RARE COAGULATION DISORDERS. Retrospective analyses of 156 patients in TURKEY

Abstract

OBJECTIVE: We evaluated the clinical findings, laboratory data, management, and outcome of children with rare coagulation deficiencies (RCDs) diagnosed in Turkey during the past 10 years, retrospectively. METHODS: A simple Excel file of questionnaire was sent to contact people in each center to standardize data collection by Turkish Society of Pediatric Hematology, Hemophilia-Thrombosis-Hemostasis subcommittee. RESULTS: Total of 156 patients were included in this study from 12 pediatric referral centers. Most common rare coagulation disorders in the study was as follows: FVII (n: 53, 34%), FV (n: 24, 15.4%), FX (n: 23 case, 14.7%). The most common initial findings in our patients were epistaxis, echymosis and gingival bleeding in all type of RCDs respectively. CONCLUSION: First symptoms are mucosal bleedings and FFP and tranexamic acid is still the most commonly used therapeutic options. We suggest that prophylactic treatment that we use for hemophilia patients should be considered at the very beginning among the therapeutic options for rare factor deficiencies with severe clinical course and factor deficiency that can lead to severe bleeding.

Keywords

• rare coagulation deficiencies
• clinical findings
• laboratory data