Classification of Myeloproliferative Disorders by Bone Marrow Histology

2015 ◽  
pp. 57-80
Author(s):  
B. Frisch ◽  
R. Bartl ◽  
R. Burkhardt ◽  
K. J�ger ◽  
G. Mahl ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Myeloproliferative Disorders ◽  
Bone Marrow Histology

JAK2V617F, CALR, and MPL Mutations and Bone Marrow Histology in Patients with Essential Thrombocythaemia

2018 ◽  
Vol 140 (4) ◽  
pp. 234-239 ◽  
Author(s):  
Achille Pich ◽  
Ludovica Riera ◽  
Paola Francia di Celle ◽  
Eloise Beggiato ◽  
Giulia Benevolo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Prognostic Value ◽  
Triple Negative ◽  
Morphological Changes ◽  
Gene Mutations ◽  
Essential Thrombocythaemia ◽  
Bone Marrow Histology ◽  
Marrow Cellularity ◽  
Morphological Patterns

Introduction: Mutations in the JAK2, CALR, and MPL genes have been shown to have prognostic value in essential thrombocythaemia (ET), but no clear association with morphological changes has been reported so far. We investigated the possible correlation between gene mutations and histopathological features in bone marrow (BM) biopsies of patients with ET. Methods: Marrow cellularity, fibrosis, and the number of total and dysmorphic megakaryocytes and clusters of megakaryocytes were compared to gene mutations in 90 cases of ET at diagnosis. Results: The JAK2V617F mutation was found in 58.9%, CALR in 28.9%, and MPL in 4.4% of the cases, and 7.8% were triple-negative. JAK2V617F-mutated ET showed a high BM cellularity, the lowest number of clusters of megakaryocytes and the highest number of dysmorphic megakaryocytes; CALR-mutated ET showed a reduced BM cellularity, many clusters of large megakaryocytes, and very few dysmorphic megakaryocytes; MPL-mutated ET showed the lowest BM cellularity, the highest number of clustered and large megakaryocytes, and the lowest number of dysmorphic megakaryocytes. Triple-negative ET cases had the highest BM cellularity. Conclusions: Distinct morphological patterns were associated with gene mutations in ET, supporting the classification of ET into different subtypes.

Prefibrotic myelofibrosis: is this diagnosis valid?

Blood ◽  
2008 ◽  
Vol 111 (1) ◽  
pp. 1-2 ◽  
Author(s):  
LoAnn Peterson
Keyword(s):  
Bone Marrow ◽  
World Health Organization ◽  
Diagnostic Criteria ◽  
Essential Thrombocythemia ◽  
World Health ◽  
Substantial Variation ◽  
Bone Marrow Histology ◽  
The World ◽  
Health Organization

Wilkins and colleagues evaluate the utility of the World Health Organization (WHO) diagnostic criteria intended to separate cases previously classified as essential thrombocythemia (ET) into 2 groups: “true ET” and “prefibrotic myelofibrosis.” Focusing on bone marrow histology, the authors found substantial variation in classification of cases.

Increased Response to Arachidonic Acid and U-46619 and Resistance to Inhibitory Prostaglandins in Patients with Chronic Myeloproliferative Disorders

1988 ◽  
Vol 59 (01) ◽  
pp. 073-076 ◽  
Author(s):  
Sergio Cortelazzo ◽  
Monica Galli ◽  
Donatella Castagna ◽  
Piera Viero ◽  
Giovanni de Gaetano ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Arachidonic Acid ◽  
Platelet Aggregation ◽  
Myeloproliferative Disorders ◽  
Bone Marrow Stem Cell ◽  
Healthy Controls ◽  
Aggregation Response ◽  
Thrombotic Complications ◽  
Cyclic Endoperoxide ◽  
Marrow Stem Cell

SummaryIn patients with myeloproliferative disorders (MPD) a group of related diseases of the bone marrow stem cell and recurrent haemorrhagic and/or thrombotic complications, the production of aggregating prostaglandins (PGs) may be normal or slightly reduced, while PGI2 production is normal. However, MPD platelet sensitivity to antiaggregatory PGs is still unknown.We studied the potency of PGD2, PGI2 and PGEi as inhibitors of platelet aggregation induced by threshold aggregating concentrations of arachidonic acid and U-46619-analogue of the cyclic endoperoxide PGH2 in 20 patients with MPD in comparison with healthy controls, with the aim of evaluating the sensitivity of MPD platelets to antiaggregatory PGs. In these patients platelet prostanoid metabolism was normal. However, the functional response of platelets to aggregating and antiaggregating prostanoids was shifted towards potentially increased platelet aggregation response. These findings could have a clinical relevance in view of the haemostatic and thrombotic complications so frequent in MPD.

