THROMBOTIC COMPLICATIONS IN PATIENTS WITH POLYCYTHEMIA VERA

Coordinator:

Asist. Univ. Dr. Marcela Candea

Authors:

Ioana-Violeta Oltean

Ioana Barsan

Madalina Crisan

Introduction

Polycythemia Vera (PV) is a myeloproliferative disorder with predominantly erythroid hyperplasia, but also leukocytosis and thrombocytosis.

Nutrimedical.com

Introduction

A mutation or change, in the body’s JAK2 (janus-associated kinase 2) is the main cause of Polycythemia Vera.

Rachel Eastwood

Introduction

Objective

To analyse the:• clinical characteristics• laboratory data• association of thrombotic complications of Polycythemia Vera with various patient characteristics.

Material and Method

retrospective study 60 patients Hematology Clinic in Tg-

Mures 2000-2013 Polycythemia Vera

WHO criteria:- Major criteria: - Hb >18,5 g/dl in men,

16,5 g/dl in women or elevated RCM > 25% above mean normal predicted value

- presence of JAK2 V617F mutation or other similar mutation

- Minor criteria: - bone marrow biopsy (hypercellularity with prominent erythroid, granulocytic and megakaryocitic proliferation)

- serum erythropoietin level below range

- endogenous erythroid colony formation in vitroWHO = World Health Organization

Hb = HemoglobinRCM = red cell mass

Material and Method

Parameters:• sex• age• laboratory data: - hemoglobin

- hematocrit

- white blood cell count

- platelets count • vascular risk factors: - hypertension

- diabetes

- dyslipidemia

- smoking

Statistical analysis: - Microsoft Excel

- GraphPad p<0,05

thrombotic complications:

- acute coronary syndrome ( acute myocardial infarction, unstable angina pectoris)

- cerebrovascular disease (ischemic stroke, transient ischemic attacks) - venous thromboembolism (deep venous thrombosis, pulmonary embolism) - erythromelalgia - superficial thrombophlebitis

Results

Average age: 57 years

Baseline characteristics:

Hemoglobin(g/dl, mean±SD)

18,2±4,1

Hematocrit(%, mean±SD)

55,7±9,3

White cells (x109/l, mean±SD)

13,7±7,8

Platelets (x109/l, mean±SD)

468±255

Splenomegaly n (%)

40 (66,6%)

JAK2 mutation, n 13

With thrombosisWithout thrombosis

p value

Median hemoglobin, g/dl

16,85 17,02 0,1759

Median hematocrit, %

51,11 52,96 0,3524

Median white blood cell count, x109/l

16,68 15,36 0,5515

Median platelet count, x109/l

493,12 468,72 0,626

OR95% CI: lower limit

95% CI upper limit

p value

Diabetes mellitus

0,492 0,0904 2,677 0,698

Smoking 0,296 0,057 1,518 0,177

Hypertension 1,372 0,481 3,909 0,743

Dyslipidemia 4,655 1,483 14,61 0,0142

- A De Stefano V. study showed that from 235 PV patients, 21,4% had an acute coronary syndrome and 38,6% a cerebrovascular disease.

- Stein BL et al. discovered that dyslipidemia was marginally independent associated with a history of vascular complication in PV patients (p=0,045).

- A report by Varma et al. showed that was not statistically difference in the mean hemoglobin, white cell count and platelet count in patients with thrombosis as compared to patients without thrombosis.

1. PV is more frequent on male patients over the age of 50 years.

2. More than half of patients develop arterial or venous thrombosis.

3. Thrombotic complications in PV are more frequent at patients with raised platelets count (>450x109/l), raised white blood cell count (>15x109/l) and dyslipidemia.

1. Tefferi A., Thiele J., Orazi A. et al. – Proposals and rationale for revision of the WHO diagnostic criteria for Polycythemia Vera: recommendations from an ad hoc international expert panel. Blood 2007 Aug. 15; 110(4): 1092-7.

2. De Stefano V., Za T., Rossi E., Elli E. et al. – Recurrent thrombosis in patients with Polycythemia Vera and Essential Thrombocythemia. Haematol. March 1, 2008 vol.93 no.3 372-380.

3. Varma S., Sharma A., Malhotra P., Varma N. – Thrombotic complications of Polycythemia Vera. Hematology 2008 Dec; 13 (6): 319-23.

4. Landolfi R., Di Gennaro L., Barbui T., Marchioli R. Et al. – Leukocytosis as a major thrombotic risk factor in patients with Polycythemia Vera. Blood 2007; 109: 2446-2452.

5. Stein B. L., Rademarker A., Spivak J. L. and Moliterno A. R. – Gender and vascular complications in the JAK2V617F positive myeloproliferative neoplasms. Thrombosis 2011 1-8.

6. Landolfi R., Marchioli R., Patrono C. Et al. – Mechanisms of bleeding and thrombosis in myeloproliferative disorders. Thromb. Haemost. 1997; 78: 617-621.