Thrombocytosis

2010 ◽  
pp. 4280-4287
Author(s):  
Stefan O. Ciurea ◽  
Ronald Hoffman
Keyword(s):  
Stem Cell ◽  
Platelet Count ◽  
Iron Deficiency ◽  
Blood Loss ◽  
Haematopoietic Stem Cell ◽  
Polycythaemia Vera ◽  
Essential Thrombocythaemia ◽  
Platelet Production ◽  
The Many ◽  
Different Levels

Thrombocytosis describes a platelet count elevated above 450 × 109/litre, which can be (1) primary—including essential thrombocythaemia, chronic myeloid leukaemia, polycythaemia vera and myelodysplastic syndromes; or (2) secondary—including iron deficiency, infection, blood loss, malignancy. Platelets are released from megakaryocytes, whose development is principally regulated by thrombopoietin. This is chiefly produced in the liver and binds to its receptor (c-Mpl) to cause activation via the JAK-STAT signalling pathway at different levels of the platelet production pathway, ranging from the proliferation and survival of haematopoietic stem cell/progenitor cells to megakaryocyte maturation. Thrombopoietin production is increased by a wide variety of stimuli, which explains the many causes of secondary thrombocytosis....

A clinical case of severe iron deficiency anemia in a patient with hiatal hernia

2021 ◽  
pp. 104-109
Author(s):  
D. V. Lukanin ◽  
A. A. Sokolov ◽  
A. A. Sokolov ◽  
M. S. Klimenko
Keyword(s):  
Iron Deficiency ◽  
Blood Loss ◽  
Hiatal Hernia ◽  
Iron Deficiency Anemia ◽  
Complete Recovery ◽  
Deficiency Anemia ◽  
Therapeutic Algorithm ◽  
The Many

Among the many reasons for the development of iron deficiency anemia, chronic blood loss from Cameron ulcers, developing with hiatal hernias, is gaining increasing clinical significance. Nevertheless, doctors of various specialties still have limited knowledge about the role of this pathology in the depletion of the iron depot, the specifics of instrumental verification of the diagnosis, and the possibilities of surgical technologies in the complex treatment of patients with this disease. The described case illustrates the long-term course of severe iron deficiency anemia in a patient with hiatal hernia (type III) due to latent blood loss from Cameron ulcers. The use of a therapeutic algorithm, which included complex conservative therapy followed by laparoscopic surgery to eliminate the hiatal hernia, led to a complete recovery of the patient.

Assessment of Immature Platelet Fraction in Patients with Ph-Negative Myeloproliferative Disorders and Thrombocytosis.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4980-4980
Author(s):  
Ondrej Zapletal ◽  
Jan Blatny ◽  
Michaela Selingerova ◽  
Jiri Jarkovsky ◽  
Miroslav Penka
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Peripheral Blood ◽  
Myeloproliferative Disorders ◽  
Optical Channel ◽  
Polycythaemia Vera ◽  
Platelet Production ◽  
Platelet Counts ◽  
Immature Platelet Fraction ◽  
Immature Platelets

Abstract Abstract 4980 Background Myeloproliferative disorders (MPD) in general result from proliferation of a clone of myeloid cells derived from a neoplastic pluripotent precursor or from connective tissue elements in bone marrow. This leads to increased numbers in one or more blood cell lines in peripheral blood. Some MPDs can be associated with thrombocytosis (MPD-T): essential thrombocythaemia (ET), polycythaemia vera (PV) and early stages of chronic idiopathic myelofibrosis (CIMF). Usually in MPD-T the thrombocytosis is caused by increased platelet production from proliferating mature megakaryocytes, especially in ET. Elevated platelet counts in these patients are often associated with both thromboembolic events and bleeding. One of the goals of MPD treatment is the control of platelet count. Immature platelets mirror the platelet production in bone marrow. In certain automated blood count analyzers it is possible to measure Immature platelet fraction (IPF) from the routine CBC samples sent to haematology lab. Reference range for IPF parameter for method used in this study is 1,1-6,1%. Objective Measurement of IPF parameter by fully automated analyzer (XE-2100, Sysmex, Kobe, Japan) in optical channel and analyzing it ( software IPF Master) in patients treated for Ph-negative MPD. We enrolled 85 pts- 67 ET (79%), 10PV(12%) and 8 CIMF (9%) patients; 57 were women and 28 men; median age 56 years ranging from 20 up to 83 years. We analyzed and evaluated IPF in whole group as well as in subgroups depending on diagnosis, gender, age, JAK-2 mutation and platelet count. Results At the time of assessment the majority of our pts were already commenced on treatment for their MPD. Platelet counts (plt) in whole cohort ranged from 164 up to 2148 ×109/l, with median 374 ×109/l. Thirty eight pts (45%) had plt < 350 ×109/l. Plt <450 ×109/l (WHO 2008 recommended cut off level for thrombocytosis) were found in 59 pts (69%). IPF median in whole cohort was 5,9% (0,7-14,4%). When comparing IPF in subgroups mentioned above statistically significant differences (p<0,05) was found only between subgroups with normal and abnormal plt counts: IPF median 7,45% (0,8-14,4%) resp. 4,6% (0,7-11,9%), (p=0,002) and between subgroups with less and more than 450×109/l plt (IPF median 6,6% (0,8-14,4%) resp. 3,85% (0,7-9,6%), (p<0,001). Fact, that patients with higher plt had lower IPF and vice versa, was confirmed also by Spearman correlation coefficient. When correlating results of plt a IPF in the whole cohort, we found the trend to indirect dependence (rs= –0,386). Conclusions MPD-T patients in our cohort did not have marked elevation of IPF parameter, neither those with high platelet counts (so far untreated pts). Thus we can speculate, that increased number of Plts in peripheral blood is caused by increased number of mature megacaryocytes in bone marrow which produce adequate numbers of platelets without increase of immature fraction rather than increased number of immature platelets released from megacaryocytes appearing in normal numbers in bone marrow Thus it seems that negative feedback of increased Plt counts on releasing of Plts from megacaryocytes is maintained also in patients with MPD-T. Further assessment is needed and should further determine, what is the clinical relevance (if any) of measurement of IPF in patients treated for MPD-T. Disclosures Blatny: Sysmex, Czech Republic: Consultancy.

