Rare and unusual bleeding manifestations in congenital bleeding disorders: an annotated review.

Abstract

Epistaxis, superficial and deep hematomas, hemarthrosis, gastrointestinal bleeding, hematuria represent the most frequent hemorrhagic events in congenital coagulation disorders. Occasionally, bleeding manifestations occur in unusual sites or are peculiar. A clotting defect may alter the clinical aspect of skin conditions or infections (hemorrhagic scabies or varicella). Hemobilia may occur as a complication of transjugular liver biopsy in hemophilia or Bernard-Soulier syndrome. Hemarthrosis of small joints of feet and hands occur in patients with hemophilia treated with protease inhibitors. Intramedullary hematomas of long bones have been described in α2-plasmin inhibitor or fibrinogen deficiencies. Spleen fracture with consequent hemoperitoneum has been reported in patients with fibrinogen deficiency. Rectus muscle sheath hematoma may occur in patients with factor VII (FVII)or FX deficiency. Acute or subacute intestinal obstruction may be caused by intramural wall hematomas in hemophilia and von Willebrand (vW)-disease. Physicians should always keep in mind that a congenital hemorrhagic disorder may cause bleeding in any tissue of the body and therefore alter the normal clinical features of a given disease.

• Authors: Antonio Girolami, Silvia Vettore, Elisabetta Ruzzon, Giulia Berti de Marinis, Fabrizio Fabris
• Journal: Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis (Clin Appl Thromb Hemost) 2012 Mar-Apr Vol. 18 Issue 2 Pg. 121-7 ISSN: 1938-2723 [Electronic] United States
• PMID: 21873355 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Plasminogen Activator Inhibitor 1
• alpha-2-Antiplasmin

Topics

• Blood Platelet Disorders (complications, congenital)
• Bone Diseases (etiology)
• Coagulation Protein Disorders (complications, congenital)
• Epistaxis (etiology)
• Female
• Hemarthrosis (etiology)
• Hematoma (etiology)
• Hemorrhage (etiology)
• Hemorrhagic Disorders (complications)
• Humans
• Male
• Menorrhagia (etiology)
• Organ Specificity
• Plasminogen Activator Inhibitor 1 (deficiency)
• Splenic Rupture (etiology)
• Tears
• alpha-2-Antiplasmin (deficiency)