Abstract
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
Severity of symptoms was assessed by a simple score of zero (none), +1 (mild),+ 2 (moderate) and +3 (severe) (table1)
Symptom Score In 90 Egyptian PV cases
| Symptom | none | mild | moderate | severe |
|---|---|---|---|---|
| Headache | 66 | 8 | 10 | 6 |
| tinnitus | 83 | 4 | 3 | 0 |
| fatigue | 76 | 7 | 7 | 0 |
| Weight loss | 85 | 3 | 2 | 0 |
| Fever and sweating | 83 | 4 | 3 | 0 |
| Bone pains | 68 | 5 | 10 | 5 |
| parasthesia | 77 | 2 | 7 | 4 |
| pruritis | 83 | 3 | 4 | 0 |
| Abdominal discomfort | 74 | 10 | 6 | 0 |
| Symptom | none | mild | moderate | severe |
|---|---|---|---|---|
| Headache | 66 | 8 | 10 | 6 |
| tinnitus | 83 | 4 | 3 | 0 |
| fatigue | 76 | 7 | 7 | 0 |
| Weight loss | 85 | 3 | 2 | 0 |
| Fever and sweating | 83 | 4 | 3 | 0 |
| Bone pains | 68 | 5 | 10 | 5 |
| parasthesia | 77 | 2 | 7 | 4 |
| pruritis | 83 | 3 | 4 | 0 |
| Abdominal discomfort | 74 | 10 | 6 | 0 |
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.
