Least chances of splenomegaly occur with (AIIMS Nov 2010)
|C||Polycythemia Rubra Vera|
Extra Edge: Other causes of Massive splenomegaly are:
1. Chronic malaria
2. Chronic kala azar
3. Portal hypertension
4. Hairy Cell Leukemia
5. Glycogen storage disease
Essential Thrombocythemia (ET) (Primary Thrombocytosis) (Ref. Hari- 18th ed., Pg 902)
This is characterized by a very high platelet count of more than 6 Lacs/ml
Usually patients are asymptomatic; others present with GI bleed, arterial clotting, paresthesias, erythromelalgia (burning feet), vascular headaches. Splenomegaly and hepatomegaly are there.
Note: In ET splenomegaly is usually mild i.e. it is never massive splenomegaly which is seen in CML PCV & myelofibrosis (AIIMS Nov 10)
Treatment depends on urgency
1. In an asymptomatic young patient < 40years, consider aspirin 60 mg/day and observe.
2. Busulphan or hydroxyurea.
3. Interferon alpha is given for extreme thrombocytosis.
4. Radio active phosphorus is used in treatment
5. Anagrelide is used to prevent thrombosis.
Thrombocytosis is almost always due either to (Ref. Hari- 18th ed., Pg. 970)
1. Iron deficiency;
2. Inflammation, cancer, or infection (reactive thrombocytosis);
3. Underlying myeloproliferative process.
Causes of Thrombocytosis (Ref. Hari-18th ed., Pg. 903, table 108.5)
3. Myeloproliferative disorders: polycythemia vera, idiopathic myelofibrosis, essential thrombocytosis, chronic myelogenous leukemia
4. Myelodysplastic disorders: 5q-syndrome, idiopathic refractory sideroblastic anemia
5. Postsplenectomy or hyposplenism
7. Iron deficiency anemia
9. Rebound: Correction of vitamin B12 or folate deficiency, post-ethanol abuse