PMC8082174_01
Severe, recurring coagulopathy with hyperfibrinolysis. ISTH DIC score; fibrinogen and D-dimer levels; PR, aPTT and FXIII as well as platelet count over time; dashed lines indicate upper limit of normal (ULN) for ISTH DIC score and lower limit of normal (LLN) for fibrinogen, PR, platelet count, respectively. alloHCT, allogeneic hematopoietic cell transplantation; aPTT, activated partial thromboplastin time; DIC, disseminated intravascular coagulation; FLAMSA-RIC, sequential conditioning regimen (fludarabine, amsacrine and cytarabine followed by fludarabine, busulfan and anti-thymocyte globulin); FXIII, factor XIII activity; IT1 DA, induction chemotherapy: daunorubicin and cytarabine; ISTH, International Society on Thrombosis and Haemostasis; PR, prothrombin ratio.
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About two weeks following admission hemorrhagic diathesis began to resolve and we could deescalate the transfusion regimen ( Figure 1 ). Fibrinogen level was <0,4 g/L (normal: 2-4 g/L), D-dimer level >4 microg/mL FEU (normal: <0,5 microg/mL FEU), PR 39% (normal: 70-120%), aPTT 36 s (normal: 24-36 s), antithrombin activity 99% (normal: 80-120%), and ISTH DIC score was 8 (normal: <5), resembling the situation at initial presentation ( Figure 1 ).