scholarly journals A review of current knowledge of myeloproliferative disorders in the horse

2021 ◽  
Vol 63 (1) ◽  
Author(s):  
Katy Satué ◽  
Juan Carlos Gardon ◽  
Ana Muñoz
Keyword(s):  
Bone Marrow ◽  
Myeloid Leukemia ◽  
Current Knowledge ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Disorders ◽  
World Health ◽  
Chronic Granulocytic Leukemia ◽  
Current State ◽  
Multiple Cell ◽  
Health Organization

AbstractMyeloid disorders are conditions being characterized by abnormal proliferation and development of myeloid lineage including granulocytes (neutrophils, eosinophils and basophils), monocytes, erythroids, and megakaryocytes precursor cells. Myeloid leukemia, based on clinical presentation and proliferative rate of neoplastic cells, is divided into acute (AML) and myeloproliferative neoplasms (MPN). The most commonly myeloid leukemia reported in horses are AML-M4 (myelomonocytic) and AML-M5 (monocytic). Isolated cases of AML-M6B (acute erythroid leukemia), and chronic granulocytic leukemia have also been reported. Additionally, bone marrow disorders with dysplastic alterations and ineffective hematopoiesis affecting single or multiple cell lineages or myelodysplastic diseases (MDS), have also been reported in horses. MDSs have increased myeloblasts numbers in blood or bone marrow, although less than 20%, which is the minimum level required for diagnosis of AML. This review performed a detailed description of the current state of knowlegde of the myeloproliferative disorders in horses following the criteria established by the World Health Organization.

Epiphyseal bone-marrow abnormalities and restitution in legg-calvé-perthes disease

1997 ◽  
Vol 38 (5) ◽  
pp. 855-862 ◽  
Author(s):  
P. Hochbergs ◽  
G. Eckervall ◽  
H. Wingstrand ◽  
N. Egund ◽  
K. Jonsson
Keyword(s):  
Bone Marrow ◽  
Mr Imaging ◽  
Perthes Disease ◽  
Mr Images ◽  
Group I ◽  
Radiological Classification ◽  
Signal Changes ◽  
Over Time ◽  
Epiphyseal Bone

Purpose: By means of MR imaging, to determine signal abnormalities in the femoral epiphysis; to determine their location, extent and restitution over time; and to correlate these findings to the Catterall radiological classification. Material and Methods: A total of 247 MR images in 86 patients (101 hips) with Legg-CalvC-Perthes disease were examined. The MR images were taken in the coronal plane, and the images through the center of the femoral head were used for this study. Results: T1-weighted images proved as good as T2-weighted images for the MR evaluation of the extent of the necrosis. In almost every case, the central-cranial part of the epiphysis showed a low initial signal. In Catterall group I, the medial part was never involved. In Catterall III and IV, almost the entire epiphysis showed signal changes. In the period 3–6 years after diagnosis, we still found signal changes in the epiphysis in some hips but there was no correlation with the Catterall classification. After 6 years, the epiphysis showed normal signal intensity in MR imaging. In T1-weighted images, Gd-enhancement occurred in the peripheral regions in the early stages of the disease. The central part of the epiphysis became more enhanced over time and peaked in the period 1–3 years after diagnosis. Conclusion: MR is a valuable modality for monitoring changes in the femoral epiphysis. We propose a new classification of the extent and pattern of epiphyseal bone-marrow abnormalities based on the 4 zones most commonly observed in MR imaging.

scholarly journals Reticulin Accumulation in Essential Thrombocythemia: Prognostic Significance and Relationship to Therapy

2009 ◽  
Vol 27 (18) ◽  
pp. 2991-2999 ◽  
Author(s):  
Peter J. Campbell ◽  
David Bareford ◽  
Wendy N. Erber ◽  
Bridget S. Wilkins ◽  
Penny Wright ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Essential Thrombocythemia ◽  
Arterial Thrombosis ◽  
Interobserver Reliability ◽  
Prognostic Significance ◽  
Underlying Disease ◽  
Major Hemorrhage ◽  
Independent Prognostic Marker ◽  
Bone Marrow Histology ◽  
Duration Of Disease

