Essential Thrombocythemia and Secondary Thrombocytosis Flashcards
What is thrombocytosis?
Platelet count above normal range
Thrombocytosis can be classified into essential thrombocythemia and secondary thrombocytosis.
What characterizes essential thrombocythemia?
Rare chronic myeloproliferative cancer characterized by the overproduction of platelets by megakaryocytes in the bone marrow
Essential thrombocythemia is more common in women and typically affects individuals around 60 years old.
What genetic mutations are associated with essential thrombocythemia?
Mutations of the JAK2, CALR, or MPL genes
These mutations result in the overproduction of hematopoietic progenitor cells.
What is secondary/reactive thrombocytosis?
Abnormally high platelet count in the absence of chronic myeloproliferative disease, caused by underlying conditions
This condition can occur in anyone and at any age.
What are some causes of secondary thrombocytosis?
Elevated levels due to infectious, inflammatory, malignant, and traumatic processes
These factors lead to overproduction of thrombopoietic factors that act on megakaryocytes or their precursors.
What are common symptoms associated with essential thrombocythemia?
- Headaches
- Dizziness and tinnitus
- Visual disturbances (e.g., blurred or double vision)
- Difficulty concentrating
- Splenomegaly causing abdominal pain
- Bleeding and bruising (e.g., epistaxis, bleeding gums)
- Burning and tingling in hands and feet
- Livedo reticularis
Symptoms and complications are generally more severe in essential thrombocythemia compared to secondary thrombocytosis.
What findings on a full blood count (FBC) indicate thrombocytosis?
Thrombocytosis, normal erythrocyte count and haematocrit, sometimes leukocytosis
These findings help differentiate between essential and secondary thrombocytosis.
What does a bone marrow biopsy show in essential thrombocythemia?
Marked proliferation (hypercellularity) and hyperplasia of large megakaryocytes
This finding is critical for confirming the diagnosis.
What laboratory tests can indicate an autoimmune condition associated with thrombocytosis?
Elevated ESR and CRP, other inflammatory markers such as RF or ANA
These tests can help identify underlying conditions contributing to secondary thrombocytosis.
True or False: Essential thrombocythemia primarily affects men.
False
It is more common in women.
Fill in the blank: Essential thrombocythemia is most common in individuals around _______ years old.
60
This demographic detail is important for understanding the epidemiology of the disease.
What is the management approach for essential thrombocythemia?
Treated with cytoreductive therapy monitored with regular FBCs.
What is the first-line treatment for essential thrombocythemia?
Hydroxycarbamide/hydroxyurea or interferons (chemotherapy) and aspirin.
What is the second-line treatment for essential thrombocythemia?
The alternate first-line drug and aspirin (e.g., interferon is better for younger patients and pregnant women).
What is the third-line treatment for essential thrombocythemia?
Anagrelide (inhibits megakaryocyte maturation but increases risk of thrombotic and bleeding events) or ruxolitinib (JAK2 inhibitor).
