The US Pacific Commonwealth of the Northern Mariana Islands is home to an underserved hemophilia population. We developed a strategy in 2014 to build sustainable island-wide medical, patient and family, and community support for this rare disease. Collaboration with regional bleeding disorder leadership galvanized a weeklong conference series. More than 200 participants attended discipline-spec...

We report a case of patient with recurrent hematomas while on anticoagulation for pulmonary embolism and prolonged hospital stay due to delayed diagnosis acquired hemophilia A. Acquired A is rare autoimmune bleeding disorder autoantibodies directed against coagulation factor VIII (FVIII), leading an FVIII deficiency. isolated activated partial thromboplastin time (aPTT) in warrants workup This ...

Factor VII (FVII) deficiency is the most frequent among rare congenital bleeding disorders, accounting for one symptomatic individual per 500,000 population, apparently without any racial/ethnic predilection. FVII deficiency prevalence in the general population is probably higher because of the presence of asymptomatic and poorly symptomatic individuals. In accordance with the role of FVII as p...

Hemorrhagic cystitis is a rare complication following radiotherapy for intrapelvic cancer types, including cervical cancer, bladder cancer and prostate cancer. The severity of hemorrhagic cystitis is different in each case, although symptoms improve spontaneously in certain cases, and often significant morbidity requiring numerous interventions occurs. Since no treatment strategy exists with hi...

Factor XIII or "fibrin-stabilizing factor," is a transglutaminase circulates in the blood circulation as a hetero tetramer with two catalytic A subunits and two carrier B subunits. This important coagulation factor has a crucial role in clotting cascade and produces strong covalent bonds between soluble formed fibrin monomers during coagulation. This stable cross linked fibrin strands are resis...

Hemophilia A is a bleeding disorder caused by defective production of factor VIII. The main concern associated with the disease is bleeding, especially after trauma and surgeries. Factor VIII replacement therapy is associated with substantial decrease of bleeding events during surgery. However, there have been a number of reports of thromboemblic events in this situ-ation. The present report de...

Acquired haemophilia (AH) is a rare bleeding disorder characterized by the presence of acquired inhibitors against Factor VIII causing disruption of coagulation cascade. It has no known genetic inheritance, and diagnosis remains a challenge. The peculiar presentations are later age of onset as acute pain in weight-bearing joints and spontaneous muscle haematoma with isolated prolonged activated...

OBJECTIVE To determine the frequency and clinical features of bleeding disorders in the tribe as a result of consanguineous marriages. DESIGN Cross Sectional Study INTRODUCTION Countries in which consanguinity is a normal practice, these rare autosomal recessive disorders run in close families and tribes. Here we describe a family, living in village Ali Murad Chandio, District Badin, labele...

background congenital factor xiii (fxiii) deficiency is a rare severs autosomal recessive bleeding disorder. objectives the aim of the study was to determine the c559t > c fxiiia genotype frequency in patients with fxiii hemophilia who lived in sistan and balouchestan province in southeast of iran. patients and methods we determined the genotype of 180 patients with factor xiii hemophilia by te...

Langerhans cell histiocytosis (LCH) is a rare disorder characterised by increased production of Langerhans-type histiocytes. It more common in the pediatric age group with predilection for osseous involvement, though any organ may be involved. A 10-year male child was brought to neurosurgical clinic slow growing painful tender mass on head. Initial attempt biopsy lesion failed due excessive ble...