Transfusion related acute Lung injury (TRALI) though a serious blood transfusion reaction with a fatality rate of 5-25 % presents with acute respiratory distress with hypoxaemia and noncardiac pulmonary oedema

This presentation is a clinical narrative and long term follow up (6-16 years) of 21 prospectively studied patients with essential thrombocythaemia (ET) in Kuwait. The median age (55.9 years) is younger than reported by others. Two patients were below the age of 40 years with one of them presenting as post-polycytheamia ET at 16 years of age. Twelve patients (57.1 %) remained asymptomatic throughout the period of follow up. Four patients complained of erythromelalgia, three (19 %) suffered from thrombotic episodes and only one (4.3 %) had excessive bleeding. Four patients presented with splenomegaly. Intensity of thrombocytosis or duration of very high platelet count had no relationship with these complications. Two patients transformed to post-ET myelofibrosis and one patient developed chronic myeloid leukaemia (CML). None transitioned to acute leukaemia. All patients are still alive after follow up for 6-16 years. Janus kinase 2 mutation was positive in eight (38 %) patients. It had no bearing on transition of our ET patients to post-ET myelofibrosis or CML. Platelet aggregation tests were performed in 14 patients. Six (42.9 %) showed defective response to ADP. Only one of these patients suffered from bleeding. All patients were given aspirin (81 mg/day). Cyto-reductive therapy with hydroxyurea was taken by six (42.9 %) subjects. Two patients who were treated with anagrelide and one with alpha-interferon did not continue treatment for long.

Warfarin (Coumadin) continues to remain the mainstay of oral anticoagulant therapy (OACT) for thromboprophylaxis for both venous thromboembolic disease (VTD) and cardiac indications. However it needs careful monitoring because of its narrow window of target activity level, interaction with numerous medications and food items, caution for use in patients with co-morbidities like hepatic and renal impairment and bleeding lesions and the risk of major hemorrhage. A large part of its success and safety requires the patients own understanding and participation in its control. In a retrospective study on 153 patients on long term OACT with warfarin, we have analyzed the influence of various personal characteristics of the local patient population like age, gender, nationality, education and financial status, family size, family style, manner of drug administration and number of other medications prescribed for co-morbidities. Ability to achieve consistently efficacious target level of anti coagulant activity is adversely affected by older age, female gender, lower education status, larger family size, joint family setting, dependence on domestic servants to administer warfarin and larger number of other medications taken for co-morbidities. Thirty-seven patients were identified from such vulnerable personal characteristics and assigned to a separate anticoagulant therapy control clinic with specific arrangements for stricter control. This group of patients was studied prospectively for 18 months. Significant improvement was apparent on comparison of their performance before and after assignment to the separate clinic.

Iron deficiency anemia (IDA) is the commonest type of anemia all over the world. It is easily treatable with oral iron therapy, but a minority of patients does remain unresponsive. This may be related to inappropriate compliance, manifest or occult blood loss, worm infestations, helicobacter infection, known malabsorption syndromes or paroxysmal nocturnal hemoglobinuria (PNH). We observed six such patients with non-responsive recurrent classical IDA who did not show any of the above conditions on specific investigations.Iron tolerance test (as an index of oral iron absorption) was performed on these patients. All of them showed negligible or no iron absorption, suggesting a defect in intestinal oral iron absorption as the cause of non-responsive IDA. This contention was supported by a dramatic response to parenteral iron therapy in all of them. Since the tests for known malabsorption syndromes were negative, it would be assumed that these subjects had an isolated defect in oral iron absorption that has been only scantily reported in the literature. Five of the six patients were siblings of two families. This raises the possibility of the defect in iron absorption to have a genetic connotation. Pending the complete work up for genetic studies, including the gene mutation for membrane bound serum protease type 6 (TMPRSS6) in these subjects and their families, this presentation is a short communication to expose and share our small experience of what looks to be an isolated defect in intestinal iron absorption.

No abstract