To investigate the cause of her
hypofibrinogenemia further, we tested the patient's fibrinogen antigen level, which was near normal (1.89 g/L).
The lab abnormalities that had the best sensitivity and specificity were hyperferritinemia, increased lactate dehydrogenase level, hypertriglyceridemia, and
hypofibrinogenemia. Accordingly, the researchers suggested criteria for diagnosing MAS in juvenile SLE patients, and bone marrow aspiration was not considered necessary except in doubtful cases (Table 2).
Hypertriglyceridemia in 53% and
hypofibrinogenemia in 46%, elevated ferritin in 10 out of 13 cases (76%), Hemophagocytosis in bone marrow was found in 11 out of 13 (84%).
Rapid and correct prediction of thrombocytopenia and
hypofibrinogenemia with rotational thromboelastometry in cardiac surgery.
ROTEM[R] is an appropriate method for estimation of PLT count and FBG level based on the MCF, particularly useful in afibrinogenemia,
hypofibrinogenemia and dysfibrinogenemia [25].
It is characterized by persistent fever, pancytopenia, liver dysfunction, hepatosplenomegaly, hyperferritinemia,
hypofibrinogenemia, elevated serum lactate dehydrogenase, and hypertriglyceridemia.
Platelets and cryoprecipitates must be transfused to manage thrombocytopenia and
hypofibrinogenemia respectively.
Lyophilized fibrinogen concentrate (RiaSTAP) is approved by the US Food and Drug Administration for the treatment of congenital
hypofibrinogenemia and also may be useful to replace fibrinogen in cases of AFE.
Hypertriglyceridaemia and /or
hypofibrinogenemia: i.
If FFP fails to adequately correct TEG parameters,
hypofibrinogenemia exists, and surgical bleeding continues, cryoprecipitate is administered.
It is characterized by fever, splenomegaly, cytopenias affecting at least 2 of 3 lineages in the peripheral blood, hyperferritinemia, hypertriglyceridemia and/or
hypofibrinogenemia, hemophagocytosis in the bone marrow, spleen, or lymphnodes, and so forth 1].
(1) Complete diagnosis is made by identification of the abnormal genetic mutations or when five of the following eight diagnostic criteria are identified: fever, splenomegaly, cytopenias, hypertriglyceridemia and/or
hypofibrinogenemia, hemphagocytosis, low or absent NK-cell activity, elevated ferritin, and elevated sCD25.
