The Hidden Danger: When High Platelets Go Unnoticed

A simple overlooked blood test result could be silently raising your risk of blood clots.

Imagine your blood slowly thickening over years, platelets accumulating unseen until a sudden heart attack or stroke reveals the truth. For many patients with essential thrombocythemia (ET), this scenario isn't fiction—it's their reality. New research reveals that thrombocytosis (elevated platelet counts) often goes unnoticed for years before ET diagnosis, during which patients face significant risks of devastating thrombotic events1 .

0.6-2.5

People per 100,000 affected each year6

65-70

Average age of diagnosis2 6

50%

Cases with JAK2 V617F mutation6 7

Essential Thrombocythemia: More Than Just Numbers

Essential thrombocythemia is a chronic myeloproliferative neoplasm—a type of blood cancer where the bone marrow produces too many platelets5 . These excess platelets, while normal in appearance, often function abnormally, creating a paradoxical situation where patients face increased risks of both blood clots and bleeding6 .

Genetic Mutations in ET
Key Facts About ET
  • Platelet counts typically > 450,000/μL
  • Slightly higher prevalence in women
  • Increased risk of both clotting and bleeding
  • Often discovered incidentally on routine blood tests

The Overlooked Warning: Groundbreaking Research

2020 Study: Chungnam National University Hospital

A compelling 2020 study published in the Korean Journal of Hematology uncovered a troubling pattern of delayed diagnosis in ET patients1 . Researchers conducted a retrospective review of medical records from 179 ET patients treated between January 1993 and June 2019.

Methodology

Patient Identification

Identified 32 patients who had undergone complete blood count (CBC) testing before their ET diagnosis1 .

Record Review

Meticulously reviewed medical records to identify patients with previously documented thrombocytosis1 .

Analysis

Analyzed circumstances surrounding why thrombocytosis wasn't addressed and tracked changes in blood counts1 .

Event Documentation

Documented thrombotic events occurring during the delay period1 .

Startling Findings

Reasons Thrombocytosis Was Overlooked

2.3 years

Median diagnostic delay1

12.8 years

Longest diagnostic delay1

Thrombotic Events During Diagnostic Delay

Event Type Number of Patients Percentage
Cerebral Infarction 2 33.3%
Transient Ischemic Attack 2 33.3%
Myocardial Infarction 1 16.7%
Thromboangiitis Obliterans 1 16.7%
Total 6 100%

Six patients developed serious thrombotic events during the diagnostic delay period1 .

Why Does This Matter? The Thrombosis Connection

Thrombotic events represent the most significant cause of morbidity and mortality in ET patients1 3 . The relationship between untreated thrombocytosis and thrombosis is well-established in hematology.

The JAK2 V617F mutation, present in approximately half of ET patients, is particularly associated with increased thrombotic risk3 4 . One study of thrombosis patients found that 29.7% of those with thrombocytosis carried this mutation7 .

"Early detection of ET and appropriate management would markedly reduce the thrombotic risks posed by this disorder"1 .

Thrombotic Risk Categories in ET

Risk Category Criteria Thrombosis Risk
Very Low Age ≤60 years, no thrombosis history, JAK2 wild-type Lowest risk
Low Age ≤60 years, no thrombosis history, JAK2 mutation present Low risk
Intermediate Age >60 years, no thrombosis history, JAK2 wild-type Moderate risk
High History of thrombosis or age >60 years with JAK2 mutation Highest risk

Based on current risk stratification models3

Recognizing the Signs: Beyond the Numbers

While many ET patients are asymptomatic at diagnosis, various symptoms can suggest the condition8 .

Vasomotor Symptoms
Headaches (most common neurologic symptom)
Lightheadedness and dizziness
Visual disturbances and scotoma (blind spots)
Erythromelalgia (burning pain and redness in hands and feet)
Thrombotic Manifestations
Transient ischemic attacks or stroke
Chest pain or symptoms of coronary artery disease
Deep vein thrombosis and pulmonary embolism
Splanchnic vein thrombosis (veins of the liver and digestive system)
Bleeding Complications
Generally associated with extremely high platelet counts (>1 million/μL)
Gastrointestinal bleeding (most common site)
Bruising, gum bleeding, or other mucocutaneous bleeding
Important Note

Many of these symptoms are non-specific and can be associated with other conditions. Persistent or concerning symptoms should always be evaluated by a healthcare professional.

The Diagnostic Journey: Piecing Together the Puzzle

Initial Evaluation

  • Complete blood count with differential
  • Peripheral blood smear examination
  • Inflammatory markers (CRP, ESR)
  • Iron studies to exclude iron deficiency

Confirmatory Testing

  • Bone marrow biopsy and histopathology
  • Genetic mutation testing (JAK2, CALR, MPL)
  • Cytogenetic studies to exclude other myeloid neoplasms

The Scientist's Toolkit: Essential Diagnostic Tools

Tool Function Importance in ET Diagnosis
Complete Blood Count (CBC) Quantifies blood cells, including platelets Initial detection of thrombocytosis; monitoring
Peripheral Blood Smear Allows visual examination of blood cells Assess platelet morphology; rule out other disorders
JAK2 V617F Mutation Test Detects specific genetic mutation Present in ~50% of ET cases; major diagnostic criterion
CALR Mutation Test Identifies calreticulin gene mutations Detects ~30% of ET cases (JAK2-negative)
MPL Mutation Test Finds mutations in thrombopoietin receptor gene Identifies small subset of JAK2/CALR-negative cases
Bone Marrow Biopsy Examines blood cell production at source Shows megakaryocyte proliferation; rules out other disorders

A Call to Action: Improving Early Detection

Key Recommendations

The findings from the Chungnam National University study highlight several critical opportunities for improving patient outcomes1 :

Enhanced Physician Awareness

Since physicians overlooked thrombocytosis in nearly 90% of cases in the study, better education about the significance of persistent thrombocytosis is essential.

Systematic Follow-up

Implementing protocols to ensure abnormal blood count results receive appropriate follow-up could significantly reduce diagnostic delays.

Interdisciplinary Collaboration

Hematologists need to collaborate closely with cardiologists, neurologists, and other specialists who may encounter ET patients initially presenting with thrombotic events.

Patient Education

Helping patients understand the importance of abnormal test results can empower them to participate more actively in their healthcare.

Looking Ahead

While current treatments effectively manage thrombotic risk for many ET patients using aspirin and cytoreductive agents like hydroxyurea, ongoing research continues to improve our approach3 5 .

The discovery of the JAK2 mutation has opened new avenues for targeted therapies, and clinical trials continue to explore more effective treatments with fewer side effects.

References

References