case_images: PMC10800925_01
case_images: Panel (A) multiple electrode aggregometry profiles. ASPI-test, arachidonic acid induced aggregation; ADP-test, adenosine-diphosphate induced aggregation; TRAP-test, thrombin receptor activating peptide induced aggregation. Panel (B) Thromboelastometry profiles. EXTEM, evaluation of the extrinsic coagulation pathway; INTEM, evaluation of the intrinsic coagulation pathway; FIBTEM, evaluation of the contribution of fibrinogen to blood clotting. CT, clotting time; CFT, clot formation time; alpha, alpha-angle; MCF, maximum clot firmness; ML, maximum lysis.
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case_images: PMC10800925_01_fcvm-10-1335296-g001.jpg
case_images: F1
case_images: In our patient, platelet function was within the normal range (Figure 1, Panel A) and the thromboelastometry profile was compatible with severe hypofibrinogenemia without hyperfibrinolysis (Figure 1, Panel B).
case_images: The levels of fibrinogen during hospitalization.
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case_images: F2
case_images: After administering RiaSTAP , we observed a rapid increase in plasma fibrinogen levels, albeit followed immediately by a rapid decrease (Figure 2). The coagulopathy subsided subsequently with normal fibrinogen activity restored a few days after the initiation of induction chemotherapy (Figure 2).
case_images: PET/MRI scan.
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case_images: PMC10800925_01_fcvm-10-1335296-g003.jpg
case_images: F3
case_images: Given that the chest x-rays findings at the time of admission showed a widening of the mediastinum, especially towards the left with blurring and poor recognition of the aortic profile, the patient underwent a positron emission tomography-magnetic resonance imaging (PET-MRI) scan that showed pathological F-fluorodeoxyglucose (FDG) uptake (maximum standardised uptake value, SUV Max 19) in adrenal glands (left 74 x 90 mm, right 65 x 35 mm), a bulky lesion in the superior-anterior mediastinum (10 cm x 5 cm with a SUV Max 19) and in one right inguinal lymph node (SUV Max 5.2) (Figure 3).
case_images: Histological and immunohistochemical findings in the biopsy of the bulky lesion in the superior-anterior mediastinum.
case_images: fcvm-10-1335296-g004.jpg
case_images: PMC10800925_01_fcvm-10-1335296-g004.jpg
case_images: F4
case_images: Immediately after fibrinogen supplementation, a biopsy of the anterior mediastinum showed a population of immature T-lineage cells, consistent with early T-precursor (ETP) lymphoblastic leukaemia/lymphoma (blasts expressed CD3, CD4, CD7, CD117 and lacked CD2, CD1a, CD5, CD8, CD10, CD20, CD30, CD33, CD34, TdT, with a MIB1 70%-80%) (Figure 4).