PurposeEssential thrombocythemia (ET) manifests substantial interpatient heterogeneity in rates of thrombosis, hemorrhage, and disease transformation. Bone marrow histology reflects underlying disease activity in ET but many morphological features show poor reproducibility.Patients and MethodsWe evaluated the clinical significance of bone marrow reticulin, a measure previously shown to have relatively high interobserver reliability, in a large, prospectively-studied cohort of ET patients.ResultsReticulin grade positively correlated with white blood cell (P = .05) and platelet counts (P = .0001) at diagnosis. Elevated reticulin levels at presentation predicted higher rates of arterial thrombosis (hazard ratio [HR], 1.8; 95% CI, 1.1 to 2.9; P = .01), major hemorrhage (HR, 2.0; 95% CI, 1.0 to 3.9; P = .05), and myelofibrotic transformation (HR, 5.5; 95% CI, 1.7 to 18.4; P = .0007) independently of known risk factors. Higher reticulin levels at diagnosis were associated with greater subsequent falls in hemoglobin levels in patients treated with anagrelide (P < .0001), but not in those receiving hydroxyurea (P = .9). Moreover, serial trephine specimens in patients randomly assigned to anagrelide showed significantly greater increases in reticulin grade compared with those allocated to hydroxyurea (P = .0003), and four patients who developed increased bone marrow reticulin on anagrelide showed regression of fibrosis when switched to hydroxyurea. These data suggest that patients receiving anagrelide therapy should undergo surveillance bone marrow biopsy every 2 to 3 years and that those who show substantially increasing reticulin levels are at risk of myelofibrotic transformation and may benefit from changing therapy before adverse clinical features develop.ConclusionOur results demonstrate that bone marrow reticulin grade at diagnosis represents an independent prognostic marker in ET, reflecting activity and/or duration of disease, with implications for the monitoring of patients receiving anagrelide.

scholarly journals The Prevalence of Myeloproliferative Disorders in A Group of Iraqi Patients And Its Relation To Blood Indices Parameters

2020 ◽  
Vol 8 (A) ◽  
pp. 660-665
Author(s):  
Marwa Abdulnabi ◽  
Enass Abdul Kareem Dagher Al-Saadi
Keyword(s):  
Bone Marrow ◽  
Blood Cells ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Disorders ◽  
Myelogenous Leukemia ◽  
High Exposure ◽  
Female Ratio ◽  
Blood Samples ◽  
Blood Indices ◽  
Male To Female

AIM: The aim of this study was to measure the prevalence of myeloproliferative disorders in a sample of Iraqi patients and to measure the changes in patients’ blood parameters. BACKGROUND: Myeloproliferative disorders are a group of neoplasms affecting the bone marrow progenitor cells characterized by excess cells with a risk of transforming to acute leukemia. There is a gap in knowledge about the prevalence of Iraqi population. Thus, we investigated the prevalence and distribution of different types of myeloproliferative disorders in a sample of Iraqi patients. MATERIALS AND METHODS: Cross-sectional study is done at the National Center of Hematology from November 2019 till March 2020 on 75 patients who were diagnosed by a specialist hematopathologist to have one subtype of myeloproliferative disorders (MPDs). Blood samples were taken from them and analyzed to get complete blood count, blood film, bone marrow aspirate, and biopsy that were analyzed for each patient. Blood samples were taken from them and analyzed in terms of blood indices, which include red blood cells, white blood cells, and platelets. RESULTS: The 75 patients were found to be comprising 35 chronic myelogenous leukemia (CML) patients (46.7%), myelofibrosis 22 patients (29.3%), essential thrombocythemia (ET) 9 patients (12%), and polycythemia vera (PV) 9 patients (12%). In terms of male/female ratios, they were as follows: Myeloproliferative neoplasms (MPNs) male-to-female ratio is 1.2, CML= 0.94, myelofibrosis= 2.14 and ET= 0.5 and PV male-to-female ratio is 2. CONCLUSIONS : MPN male-to-female ratio in Iraq, which is 1.2, CML is the most common subtype. Regarding myelofibrosis, in our study, the male-to-female ratio is 2.14, which is much higher other countries. This could be attributed to high exposure to benzene and toluene which are well known to be causative agents for myelofibrosis. Regarding ET or PV, the male-to-female ratios were compatible with other countries.

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