Clinical Spectrum of Primary Polycythaemia and Its Complications -Experience from a Single Center in Karnataka.

ABSTRACT

Awareness and diagnosis of Philadelphia Negative Chronic Myeloproliferative Disorders has now improved and there is a need for more epidemiological data from India. MethodsThis is a retrospective study of patients of polycythaemia conducted at clinical haematology services, BMCRI, Bengaluru from 2010 to 2017. Results88 patients of polycythaemia were retrospectively studied. 84.1% were male and 15.9% were female. Their ages ranged from 19 to 79 years. 75 (85.23%) had Polycythaemia Rubra Vera (PRV). JAK-2 (V617F) mutation was positive in 33.33%. The commonest presentation was with unexplained erythrocytosis in 50 (66.66%), thrombosis in 20 (26.66%) and with bleeding in 2 (2.66%). 22 thrombotic events occurred in 20 PRV patients. Cortical sinus thrombosis was seen in 27.3%, cerebrovascular accidents in 22.8%, portal vein thrombosis in 13.6%, pulmonary embolism in 9.1%, central retinal artery occlusion in 13.6%, myocardial infarction in 4.5% and digital infarction in 9.1% patients. 3 cases of PRV presented with diplopia. No other definitive cause for ocular palsy could be found. The JAK 2 positive group was slightly older than the negative group and had higher frequency of splenomegaly (p<0.05) and higher values for haemoglobin (p<0.001) and neutrophil counts (p<0.001) and platelet counts (p<0.05). ConclusionsPatients with thrombosis, erythrocytosis, thrombocytosis and haemorrhage should be suspected to have myeloproliferative disorders like PRV and investigated. Ophthalmoplegia is a rare presentation and should raise the suspicion for polycythaemia. There is a higher probability of splenomegaly and higher values for haemoglobin and neutrophil counts and platelet counts in JAK 2 positive group.