Demographic Analysis Of 90 Polycythemia Vera Egyptian Cases :  An Egyptian Reference Center Experience

We report 90 cases of Polycythemia Vera ( PV )during the period between 2003 and 2013 followed up in an adult Cairo  hematology reference center

Among 90 PV cases registered between 2003 and 2013, sixty eight cases (75.6%) of  whompresented in the last three years alone.  The age range was between 19 and 72 years with a mean of 53.5 years and 25 cases ( 27.78%) presented in the age group between 50-55 years.   Male : female ratio was 1.3:1.Fifty per cent of patients were urban while 50% lived in the country.

Symptoms included headache (24 cases) (26.67%), tinnitus (7 cases) (7.78%), fatigue (14 cases) (15.56%), weight loss (5 cases)(5.56%), fever and sweating( 7 cases)( 7.78%) , bone or joint pains (22 cases)(24.44%), parasthesias (13 cases)(14.44%), pruritis(7 cases) (7.78%)  or abdominal discomfort (16 cases)(17.78% (Fig 1)

Figure (1) : Incidence Of Symptoms Among 90 Egyptian PV Cases

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Severity of symptoms was assessed by a simple score of zero (none), +1 (mild),+ 2 (moderate) and +3 (severe) (table1)

Nine cases (10%) presented by venous thrombosis , 4 of whom had portal vein thrombosis (4.44%) Cerebro-vascular strokes were noted in 5 cases (5.56%) and acute myocardial infarction in 2 cases (2.22%).

Splenomegaly was noted in 29 cases (32.22%) of cases and hepatomegaly in 13 cases (14.44%).

Associated conditions included hypertension in 24 cases (26.67%), gastrointestinal symptoms in 16 cases(17.78%) Diabetes mellitus in 15 cases (16.67%),Hepatitis C virus infection in 6 cases ( 6.67%), Hepatitis B in 2 cases ( 2.26%), coronory heart disease in 5 cases (5 .56%) and renal problems in 3 cases ( 3.33%). Bleeding episodes included 6 (6.67%) GI and 3(3.33%) gum bleeds.

Familial MPN, Cancer prostate, intestinal Schistosomiasis   and post-liver transplant done post- hepatitis C virus cirrhosis were reported in one case each.

Twenty two cases(24.44%) were controlled on Hydroxyuria only,16 cases 17.78%) on venesection plus hydroxyuria,16 cases(17.78%) on venesection only. Progression  was noted in 13 cases (14.44%) with marrow fibrosis in five cases (5.56%) and 14 cases (15.56%) were lost to follow-up. Mortality was seen in 10% (nine cases).

PV among Egyptians may have distinct presentations regarding incidence, age and  presentation.Simpler methods of symptom severity assessments may be more suitable for Egyptian patients.

Mattar: Novartis, BMS, Amgen: Honoraria.