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.
ABSTRACT
The most frequent emergency event in haemophilia and other bleeding disorders is intracranial haemorrhage (ICH), most of which is caused by trivial trauma, affecting around 3–10%. We studied the spectrum of presentation of ICH at our institute.METHODSThis was retrospective analysis of all patients with diagnosed or suspected bleeding diathesis and ICH presenting at the Clinical Haematology Department, BMCRI. They underwent complete coagulation workup, inhibitor screening, plain CT- brain and other relevant investigations.RESULTS3.26% of patients with bleeding disorders presented with ICH of over 2 years. M:F ratio was 13:1. 78.6% had Haemophilia A, 7.1% each had Haemophilia B, Factor XIII deficiency and Hypofibrinogenemia. 13 (92.9%) had severe Haemophilia A or B and 1 (7.1%) had mild Haemophilia B. Their ages ranged from 2 - 69 years. 50% were adults with the oldest being 69 years. Out of 7 children with Intracranial bleeds, 5 (71.4%) were <5 years. 2 (18.2%) with Haemophilia A had inhibitors. None were hypertensive. There was recent history of trauma in 9 (64.3%). All (100%) had severe headache, 21.4% had vomiting and 28.6% had seizures at presentation. Subdural haemorrhage was seen in 9 (64.3%), subarachnoid haemorrhage in 1 (7.1%), epidural bleed in 1 (7.1%), and intraparenchymal bleed in 3 (21.4%). All patients received appropriate factor replacement and supportive treatment. 9 (64.3%) received factor replacement only, 5 (35.7%) with SDH required additional burr hole evacuation. 2 (14.3%) patients died and 12 (85.7%) responded to treatment. None had sequelae in this series.CONCLUSIONS3.26% of our cohort presented with intracranial bleed over a period of two years. Majority with ICH were Haemophilia A and had severe deficiency with history of trauma. Headache is the commonest presentation and astute and keen clinical suspicion is paramount for instituting early treatment. SDH was most common in our series and majority responded to conservative management with factors.