Factor XIII deficiency

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Factor XIII deficiency Classification and external resources
ICD-10	D68.2
ICD-9	286.3
OMIM	134570 134580
DiseasesDB	31412
eMedicine	ped/3040
MeSH	D005177

Factor XIII deficiency may occur very rarely, and can cause a severe bleeding tendency. Incidence is 1 in a million to 1 in 5 million people. Most are due to mutations in the A subunit. Administration of recombinant A subunit improves clot stability and may become a therapeutic option for patients with this condition^[1].

[edit] See also

• Factor XIII

[edit] References

1. ^ Lovejoy A, Reynolds T, Visich J, Butine M, Young G, Belvedere M, Blain R, Pederson S, Ishak L, Nugent D (2006). "Safety and pharmacokinetics of recombinant factor XIII-A2 administration in patients with congenital factor XIII deficiency.". Blood 108 (1): 57–62. doi:10.1182/blood-2005-02-0788. PMID 16556896. 

v • d • e Pathology: hematology, hematologic disease (primarily D50-D77, 280-289) RBCs/anemia/ hemoglobinopathy (Myeloid) nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia) hereditary hemolytic anemia: enzyme disorders (G6PD Deficiency, Pyruvate kinase deficiency,Triosephosphate isomerase deficiency), Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia Polycythemia - Methemoglobinemia Coagulation/platelets (Myeloid) coagulopathy: DIC (Congenital afibrinogenemia, Purpura fulminans) • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease • Hypoprothrombinemia Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Factor V Leiden - Activated protein C resistance - Antiphospholipid syndrome other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly Essential thrombocytosis - Thrombocytopenia Monocytes/Macrophages (Myeloid) WHO-I histiocytosis (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell histiocytosis (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant histiocytic disorders (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) -cytosis: Monocytosis -penia: Monocytopenia Granulocytes (Myeloid) -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) -penia: Granulocytopenia/agranulocytosis (Neutropenia, Kostmann syndrome) Other/general myeloid Chronic granulomatous disease Lymphoid -cytosis: Lymphocytosis -penia: Lymphopenia Other Splenic disease (Asplenia/hyposplenism, Wandering spleen) - Pancytopenia See also hematological malignancy and immune disorders