The document summarizes essential thrombocytosis, a rare chronic blood disorder characterized by overproduction of platelets. It is one of four myeloproliferative disorders. The summary describes the epidemiology, pathophysiology involving abnormal megakaryocytes and platelet function, clinical features such as bleeding, thrombosis, and splenomegaly. Diagnostic criteria include persistent thrombocytosis over 600x109/L and exclusion of other causes, with some cases associated with a JAK2 kinase mutation. Treatment aims to reduce platelet count and risk of thrombosis.
Learning Objectives:
- Introduction
- Definition
- Classification
- Acute Leukaemia
- Predisposing Factors
- Acute Myeloid Leukaemia
- FAB & WHO Classification of AML
- Pathogenesis
- Clinical Features of AML
- Lab Diagnosis of AML
- Treatment of AML
he term myeloproliferative neoplasms (MPN) describes a
group of conditions arising from marrow stem cells and characterized by clonal proliferation of one or more haemopoietic
components in the bone marrow and, in many cases, the liver
and spleen. They are often called the myeloproliferative diseases. The three major non‐leukaemic disorders included in
this classification are:
1 Polycythaemia vera (PV);
2 Essential thrombocythaemia (ET); and
3 Primary myelofibrosis.
There are several important changes in the WHO 5th edition hemato-lymphoid with a paradigm shift towards genetic diagnosis along with morphological aspects. Precursor lesions of Clonal hematopoiesis, CHIP and CCUS are formally included, Changes include those in AML, MPN, JMML is now a part of MPN, MDS-MPN, ALAL etc.
Learning Objectives:
- Introduction
- Definition
- Classification
- Acute Leukaemia
- Predisposing Factors
- Acute Myeloid Leukaemia
- FAB & WHO Classification of AML
- Pathogenesis
- Clinical Features of AML
- Lab Diagnosis of AML
- Treatment of AML
he term myeloproliferative neoplasms (MPN) describes a
group of conditions arising from marrow stem cells and characterized by clonal proliferation of one or more haemopoietic
components in the bone marrow and, in many cases, the liver
and spleen. They are often called the myeloproliferative diseases. The three major non‐leukaemic disorders included in
this classification are:
1 Polycythaemia vera (PV);
2 Essential thrombocythaemia (ET); and
3 Primary myelofibrosis.
There are several important changes in the WHO 5th edition hemato-lymphoid with a paradigm shift towards genetic diagnosis along with morphological aspects. Precursor lesions of Clonal hematopoiesis, CHIP and CCUS are formally included, Changes include those in AML, MPN, JMML is now a part of MPN, MDS-MPN, ALAL etc.
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
1. Web Εικόνες Περισσότερα MSN Hotmail Είσοδος | Ελλάδα | Προτιμήσεις
Bing myeloproliferative disorders
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ΌΛΑ ΤΑ REFERENCE » WIKIPEDIA ARTICLES
ΑΠΟΤΕΛΈΣΜΑΤΑ
Αναφορά
Myeloproliferative disease
view original wikipedia article
Myeloproliferative disease
The myeloproliferative diseases ("MPD"s)
Myeloproliferative disease
are a group of diseases of the bone
Classification and external resources
marrow in which excess cells are
ICD-10 D47.1 overview outline images locations
produced. They are related to, and may
ICD-9 205.1, 238.4, 289.89, 289.9
evolve into, myelodysplastic syndrome and Search this article
ICD-O: 9950/0-9964/3 high
acute myeloid leukemia, although the
MeSH D009196
myeloproliferative diseases on the whole
have a much better prognosis than these conditions. The concept of myeloproliferative Myeloproliferative disease
disease was first proposed in 1951 by the eminent hematologist William Dameshek.[1] Classification
In the most recent World Health Organization classification of Hematologic Causes
malignancies, this group of diseases was renamed from "myeloproliferative diseases" to Diagnosis
"myeloproliferative neoplasms". This reflects the underlying clonal genetic changes that References
are a salient feature of this group of disease. External links
Images Videos
Classification
Although not a malignant neoplasm like other cancers, MPDs are classified within the
hematological neoplasms.