Thrombocytosis and essential thrombocythaemia

2020 ◽  
pp. 5239-5247
Author(s):  
Daniel Aruch ◽  
Ronald Hoffman
Keyword(s):  
Platelet Count ◽  
Myeloproliferative Neoplasms ◽  
Receptor Gene ◽  
Jak2 V617f ◽  
Prior History ◽  
Polycythaemia Vera ◽  
Essential Thrombocythaemia ◽  
Minor Criterion ◽  
History Of ◽  
The One

The term thrombocytosis refers to a platelet count elevated above 450 × 109/litre, which can be (1) primary—including essential thrombocythaemia, chronic myeloid leukaemia, polycythaemia vera, and myelodysplastic syndromes; or (2) secondary—including iron deficiency, infection, blood loss, and malignancy. Essential thrombocythaemia: aetiology—the JAK2 V617F missense mutation typical of polycythaemia vera is found in about 50% of cases. In addition, 10% of patients have a mutation in the thrombopoietin receptor gene, MPL, and 30% have a mutation in calreticulin (CALR). Approximately 10% of patients have none of these mutations and are referred to as ‘triple negative’ essential thrombocythaemia. Diagnosis requires all of the following four major criteria: (1) platelet count greater than 450 × 109/litre; (2) bone marrow biopsy showing proliferation mainly of the megakaryocyte lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei without a significant increase or left shift in neutrophil granulopoiesis or erythropoiesis and very rarely minor (grade 1) increase in reticulin fibres; (3) failure to meet the criteria for other myeloproliferative neoplasms; and (4) presence of JAK2, CALR, or MPL mutations. Alternatively, diagnosis can be met when the first three major criteria are present and the one minor criterion, namely the presence of another clonal marker or absence of evidence for reactive thrombocytosis. Treatment requires risk stratification based on the age of the patient and any prior history of thrombosis, with treatment being reserved for those at a high risk of developing complications and not introduced simply on the basis of platelet counts alone unless there is extreme thrombocytosis (>1500 × 109/litre). Therapies include low-dose aspirin and cytoreduction.

Impaired Platelet Production and Autoantibody-Mediated Platelet Destruction Are Two Major Causes for Prolonged Thrombocytopenia after Allogeneic HSCT.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 2256-2256
Author(s):  
Rie Yamazaki ◽  
Masataka Kuwana ◽  
Yuka Okazaki ◽  
Yutaka Kawakami ◽  
Yasuo Ikeda ◽  
...  
Keyword(s):  
Stem Cell ◽  
Platelet Count ◽  
B Cells ◽  
Immune Thrombocytopenia ◽  
Therapeutic Response ◽  
Healthy Controls ◽  
Platelet Destruction ◽  
Platelet Production ◽  
Mixed Features ◽  
Pathogenic Process

