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Case Reports
. 2024 Jan 8:10:1335296.
doi: 10.3389/fcvm.2023.1335296. eCollection 2023.

Severe haemorrhagic diathesis due to acquired hypofibrinogenemia in a patient with early T-cell precursor acute lymphoblastic leukaemia/lymphoma: a case report

Luca Spiezia  1 , Marcello Riva  2 , Carmela Gurrieri  2 , Elena Campello  2 , Paolo Simioni  1
Affiliations
Case Reports

Severe haemorrhagic diathesis due to acquired hypofibrinogenemia in a patient with early T-cell precursor acute lymphoblastic leukaemia/lymphoma: a case report

Luca Spiezia et al. Front Cardiovasc Med. .

Abstract

The most frequent haematological malignancy associated with acquired hypo/dysfibrinogenemia is multiple myeloma. We present an unusual case of severe haemorrhagic diathesis due to acquired hypofibrinogenemia in a patient with early T-cell precursor acute lymphoblastic leukaemia/lymphoma (ETP-ALL/LBL). A 57-year-old male was admitted to the General Internal Medicine Department of Padova University Hospital for acute massive haematomas of the left lower extremity associated with macrohaematuria. Coagulation tests showed prolonged prothrombin time, activated partial thromboplastin time and thrombin time due to isolated severe hypofibrinogenemia (antigen 0.70 g/L and activity 26%). The radiological workup showed a bulky lesion located in the anterior mediastinum, and a biopsy led to the diagnosis of ETP-ALL/LBL. Fibrinogen replacement therapy failed to correct the bleeding diathesis and we were able to exclude other frequent causes of acquired hypofibrinogenemia (i.e., liver dysfunction, fibrinogen-specific antibody or drug toxicity); therefore, we hypothesised that hypofibrinogenemia might stem either from enhanced removal of fibrinogen from the circulation or consumptive coagulopathy. Notably, only after initiating a specific chemotherapy treatment did the patient start showing improvement in bleeding symptoms and achieve normal fibrinogen levels.

Keywords: acquired hypofibrinogenemia; case report; consumptive coagulopathy; early T-cell precursor acute lymphoblastic leukaemia/lymphoma; haemorrhagic diathesis.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Figures

Figure 1
Figure 1
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; α, α-angle; MCF, maximum clot firmness; ML, maximum lysis.
Figure 2
Figure 2
The levels of fibrinogen during hospitalization.
Figure 3
Figure 3
PET/MRI scan.
Figure 4
Figure 4
Histological and immunohistochemical findings in the biopsy of the bulky lesion in the superior-anterior mediastinum.
See this image and copyright information in PMC

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