RESUMEN
To address the scarcity of real world data on adult prophylaxis from developing world, a short term intermediate dose prophylaxis in adult haemophilia A patients was initiated. A total of eight patients aged > 18 years with moderate/severe haemophilia A were given an average dose of 23 IU/kg recombinant factor VIII (rFVIII) concentrate twice weekly for 2 months. A clinical audit was done on completion of four months. The mean age of the participants was 31.63 (± 6.98) years. The mean bleed rate during two months of episodic versus prophylactic regimen was 5.13 versus 0.63 (p = 0.01) and that of work days lost, hospital visits for hemophilia care were 30.63 (± 24.69) versus zero days, 20.63 (± 16.19) versus zero days respectively. The mean of factor VIII consumed during prophylaxis was 13,500 IU/month (i.e., 23 IU/kg/dose).The median time gap between prophylactic infusion to trough level was 67.50 h (60-74 h) and the median trough level observed was 2.50% (range 1-5%). The results of our clinical audit show that Intermediate dose prophylaxis with rFVIII concentrates in young adult patients with moderate/severe haemophilia A appears to be effective in reducing the frequency of bleeds.
RESUMEN
Cytophagic histiocytic panniculitis (CHP) was first described in 1980 by Winkelmann as a chronic histiocytic disease of the subcutaneous adipose tissue, which is characterized clinically by tender erythematous nodules, recurrent high fever, malaise, jaundice, organomegaly, serosal effusions, pancytopenia, hepatic dysfunction, and coagulatory abnormalities. CHP may occur either isolated or as part of cutaneous manifestations of hemophagocytic syndrome. Here, we report two different presentations of CHP.