Guideline for investigation and management of adults and children presenting with a thrombocytosis

Harrison, Claire; Bareford, David; Butt, Nauman M.; Campbell, Peter J.; Conneally, Eibhlin; Drummond, Mark; Erber, Wendy N.; Everington, Tamara; Green, Anthony; Hall, Georgina; Hunt, Beverley J.; Ludlam, Christopher A.; Murrin, Richard; Nelson‐Piercy, Catherine; Radia, Deepti; Reilly, John T.; Walt, Jon van der; Wilkins, Bridget S.; McMullin, Mary Frances

doi:10.1111/j.1365-2141.2010.08122.x

Cited by 262 publications

(296 citation statements)

References 132 publications

(162 reference statements)

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“…26 Essential thrombocythemia ET is also rare in children o18 years and no sufficient data to recommend treatment are available, especially as in the current literature the WHO criteria for ET were not stringently applied. 8,27 Moreover, no evidence for a risk stratification particularly useful to guide therapeutic decisions is available and there are no indications on how to monitor cytoreductive therapy. In adults, ELN recently reported criteria for monitoring therapy.…”

Section: How To Manage Childrenmentioning

confidence: 99%

“…The long-term leukemogenicity of HU may be a special concern, although none of the pediatric patients treated with this agent have yet undergone malignant transformation. 27,30 Adverse effects of IFN-a such as flu-like syndrome, neuropsychiatric symptoms and autoimmune phenomena can be particularly dangerous for children. Anagrelide is not licensed as first-line therapy for ET in Europe and the ELN group recommends this drug as second line therapy.…”

Section: How To Manage Childrenmentioning

confidence: 99%

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How to manage children and young adults with myeloproliferative neoplasms

Barbui

2012

Leukemia

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On the basis of my personal clinical and research experience and validated by the current literature, my approach to the management of pediatric (age o18 years) and young patients (age o40 years) with classic myeloproliferative neoplasms is presented by focusing on diagnosis, patient communication, risk stratification and therapy. The WHO-2008 diagnostic criteria are recommended, even though in children suspected with essential thrombocythemia (ET), a specific set of diagnostic features may be required. Patient communication includes information on natural history, genetic abnormalities and counseling in all women of child-bearing age. The main challenge in children and young adults with ET and polycythemia vera (PV) is to avoid recurrence of major thrombosis by selecting those patients who ultimately can benefit from cytotoxic and antithrombotic therapy without increasing the incidence of drug-induced side effects. In asymptomatic low-risk patients no therapy is prescribed while in high-risk low-dose aspirin, hydroxyurea and interferon-alpha are my first line drugs. My first decision when considering treatment of a young patient with primary myelofibrosis (PMF) or post-PV or post ET-myelofibrosis, is whether he/she qualifies for bone marrow allotransplantation. In the remaining young PMF patients palliative therapy or experimental drugs are considered.Leukemia ( Keywords: myeloproliferative neoplasms; treatment; young people INTRODUCTION Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are currently classified among the bcr/abl-negative, 'classic' myeloproliferative neoplasms (MPNs) and represent a stem cell-derived clonal myeloproliferation. Hematopoietic progenitors derived from patients with MPNs are hypersensitive to the stimulation of physiological growth factors such as thrombopoietin or erythropoietin (EPO). 1 Since 2005, the pathophysiology of these diseases has advanced considerably with the discovery of an acquired mutation of JAK2 V617F in a vast majority of PV patients and in almost half of those with ET and PMF. 2 The mutation, located within the negative regulatory pseudokinase, or Janus homology 2 domain, causes cytokine-independent activation of several biochemical pathways implicated in EPO receptor signaling. Subsequently, mutations in MPL were reported in B4% of patients with ET or PMF. The significance of JAK2 and MPL mutations for the evolution of the MPNs and their relative roles in determining disease phenotype as well as progression to myelofibrosis and leukemic transformation are unclear at present.In this context, it should be underscored that most of the information in terms of diagnosis, prognosis and therapy focuses on the 'average' MPN patients of whom median age ranges between 60 and 65 years. On the other hand, less consistent data are available in the groups of MPN patients who are presenting below this median age such as pediatric and young adults (20% of MPN patients). This issue is now becoming more and more important, because with the ad...

