Table 1 Comparison of sepsis-associated DIC and circumferential diseases
From: Sepsis-associated disseminated intravascular coagulation and its differential diagnoses
Category | Disease | Cause | Clinical features | Treatment |
|---|---|---|---|---|
DIC | Infection-induced expression of tissue factor and phosphatidylserine of the cellular membrane | Thrombotic phenotype of coagulation disorder with fibrinolysis suppression | Management of infectious focus, potentially anticoagulant therapy | |
TMA | TTP (acquired) | Autoantibody inhibition of ADAMTS13 activity | TTP pentad (thrombocytopenia, MAHA, fluctuating neurological signs, renal impairment and fever) | Plasma exchange, immunosuppressive therapy, recombinant ADAMTS13 if possible |
STEC-HUS | Shiga toxin-producing Escherichia coli | Hemorrhagic enterocolitis, fever, thrombocytopenia, MAHA, acute kidney injury | Avoiding antibiotic therapy and supportive care | |
aHUS | Uncontrolled activity of alternative complement pathway. | Thrombocytopenia, MAHA, acute kidney injury | Plasma exchange, and anti-C5 monoclonal antibody (eculizumab) | |
Secondary TMA | HELLP syndrome | Inadequate placentation secondary to maternal immune response to invading trophoblast. | Hemolysis, elevated liver enzymes, and low platelets | Timely delivery |
APS/CAPS (primary) | Antiphospholipid antibodies (β2-glycoprotein I) | Multiple venous and arterial thrombosis, repeated miscarriage, multi-organ failure (CAPS) | Anticoagulation, glucocorticoids, plasma exchange, IVIg | |
HIT | Platelet-activating antibodies to platelet factor 4 bound to heparin | 4Ts scoring system (thrombocytopenia, the timing of onset, thrombosis, and other causes of thrombocytopenia) | Discontinuation of heparin and anticoagulant therapy by argatroban | |
ITP | IgG antibodies against GP IIb/IIIa, Ia/IIa, IV, and V | Thrombocytopenia (with AIHA, kidney injury, and neurological disorder [Evans syndrome]) | Thrombopoietin-receptor agonists (eltrombopag and romiplostim), steroid, IVIg, splenectomy | |
Others | HPS (acquired) | Over-produced cytokines triggered commonly by infection | Fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis | Treatment for the underlying cause, steroid, immunosuppressive therapy |
AIPF | Bacteria or rickettsiae infection-induced protein C deficiency | Purpura, symmetrical acral necrosis, fever, hemorrhage, and shock | Treatment for the underlying infection, protein C supplementation | |
TAFRO | Human herpesvirus 8 infection-induced interleukin-6 elevation | Thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly | Steroid, anti-interleukin-6 receptor antibody (tocilizumab) | |
SFTS | Tick-borne SFTS virus infection | Fever, thrombocytopenia, leukopenia, gastrointestinal symptoms, muscular symptoms, neurological abnormalities, coagulopathy | Plasma exchange, ribavirin, IVIg, steroid | |