There are four main myeloproliferative diseases, which can be further categorized by the
view all 24 view all 15
presence of the Philadelphia chromosome:
Philadelphia Chromosome "positive" Philadelphia Chromosome "negative"
Chronic myelogenous leukemia (CML) Polycythemia vera (PV)
Essential thrombocytosis (ET)
Myelofibrosis (MF)
In 2001, the World Health Organization classified "chronic eosinophilic leukemia /
hypereosinophilic syndrome" and chronic neutrophilic leukemia under "Chronic
myeloproliferative diseases". [2]
Causes
All MPDs arise from precursors of the "myeloid" lineage in the bone marrow. The
lymphoid lineage may produce similar diseases, the lymphoproliferative disorders (acute
lymphoblastic leukemia, lymphomas, chronic lymphocytic leukemia and multiple
myeloma).
Diagnosis
Depending on the nature of the myeloproliferative disorder, diagnostic tests may include
red cell mass determination (for polycythemia), bone marrow aspirate and trephine
biopsy, arterial oxygen saturation and carboxyhaemoglobin level, neutrophil alkaline
phosphatase level, vitamin B12 (or B12 binding capacity) and serum urate. [3]
According to the WHO Classification of Hematopoietic and Lymphoid Neoplasms 2008
myeloproliferative disorders are divided into the following by diagnostic characteristics:
1. Chronic myelogenous leukemia (CML) with defining translocation t(9;22) BCR-ABL
translocation which has three breakpoints:
a. u-BCR-ABL (p230): leads to CML with usual neutrophilia and
basophilia. b. minor-BCR-ABL (p190): leads to CML which has a tendency
to become acute lymphoblastic leukemia (ALL) usually precursor B ALL
and rarely precursor T ALL. c. major-BCR-ABL (p210): normal usual
breakpoint
2. Primary myelofibrosis associated with JAK2 mutation in up to 50% of cases and MPL
(thrombopoietin receptor) mutation in up to 5% of cases
a. Cellular phase - increased megakaryocytes which cluster, reticulin
fibrosis, later trichrome (collagenous) fibrosis, and increased myeloid
3. In English | En español
What Are Myeloproliferative Disorders?
Quick Links Myeloproliferative disorders are a group of slow-growing blood cancers, including chronic myelogenous
leukemia, in which large numbers of abnormal red blood cells, white blood cells, or platelets grow and
Director's Corner
spread in the bone marrow and the peripheral blood.
Dictionary of Cancer Terms
NCI Drug Dictionary The following PDQ treatment summaries are available:
Funding Opportunities
Childhood Acute Myeloid Leukemia/Other Myeloid Malignancies
NCI Publications
Includes childhood myelodysplastic syndromes and other myeloproliferative disorders.
Advisory Boards and Groups [ patients ] [ health professionals ]
Science Serving People
Español Acute Myeloid Leukemia
[ patients ] [ health professionals ]
Questions about cancer?
Chronic Myelogenous Leukemia
1-800-4-CANCER
[ patients ] [ health professionals ]
Chronic Myeloproliferative Disorders
[ patients ] [ health professionals ]
Subsections of this summary include:
NCI Highlights Polycythemia Vera
Office of Biorepositories and Chronic Idiopathic Myelofibrosis
Biospecimen Research Essential Thrombocythemia
The Nation's Investment in Chronic Neutrophilic Leukemia
Cancer Research FY 2010 Chronic Eosinophilic Leukemia
Report to the Nation Finds
Myelodysplastic Syndromes
Continued Declines in Cancer
Rates Includes refractory anemia, refractory anemia with excess blasts, refractory anemia with ringed
sideroblasts, refractory cytopenia with multilineage dysplasia, unclassifiable myelodysplastic syndrome,
and myelodysplastic syndrome associated with del (5q).
