Classification of myeloproliferative disorders in the JAK2 era: is there a role for red cell mass?

Cassinat, Bruno; Laguillier, Christelle; Gardin, Claude; Beco, V. De; Burcheri, Sara; Fenaux, Pierre; Chomienne, Christine; Kiladjian, Jean‐Jacques

doi:10.1038/sj.leu.2404908

Cited by 42 publications

(28 citation statements)

References 8 publications

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“…Indeed, based on a recent publication by Cassinat et al, 80 the new WHO dictum that red cell mass and plasma volume studies are not required in the evaluation of isolated thrombocytosis 1 is invalid. These authors found that red cell mass and plasma volume determinations identified erythrocytosis in 46% of patients initially considered to have ET by the WHO hemoglobin criteria, but when the JAK2 V617F mutation was present, the proportion rose to 64%.…”

Section: Jak2 V617fmentioning

confidence: 99%

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The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal

Spivak

Silver

2008

Blood

164

140

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Section: Jak2 V617fmentioning

confidence: 99%

“…These authors found that red cell mass and plasma volume determinations identified erythrocytosis in 46% of patients initially considered to have ET by the WHO hemoglobin criteria, but when the JAK2 V617F mutation was present, the proportion rose to 64%. 80 An example of such a patient is provided in Table 1.…”

Section: Jak2 V617fmentioning

confidence: 99%

The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal

Spivak

Silver

2008

Blood

164

140

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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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“…Twenty-three percent of patients were detected during a checkup. In a study by Cassinat [14], polycythemia vera was also discovered incidentally in 46.11% of cases. These systematic discovery circumstances of PV, both in our study and in the literature [18] imply the importance of achieving blood counts routinely in our populations.…”

Section: Discussionmentioning

confidence: 95%

Profile of Polycythemia Vera in Campus Teaching Hospital of Lome - Togo

Padaro¹

2016

CRJ

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The annual prevalence of polycythemia vera is about 2.16 new cases. The average age of patients was 46, 7 years with ranges from 31 to 65 years. There were 9 men and 4 women. The most frequent factor of finding was the fortuitous ones (23%). Most clinical signs represented were headache (69.2%), skin érythroses (53.8%) and paresthesia (30.8%). The average value of hemoglobin, hematocrit, the number of white blood cells and platelets in our patients with polycythemia who had positive JAK2V617F was respectively: 198g/L ± 44; 62.7 ± 4.4%; 9

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Classification of myeloproliferative disorders in the JAK2 era: is there a role for red cell mass?

Cited by 42 publications

References 8 publications

The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal

The revised World Health Organization diagnostic criteria for polycythemia vera, essential thrombocytosis, and primary myelofibrosis: an alternative proposal

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

Profile of Polycythemia Vera in Campus Teaching Hospital of Lome - Togo

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