Abstract Prolonged thrombocytopenia is one of late complications in patients underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT), but its pathogenic process is unclear in the majority of cases. In this study, mechanisms for thrombocytopenia in allo-HSCT recipients were examined using a series of parameters useful for discriminating immune thrombocytopenia from bone marrow failure. Forty-one patients who underwent allo-HSCT and survived for >100 days without recurrence were enrolled. Of these, 20 (49%) had prolonged thrombocytopenia (<100 x 109/L for ≥3 months). As controls, 81 with idiopathic thrombocytopenic purpura (ITP), 12 with aplastic anemia (AA), 41 healthy donors were used. Circulating B cells producing anti-GPIIb/IIIa antibodies, an index for plasma glycocalicin normalized for the individual platelet count (GCI) and plasma thrombopoietin (TPO) were measured as indices of anti-platelet autoantibody response, platelet turnover and platelet production, respectively. A period between HSCT and blood examination was shorter in allo-HSCT with thrombocytopenia than in those without (P = 0.02), but other clinical characteristics, including age, underlying disease, stem cell source, GVHD prophylaxis regimen, and the presence or absence of chronic GVHD, were not different. Anti-GPIIb/IIIa antibody-producing B cells in allo-HSCT were significantly more frequent than those in AA (P = 0.002) and healthy controls (P < 0.001), but were less frequent than those in ITP (P = 0.002). GCI in allo-HSCT was higher than that in healthy controls (P = 0.01), but was lower than GCI in ITP (P < 0.001). Allo-HSCT recipients had a higher TPO level compared with ITP (P = 0.01) and healthy controls (P = 0.02), but had a lower level compared with AA (P = 0.004). In allo-HSCT, anti-GPIIb/IIIa antibody-producing B cell frequency, TPO and GCI were greater in patients with thrombocytopenia than those without (P = 0.01, < 0.001 and < 0.001, respectively). In addition, there were a negative correlation between platelet count and GCI (r = 0.66, P < 0.001), or TPO (r = 0.52, P < 0.001). Step-wise multiple regression analysis revealed that GCI and TPO were factors independently associated with platelet count in allo-HSCT. ITP and AA patients and healthy controls were classified based on levels of GCI and TPO: 72% of 32 ITP showed GCIhigh/TPOlow; 67% of 9 AA showed GCIlow/TPOhigh; and all 17 healthy controls showed GCIlow/TPOlow. Eighteen allo-HSCT recipients with thrombocytopenia represented heterogeneous distribution: 3 with GCIhigh/TPOlow (ITP-like), 6 with GCIlow/TPOhigh (AA-like), 6 with GCIhigh/TPOhigh (mixed features) and 3 with GCIlow/TPOlow. A therapeutic response to immunosuppressive treatment was observed in 2 of 2 GCIhigh/TPOlow, one of 4 GCIlow/TPOhigh, 2 of 6 GCIhigh/TPOhigh, and none of one GCIlow/TPOlow. In an allo-HSCT recipient with GCIhigh/TPOlow, platelet count responded to high-dose prednisolone and anti-GPIIb/IIIa antibody-producing B cells and GCI were negatively correlated with platelet count during the course, compatible with immune thrombocytopenia. In summary, mechanisms for prolonged thrombocytopenia after allo-HSCT are heterogeneous, but mainly caused by impaired platelet production, autoantibody-mediated platelet destruction, or a combination of both. GCI and TPO are useful markers for evaluating a pathogenic process for thrombocytopenia and predicting a therapeutic response to immunosuppressive therapies in allo-HSCT recipients.

scholarly journals Recent Progress in Interferon Therapy for Myeloid Malignancies

2021 ◽  
Vol 11 ◽  
Author(s):  
Fiona M. Healy ◽  
Lekh N. Dahal ◽  
Jack R.E. Jones ◽  
Yngvar Floisand ◽  
John F. Woolley
Keyword(s):  
Stem Cell ◽  
Myeloid Leukaemia ◽  
Haematopoietic Stem Cell ◽  
Polycythaemia Vera ◽  
Type I ◽  
Cell Transplant ◽  
Bone Marrow Niche ◽  
Term Survival ◽  
Myeloid Malignancies ◽  
The Impact

Myeloid malignancies are a heterogeneous group of clonal haematopoietic disorders, caused by abnormalities in haematopoietic stem cells (HSCs) and myeloid progenitor cells that originate in the bone marrow niche. Each of these disorders are unique and present their own challenges with regards to treatment. Acute myeloid leukaemia (AML) is considered the most aggressive myeloid malignancy, only potentially curable with intensive cytotoxic chemotherapy with or without allogeneic haematopoietic stem cell transplantation. In comparison, patients diagnosed with chronic myeloid leukaemia (CML) and treated with tyrosine kinase inhibitors (TKIs) have a high rate of long-term survival. However, drug resistance and relapse are major issues in both these diseases. A growing body of evidence suggests that Interferons (IFNs) may be a useful therapy for myeloid malignancies, particularly in circumstances where patients are resistant to existing front-line therapies and have risk of relapse following haematopoietic stem cell transplant. IFNs are a major class of cytokines which are known to play an integral role in the non-specific immune response. IFN therapy has potential as a combination therapy in AML patients to reduce the impact of minimal residual disease on relapse. Alongside this, IFNs can potentially sensitize leukaemic cells to TKIs in resistant CML patients. There is evidence also that IFNs have a therapeutic role in myeloproliferative neoplasms (MPNs) such as polycythaemia vera (PV) and primary myelofibrosis (PMF), where they can restore polyclonality in patients. Novel formulations have improved the clinical effectiveness of IFNs. Low dose pegylated IFN formulations improve pharmacokinetics and improve patient tolerance to therapies, thereby minimizing the risk of haematological toxicities. Herein, we will discuss recent developments and the current understanding of the molecular and clinical implications of Type I IFNs for the treatment of myeloid malignancies.

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