hemoglobin (Hb) and hematocrit (Ht) for PV: Hb >18.5 g/dl and Ht >0.60 in men and Hb>16.5 and Ht >0.56 in women for the diagnosis of PV (Table 6) [12,[20][21][22][23][24]. At RCM above 30 ml/kg the erythrocytes are above 5.8×10 12 /L in 3 of 10 ET and all 16 PV patients.…”

Section: Rcm and Red Cell Counts On Top Of Bone Marrow Histology Dismentioning

confidence: 99%

The Erythrocyte Count on Top of Bone Marrow Histology Discriminates Essential Thrombocythemia and Polycythemia Vera in JAK2v617f Mutated Prefibrotic Myeloproliferative Neoplasm with No or Minor Splenomegaly

Michiels¹

2014

J Hematol Thrombo Dis

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Blood and bone features in JAK2 V617F mutated prefibrotic essential thrombocythemia (ET) and polycythemia vera (PV) are overlapping in terms of increased cellularity due to increased erythropoiesis and pleomorphic megakaryocytes indicating the need to measure red cell mass (RCM) according to PVSG and WHO criteria. The JAK2 V617F mutated myeloproliferative neoplasms (MPN) appeared to be a broad biological continuum of normocellular ET, ET with features of polycythemia vera (prodromal PV), classical PV, advanced PV, Inapparent PV (IPV) with splenomegly, masked PV or hypercellular ET due to megakaryocytic granulocytic myeloproliferation (ET.MGM) when the 2013 WHO and European Clinical, Molecular and Pathological (WHO-CMP) criteria are applied. The megakaryocytes morphology may change from pleomorphic to dysmorphic in advanced PV, in IPV and in masked PV (ET.MGM) as bone marrow cellularity, the degree myelofibrosis and the JAK2 V617F mutation load increase during long-term follow-up. Erythrocytes above the upper limit of normal (5.8×10 12 /L), but not hemoglobin and hematocrit is clearly correlated with increased red cell mass (RCM) above (30) ml/kg) and therefore diagnostic for PV and idiopathic erythrocythemia (IE) on top of pathognomonic MPN bone marrow histology in newly diagnosed PV patients with normal mean cell volume (MCV) of erythrocytes and no or minor of splenomegaly. Erythrocyte count remain above the upper limit of normal in PV and IE in complete hematological remission by phlebotomy alone as the consequence of iron deficiency induced microcytic erythrocytes that correct the blood volume (RCM), hemoglobin and hematocrit to normal. The combination of increased RCM, increased plasma volume, and normal or low erythrocyte counts is characteristic for IPV with significant splenomegaly as the only cause of increased blood volume without symptoms of hypervolumemia.

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An elevated venous haemoglobin concentration cannot be used as a surrogate marker for absolute erythrocytosis: a study of patients with polycythaemia vera and apparent polycythaemia

Cited by 98 publications

References 11 publications

Red cell mass measurement in patients with clinically suspected diagnosis of polycythemia vera or essential thrombocythemia

Red cell mass measurement in patients with clinically suspected diagnosis of polycythemia vera or essential thrombocythemia

Increased Erythrocyte Count on Top of Bone Marrow Histology but not Serum EPO Level or JAK2 Mutation Load Discriminates between JAK2V617F Mutated Essential Thrombocythemia and Polycythemia Vera

The Erythrocyte Count on Top of Bone Marrow Histology Discriminates Essential Thrombocythemia and Polycythemia Vera in JAK2v617f Mutated Prefibrotic Myeloproliferative Neoplasm with No or Minor Splenomegaly

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