[ patients ] [ health professionals ]
Subsections of this summary include:
De novo Myelodysplastic Syndrome
Secondary Myelodysplastic Syndrome
Previously Treated Myelodysplastic Syndrome
Myelodysplastic/Myeloproliferative Diseases
[ patients ] [ health professionals ]
Subsections of this summary include:
Chronic Myelomonocytic Leukemia
Juvenile Myelomonocytic Leukemia
Atypical Chronic Myeloid Leukemia
Myelodysplastic/Myeloproliferative Disease, Unclassifiable
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Bing essential thrombocythemia
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ΌΛΑ ΤΑ REFERENCE » WIKIPEDIA ARTICLES
ΑΠΟΤΕΛΈΣΜΑΤΑ
Αναφορά
Essential thrombocytosis
view original wikipedia article
Essential thrombocytosis
'Essential thrombocytosis (ET, also known
ICD10 = D75.2, D47.3
as essential thrombocythemia) is a rare
Classification and external resources
chronic blood disorder characterized by
ICD-9 238.71 overview outline images locations
the overproduction of platelets by
ICD-O: M9962/3
megakaryocytes in the bone marrow in Search this article
OMIM 187950 high
the absence of an alternative cause. In
DiseasesDB 4522
some cases this disorder may be
MedlinePlus 000543
progressive, and rarely may evolve into Essential thrombocytosis
eMedicine med/2266
acute myeloid leukemia or
MeSH D013920 Epidemiology
myelofibrosis. It is one of four
Pathophysiology
myeloproliferative disorders.
Clinical features
Epidemiology Diagnostic criteria
Treatment
Essential thrombocytosis is diagnosed at a rate of about 2 to 3 per 100,000 individuals Prognosis
annually. [1][2] The disease usually affects middle aged to elderly individuals, with an Special care related to pregnancy
average age at diagnosis of 50–60 years, although it can affect children and young References
adults as well. [3] External links
Images
Pathophysiology
The pathologic basis for this disease is unknown. However, essential thrombocytosis
resembles polycythemia vera in that cells of the megakaryocytic series are more
sensitive to growth factors. Platelets derived from the abnormal megakaryocytes do not
function properly, which contributes to the clinical features of bleeding and thrombosis. view all 24
In 2005, a mutation in the JAK2 kinase (V617F) was found by multiple research groups
[4][5][6] to be associated with essential thrombocytosis in around 30% of cases. JAK2 is
a member of the Janus kinase family. This mutation may be helpful in making a
diagnosis or as a target for future therapy.
Clinical features
The major symptoms are bleeding and thrombosis. Other symptoms include epistaxis
(nosebleeds) and bleeding from gums and gastrointestinal tract. One characteristic
symptom is throbbing and burning of the hands and feet due to the occlusion of small
arterioles by platelets (erythromelalgia). An enlarged spleen (splenomegaly) may be
found on examination.
Diagnostic criteria
The diagnosis of essential thrombocytosis requires the presence of a persistent
thrombocytosis of greater than 600 x109 /L in the absence of an alternative cause.
The following revised diagnostic criteria for essential thrombocytosis were proposed in
2005 [7] . The diagnosis requires the presence of both A criteria together with B3 to B6,
or of criterion A1 together with B1 to B6.
A1. Platelet count > 600 x 10 9 /L for at least 2 months
A2. Acquired V617F JAK2 mutation present
B1. No cause for a reactive thrombocytosis
normal inflammatory indices
B2. No evidence of iron deficiency
stainable iron in the bone marrow or normal red cell mean corpuscular
volume
B3. No evidence of polycythemia vera
6. hematocrit < midpoint of normal range or normal red cell mass in presence
of normal iron stores
B4. No evidence of chronic myeloid leukemia
But the Philadelphia chromosome may be present in up to 10% of cases. Patients with
the Philadelphia chromosome have a potential for the development of acute leukemia,
especially acute lymphocytic leukemia.
