Essential thrombocythemia or chronic idiopathic myelofibrosis? A single-center study based on hematopoieticbone marrow histology

Gianelli, Umberto; Vener, Claudia; Raviele, Paola Rafaniello; Moro, Alessia; Savi, Federica; Annaloro, C.; Somalvico, Francesco; Radaelli, Franca; Franco, Vito; Deliliers, Giorgio Lambertenghi

doi:10.1080/10428190600678975

Cited by 56 publications

(60 citation statements)

References 14 publications

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“…2C,D) as well as an increased frequency of bare (denuded) nuclei * About 80% of this cohort reveal a platelet count in excess of 600 3 10 9 /l, and therefore, may mimic ET when considering the relevant PVSG criteria [5,7,21]. [93,94,96,97,102,107]. All these histopathological anomalies of megakaryopoiesis are definitely more conspicuous in PMF than in ET (compare with Fig.…”

Section: Primary Myelofibrosismentioning

confidence: 82%

“…Considering the WHO criteria and the proposals for a revised classification [1,2,10], one has to realize that significant differences exist in comparison with the diagnostic guidelines of the PVSG [92,[96][97][98]. Unfortunately, the PVSG [5,7,21] and other classification systems [8,9] do not regard BM morphology as one of the yardsticks for diagnosis.…”

Section: Essential Thrombocythemiamentioning

confidence: 99%

“…An important feature is the large to giant cell forms with extensively folded (hyperlobulated) nuclei (Fig. 1C,D) surrounded by well differentiated (mature) cytoplasm [96,97,99,100]. Any increase in the reticulin fibers, i.e., myelofibrosis (MF) is not compatible with early stage ET [98,101].…”

Section: Essential Thrombocythemiamentioning

confidence: 99%

“…Any increase in the reticulin fibers, i.e., myelofibrosis (MF) is not compatible with early stage ET [98,101]. Contrasting prefibrotic PMF with accompanying thrombocytosis which is frequently confused with ET [92][93][94][95][96][97], neither a relevant increase in age-matched cellularity nor a significantly expressed left-shifted neutrophil granulopoiesis is found (compare Fig. 1C with Fig.…”

Section: Essential Thrombocythemiamentioning

confidence: 99%

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Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Kvasnicka

Thiele

2009

American J Hematol

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The concept of prodromal chronic myeloproliferative neoplasms has been endorsed by the WHO classification implicating a stepwise evolution of disease. Histology of the bone marrow (BM) and borderline to mildly expressed clinical features play a pivotal role for diagnosing prefibrotic-early primary myelofibrosis. By lowering the platelet count for essential thrombocythemia and regarding BM morphology, early manifestations are tackled. Pre-polycythemic stages of polycythemia vera with a low hemoglobin level at onset are diagnosed by positive JAK2V617F mutation status, a low erythropoietin value, and characteristic BM features. The revised WHO classification incorporates hematological, morphological, and moleculargenetic parameters to generate a consensus-based working diagnosis. Am. J. Hematol. 85:62-69, 2010. V

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Section: Primary Myelofibrosismentioning

confidence: 82%

Section: Essential Thrombocythemiamentioning

confidence: 99%

Section: Essential Thrombocythemiamentioning

confidence: 99%

Section: Essential Thrombocythemiamentioning

confidence: 99%

See 2 more Smart Citations

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Kvasnicka

Thiele

2009

American J Hematol

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“…Incidences may vary depending on the accuracy of applied diagnostic guidelines [4][5][6] and was recently reported in about 18% of cases of socalled ET [7]. Laboratory tests which are significantly different in early PMF as compared with histologically confirmed ET (WHO-ET) include decreased Hb, increased white blood cell (WBC) and lactate dehydrogenase (LDH) values.…”

mentioning

confidence: 99%

Blood tests may predict early primary myelofibrosis in patients presenting with essential thrombocythemia