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Section: How To Manage Childrenmentioning

confidence: 99%

Section: How To Manage Childrenmentioning

confidence: 99%

How to manage children and young adults with myeloproliferative neoplasms

Barbui

2012

Leukemia

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“…Thrombocytosis (or thrombocythemia) is the presence of high platelet counts in the blood. 23 Although often symptomless it can predispose to thrombosis in some patients. Thrombocytosis can be contrasted with thrombocytopenia, a loss of platelets in the blood.…”

Section: Discussionmentioning

confidence: 99%

Preclinical hematological profile studies of an ayurvedic medicine Siddha Makardhwaja after chronic administration to male sprague-dawley rats

Sikder

Chakraborty

Mahbub

et al. 2017

Int J Basic Clin Pharmacol

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Background: Siddha Makardhwaja (SMD) is a classical Ayurvedic formulation markedly used as a traditional medicine in the rural population for various purposes such as stimulant, tonic, and rejuvenator.Methods: The present study is conducted to evaluate the effect of conventionally prepared SMD on the hematological parameters in experimental animals, for providing scientific data base for its logical use in clinical practice. Acute toxicity tests were conducted to determine the LD50 of the drug. To find out the effect of chronic administration of SMD on hematological parameters it was administered chronically to the male Sprague-Dawley rats at a dose of 40mg/kg for 28 days.Results: In this experiment the TC, DC, various erythrocytic parameters, platelet parameters, ESR were determined. The results of the studies are given below. There is an (13.41%) increase in the number of white blood cell count of the male rat, the increase though not significant yet it was prominent (p=0.257). There is an (15.87%) increase in the absolute count of Neutrophils of the male rat, the increase though not significant yet it was prominent (p=0.371). There is an (12.29%) increase in the absolute count of Lymphocytes of the male rat, the increase though not significant yet it was prominent (p=0.388). There is a statistically significant (p=0.035) increase in the number of platelet count of the male rat (11.13% increase). There is a (2.03%) decrease in the platelet volume distribution width of the male rat, the decrease though not significant yet it was noticeable (p=0.094). There is a statistically insignificant (p=0.619) (10.0%) increase in Erythrocyte sedimentation rate in blood from the male rat.Conclusions: As SMD decreases and increases abnormally on the hematological parameters in body of treated rats, so it should not be administered chronically at a higher dose. Further studies should be done by reducing the administered dose.

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“…Anecdotal use of Peg-IFNa in pregnant patients with hepatitis or myeloproliferative disorders (including ET) has been reported with encouraging results concerning safety and disease remission [53][54][55][56][57].…”

Section: Pegylated Interferon-amentioning

confidence: 99%

Patients with essential thrombocytosis during pregnancy: Challenges and therapeutic dilemmas

Kanellopoulou¹

2017

Obstet Gynecol Rep

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Treatment of patients with essential thrombocytosis during pregnancy is challenging and young women are reported to be at increased risk of first trimester abortions and other pregnancy complications. In literature, there are case reports and small case series where the treatment approach differs and the results are conflicting. The addition of low dose aspirin seems to be beneficial in most cases improving pregnancy outcome whereas interferon-a is the cytoreductive drug of choice when platelet control is needed during pregnancy. Anticoagulation with low-molecular weight heparin is not generally recommended and should be considered during the last few weeks of pregnancy and postpartum.

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Guideline for investigation and management of adults and children presenting with a thrombocytosis

Cited by 262 publications

References 132 publications

How to manage children and young adults with myeloproliferative neoplasms

How to manage children and young adults with myeloproliferative neoplasms

Preclinical hematological profile studies of an ayurvedic medicine Siddha Makardhwaja after chronic administration to male sprague-dawley rats

Patients with essential thrombocytosis during pregnancy: Challenges and therapeutic dilemmas

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