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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
  1. DIC disseminated intravascular coagulation, TMA thrombotic microangiopathy, TTP thrombotic thrombocytopenic purpura, ADAMTS13 a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13, MAHA microangiopathic hemolytic anemia, STEC Shiga toxin-producing Escherichia coli, HUS hemolytic uremic syndrome, aHUS atypical HUS, HELLP hemolysis, elevated liver enzymes low platelets, APS antiphospholipid syndrome, CAPS catastrophic antiphospholipid syndrome, IVIg intravenous immunoglobulin, HIT heparin-induced thrombocytopenia, ITP immune thrombocytopenia purpura, HPS hemophagocytic syndromes, AIPF acute infectious purpura fulminans, SFTS fever and thrombocytopenia syndrome