Essential thrombocythemia

Essential thrombocythemia
ICD-O-3 Morphology
9962/3: Essential thrombocythemia
Effective 2001 and later
for cases diagnosed 2001 and later
Primary Site(s)
Primary site must be bone marrow (C421)

9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
Alternate Names
Early essential thrombocythemia
Essential hemorrhagic thrombocythemia
Essential thrombocytosis
Hemorrhagic thrombocythemia
Idiopathic hemorrhagic thrombocythemia
Idiopathic thrombocythemia
Idiopathic thrombocytosis
Primary thrombocythemia
Primary thrombocytosis
Thrombocythemia vera
Essential thrombocythemia is a clonal myeloproliferative neoplasm. Rare chronic blood disorder characterized by elevated platelet count. Sustained thrombocytosis in blood and increased numbers of large, mature megakaryocytes in bone marrow; clinically episodes of thrombosis and/or hemorrhage.

A diagnosis of "post essential thrombocythemia myelofibrosis" is a progression of essential thrombocythemia and would be the same primary.
Abstractor Notes
Disease is usually suspected when the patient has a CBC or peripheral blood smear that shows an overproduction of platelets.

The diagnosis is clinical (diagnosis of exclusion); the physician diagnoses this disease by excluding other myeloproliferative neoplasms and correlating those equivocal results with the patient's symptoms. Bone marrow and blood are the principle sites of involvement. The spleen does not show significant extramedullary hematopoiesis but is a sequestration site for platelets. In some cases this disorder may be progressive, and only rarely evolves into acute myeloid leukemia or myelofibrosis.

50-60% of patients will have a positive JAK2.

Aspirin, in low dose only (< 100 mg/day) is used as treatment for this disease.
Definitive Diagnostic Methods
Bone marrow biopsy
Clinical diagnosis
Genetic testing
Genetics Data
Janus kinase-2, JAK2
Blood thinners, anti-clotting medications, aspirin
Other therapy (Anagrelide) (for essential thrombocythemia only)
Watchful waiting (for asymptomatic patients)
Transformations from
Corresponding ICD-9 Codes
238.71 Essential thrombocythemia
Corresponding ICD-10 Codes
D47.3 Essential (hemorrhagic) thrombocythemia
Corresponding ICD-10-CM Codes (U.S. only)
D47.3 Essential (hemorrhagic) thrombocythemia (effective October 01, 2015)
Signs and Symptoms
Bleeding from mucosal surfaces
Burning or tingling in the hands or feet
GI bleeding
Redness and warmth of the hands or feet
Vision or hearing problems
Progression and Transformation
Epidemiology and Mortality
Age: 50-60 years median age
Incidence: 0.6-2.5 per 100, 000 cases per year
Sex: no male or female predominance
Survival: 10-15 years median survival