hypofibrinogenemia


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hypofibrinogenemia

[¦hī·po·fī¦brin·ə·jə′nē·mē·ə]
(medicine)
A decrease in plasma fibrogen level.
McGraw-Hill Dictionary of Scientific & Technical Terms, 6E, Copyright © 2003 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
(1,4) According to the HLH-2004 diagnostic and therapeutic guidelines for HLH, the diagnosis is made by the presence of five of the following eight criteria: fever, splenomegaly, cytopenia, hypertriglyceridemia or hypofibrinogenemia, hemophagocytosis, low natural killer cell activity, elevated ferritin, and elevated soluble interleukin-2 levels.
At that time, the cause of anemia was contributed to her increased menstruation; however, the cause of her hypofibrinogenemia was unknown.
Coagulation tests revealed a grossly elevated INR (4.66), hypofibrinogenemia (113mg/dL), and a prolonged aPPT time (63 seconds).
Type of platelet Diagnoses Protein or functional function defect deficiency Cytoskeletal assembly Wiskott-Aldrich Wiskott-Aldrich syndrome syndrome protein Granule storage or Storage pool Granule platelet release deficiencies content Platelet adhesion Von Willebrand VWF disease Bernard-Soulier GPIb syndrome Platelet aggregation Glanzmann's GPIIb/IIIa thrombasthenia Congenital Fibrinogen hypofibrinogenemia Platelet-agonist Agonist receptor ADP or thromboxane interactions deficiencies A2 Platelet coagulant- Scott syndrome Platelet membrane protein interactions phospholipid signaling ADP, adenosine diphosphate; GP, glycoprotein; VWF, Von Willebrand factor.
It could be concluded that this patient had diffuse intravascular coagulation with severe hypofibrinogenemia of very short duration, due to a hepatogenic metastasised renal cell carcinoma.
The current (2008) diagnostic criteria for HLH are a molecular diagnosis consistent with HLH (pathologic mutations of PRF1, UNC13D, or STX11) or fulfillment of five out of the eight criteria: (1) fever (>100.4[degrees]F, >38[degrees]C), (2) splenomegaly, (3) cytopenias (affecting at least two of three lineages in the peripheral blood: hemoglobin < 9g/dL, platelets < 100 x [10.sup.9] /L, or Neutrophils < 1 x [10.sup.9]/L), (4) hypertriglyceridemia and/or hypofibrinogenemia ([less than or equal to]150 mg/100 ml), (5) ferritin [greater than or equal to] 500 ng/ml, (6) hemophagocytosis in the bone marrow, spleen, or lymph nodes, (7) low or absent natural killer cell activity, and (8) soluble CD25 (soluble IL-2 receptor) >2400 U/ml (or per local reference laboratory) [5].
There are usually pancytopenia, elevation of serum liver enzymes, and abnormal coagulation profile with hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia [16].
The diagnosis may be established genetically (based on mutations of the genes loci PRF1, UNC13D, or STX11) or by fulfillment of 5 out of 8 criteria: fever; splenomegaly; cytopenia; hypertriglyceridemia; hypofibrinogenemia; hemophagocytosis in the bone marrow, spleen, or lymph nodes; low or absent natural killer cell activity; and an elevated ferritin level (>500 ng/mL).
The diagnosis of HLH was ruled out, as the patient did not manifest some of the main diagnostic criteria such as cytopenia, splenomegaly, hypertriglyceridemia, and hypofibrinogenemia.
Their ages ranged from 3 months to 10 years, male: female ratio was 2: 1, fever was present in 100% cases, splenomegaly in 100%, cytopenias in two out of three cell lines in 100%, hypertriglyceridemia in 50%, hypofibrinogenemia in 50%, elevated ferritin in 66.6%, hemophagocytosis in bone marrow in 83%, HLH was diagnosed in one patient with Chedik - Higashi syndrome, in one patient with malaria, in one patient with typhoid fever and in one case with systemic juvenile idiopathic arthritis (sJIA).
However, hypofibrinogenemia (17.14%) and hyperfibrinogenemia (37.14%) were seen only in patients with malignancies.
The main clinical findings include prolonged fever, hepatosplenomegaly, rapidly developing pancytopenia and increased transaminases, sudden decrease in erythrocyte sedimentation rate (ESR), abnormal coagulation tests, hypofibrinogenemia, hypertriglyceridemia and hyperferritinemia.