B5. No evidence of myelofibrosis
no collagen fibrosis and ≤ grade 2 reticulin fibrosis (using 0–4 scale)
B6. No evidence of a myelodysplastic syndrome
no significant dysplasia
no cytogenetic abnormalities suggestive of myelodysplasia
Treatment
Not all patients will require treatment at presentation. In those who are at increased risk
of thrombosis or bleeding (older age, prior history of bleeding or thrombosis, or very
high platelet count), reduction of the platelet count to the normal range can be achieved
using hydroxyurea (also known as hydroxycarbamide), interferon-α or anagrelide. Low-
dose aspirin is widely used to reduce the risk of thrombosis, but there may be an
increased risk of bleeding if aspirin is initiated while the platelet count is very high.
The PT1 study [8] compared hydroxyurea in combination with aspirin to anagrelide in
combination with Aspirin as initial therapy for essential thrombocytosis. Hydroxyurea
was superior, with lower risk of arterial thrombosis, lower risk of severe bleeding and
lower risk of transformation to myelofibrosis (although the rate of venous thrombosis
was higher with hydroxycarbamide than with anagrelide).
In rare cases where patients have life-threatening complications, the platelet count can
be reduced rapidly using platelet apheresis (a procedure that removes platelets from the
blood directly). ...
Prognosis
Essential thrombocytosis is sometimes described as a slowly progressive disorder with
long asymptomatic periods punctuated by thrombotic or hemorrhagic events.
However, well-documented medical regimes can reduce and control the number of
platelets, which reduces the risk of these thrombotic or haemorrhagic events. The
lifespan of a well controlled ET person is well within the expected range for a person of
similar age but without ET.
Special care related to pregnancy
Hydroxyurea and anagrelide are contraindicated during pregnancy and nursing. There is
current debate as to the safety of interferon during pregnancy and nursing. Essential
thrombocytosis can be linked with increased risk of spontaeous abortion or miscarriage
in the first trimester of pregnancy. Throughout pregnancy, close monitoring of the
mother for thrombosis and placenta is recommended to ensure blood clots are caught.
Post partum, often daily injections of low dose low molecular weight heparin (e.g.
enoxaparin) are prescribed for several weeks as this is a period where the mother is at
higher risk of developing a blood clot.
References
1. ↑ Mesa R, Silverstein M, Jacobsen S, Wollan P, Tefferi A (1999). "[Expression error: Missing operand
for > Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid
metaplasia: an Olmsted County Study, 1976-1995]". Am J Hematol 61 (1): 10–5. doi:10.1002/(SICI)1096-
8652(199905)61:1<10::AID-AJH3>3.0.CO;2-I. PMID 10331505.
2. ↑ Kutti J, Ridell B (2001). "[Expression error: Missing operand for > Epidemiology of the
myeloproliferative disorders: essential thrombocythaemia, polycythaemia vera and idiopathic myelofibrosis]".
Pathol Biol (Paris) 49 (2): 164–6. PMID 11317963.
3. ↑ Hoffman: Hematology: Basic Principles and Practice, 4th ed., 2005 Churchill Livingstone, Chapter 71.
4. ↑ Kralovics R, Passamonti F, Buser AS, Teo SS, et al. (2005). "[Expression error: Missing operand for >
A gain-of-function mutation of JAK2 in myeloproliferative disorders]". N Engl J Med 352 (17): 1779–90.
doi:10.1056/NEJMoa051113. PMID 15858187.
5. ↑ Baxter EJ, Scott LM, Campbell PJ, et al. (2005). "Acquired mutation of the tyrosine kinase JAK2 in
human myeloproliferative disorders". Lancet 365 (9464): 1054–61. doi:10.1016/S0140-6736(05)71142-9.
PMID 15781101. http://linkinghub.elsevier.com/retrieve/pii/S0140-6736(05)71142-9.