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. The diagnosis of bleeding disorders can be complex, and no single diagnostic tests are suitable for all patients. The guideline was developed after reviewing relevant publications, summarizing current understanding of bleeding disorders and classification, and present a consensus diagnostic recommendation based on analysis of the literature and expert opinion. They also suggest an approach for clinical and laboratory evaluation of individuals with bleeding symptoms, history of bleeding or conditions associated with increased bleeding risk. The document summarizes needs for improvement in laboratory testing and quality which is very much needed in Pakistan to make a correct diagnosis, train master trainers, identify complications of bleeding disorders in local population, increase awareness among masses, involve government in haemophilia care, education of patients and their families and health care community. It further enhances the need for research in bleeding disorders, including clinical research to obtain more objective information about bleeding symptoms, advancements in diagnostic and therapeutic tools.

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. Each file record was checked for the diagnosis of the patient, coagulation profile and doctor's trigger for blood transfusion. The indications of FFP were checked according to guidelines set by British Committee of Standards and Haematology. RESULTS: Of 1486 units of FFP that were transfused to 300 patients, it was observed that 78.6% (1169 / 1486) of FFP was appropriately transfused while the remaining 21.3% (317/1486 units) was used without any supportive evidence. CONCLUSION: Although majority of the plasma was used appropriately, a considerable volume of plasma was also wasted.

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.