Essential Thrombocytosis ET

Definition

• t in platelets ( 600.000 nl) • * RBC and granulocytes Etiologies of thrombocytosis

• 1° ET or other MPD: myelodysplastic syndromes (5q-syndrome)

• 2° reactive thrombocytosis: inflammation (RA. IBD, vasculitis), infection, acute bleeding, iron deficiency, postsplenectomy. neoplasms (particularly Hodgkin's disease)

• Of Pts w pit >10* |il. <1 in 6 will have ET

Clinical manifestations (see "Polycythemia Vera")

• Thrombosis with erythromelalgia (risk of thrombosis highest in Pts with WBC -8700).

bleeding, pruritus; mild splenomegaly

Diagnostic evaluation

• Peripheral smear: large hypogranular platelets

• BM bx: megakaryocyte hyperplasia: absence of Philadelphia chromosome and lack of collagen fibrosis; normal iron stores

Treatment of ET

Risk

Features

ASA 81 mg qd

Cytoreduction

Low

Age ■ 60 and no h o thrombosis and pit <1.5 x 10' p.l and no CV risk factors

Consider for vasomotor symptoms

No

Int.

Neither low nor high risk

t

Consider if pit >1.5 x 04 p.l

High

Age -60 or h o thrombosis or pit >1.5 x 10* nl

®

Hydroxyurea superior to anagrelide (NEjm 2005.353 33)

Goal pit <400.000 jil

Prognosis

• Overall survival similar to control population with low rate of transformation into PV. AMM MF or acute leukemia: low-risk Pts (see below) do not need treatment

Was this article helpful?

0 0

Post a comment