et al. 2012

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According to World Health Organization (WHO)-defined criteria, patients presenting clinically as essential thrombocythemia (ET) may show early primary myelofibrosis (PMF) with accompanying thrombocythemia [1]. Previous clinicopathological studies revealed that laboratory parameters like gender-matched hemoglobin (Hb), white blood cell (WBC) count, and particularly lactate dehydrogenase (LDH) values are significantly different in PMF [2]. By strictly applying the WHO criteria, our investigation was aimed to study sensitivity and specificity of these features in an exploratory cohort of 536 patients and to validate the results on an independently recruited series of 321 strictly corresponding patients. The discriminatory power of these parameters (Hb, WBC, and LDH) was tested by plotting their receiver operating characteristic curves. The best performance was found for LDH (areas under the curve, AUC 5 0.7059). WBC and Hb had superimposable curves, with AUC of 0.6279 and 0.6257, respectively. A diagnostic algorithm was generated by applying these parameters in a stepwise fashion. Nearly half of the patients could be correctly allocated to WHOdefined ET or early PMF in both cohorts investigated. It is important to note that this result does not substitute bone marrow morphology with hematological parameters, however, in clinical practice may alert physicians to get more suspicious of early PMF in a patient presumably presenting with ET.Following current criteria established by the World Health Organization (WHO), patients presenting with a clinical picture of essential thrombocythemia (ET) can actually have an early primary myelofibrosis (PMF) [3]. Incidences may vary depending on the accuracy of applied diagnostic guidelines [4][5][6] and was recently reported in about 18% of cases of socalled ET [7]. Laboratory tests which are significantly different in early PMF as compared with histologically confirmed ET (WHO-ET) include decreased Hb, increased white blood cell (WBC) and lactate dehydrogenase (LDH) values. Although bone marrow biopsy represents the gold standard for differentiating WHO-ET from early PMF [8], in clinical practice it might be very useful to know whether one or more of these baseline laboratory parameters may help to differentiate these two entities. To tackle this issue, we evaluated sensitivity (SE) and specificity (SP) of blood cells counts and LDH for the diagnosis of early PMF versus WHO-ET in an exploratory cohort of 536 histologically diagnosed patients aimed to propose an algorithm for the practical clinical use of these tests. This algorithm was then applied to an independently recruited validation cohort of 321 patients presenting with similar diagnosis of early PMF versus WHO-ET. The starting point of this study was an international database of 1,071 patients with ET either confirmed by WHO criteria (891 cases) or revised to early PMF (180 cases) as detailed elsewhere [7]. From this database, 536 patients (50%) who had complete laboratory data measured at diagnosis were extracted and c...

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Leukocytosis as an important risk factor for arterial thrombosis in WHO‐defined early/prefibrotic myelofibrosis: An international study of 264 patients

et al. 2012

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We aimed to determine risk factors for thrombotic events in early/prefibrotic myelofibrosis diagnosed according to the World Health Organization criteria. Multivariate Cox regression analysis was calculated on a total number of 264 patients derived from an international database. After a median follow‐up of 6.28 years, 42 (15.9%) patients experienced arterial (n = 31) or venous thrombosis (n = 11). A higher leukocyte count correlated with an increased risk for total thrombosis and in particular, with an increased risk for arterial thrombosis (P = 0.005, HR 1.15 and P = 0.047, HR 1.12, respectively). A platelet count above 870 × 109/L was associated with a lower risk for total thrombosis and also for venous thrombosis (P = 0.022, HR 0.44 and P = 0.027, HR 0.19). Moreover, a lower hemoglobin level was associated with an increased risk for venous thrombosis (P = 0.007, HR 0.59). Our data indicate that leukocytosis is a prominent risk factor for thrombosis in early/prefibrotic MF. Am. J. Hematol. 87:669–672, 2012. © 2012 Wiley Periodicals, Inc.

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Essential thrombocythemia or chronic idiopathic myelofibrosis? A single-center study based on hematopoieticbone marrow histology

Cited by 56 publications

References 14 publications

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Prodromal myeloproliferative neoplasms: The 2008 WHO classification

Blood tests may predict early primary myelofibrosis in patients presenting with essential thrombocythemia

Leukocytosis as an important risk factor for arterial thrombosis in WHO‐defined early/prefibrotic myelofibrosis: An international study of 264 patients

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