Guidelines on the laboratory diagnosis of congenital bleeding disorders in Pakistan.

Congenital bleeding disorders are found in all racial groups and are present worldwide. Among all of them haemophilia A, B and Von Willebrand's disease are the commonest and they are characterized by the low blood levels of factor VIII, IX and Von Willebrand's factor respectively. Severity of bleeding is proportional to the severity of factor deficiency.

Appropriate and inappropriate use of fresh frozen plasma.

Objective: To analyze the current situation of use and misuse of fresh frozen plasma (FFP) in various clinical situations.

Methods: This was a cross sectional study done at Liaquat National Hospital (LNH) during a period of 4 months from December 2002 to March 2003. About 300 file records of those patients who received fresh frozen plasma were studied.

Rosai Dorfman disease.

We report an unusual presentation of sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai Dorfman disease. The child presented with bilateral proptosis with massive cervical lymphadenopathy. The patient had fair response to steroid therapy but relapsed during the course of the disease.