Inherited fibrinogen disorders (IFDs) are rare, and limited evidence-based management guidelines exist, especially regarding haematological malignancies and haematopoietic stem cell transplantion (HSCT). This case highlights the successful multidisciplinary management of an adult patient with acute Philadelphia-negative B-cell lymphoblastic leukaemia (B-ALL) and IFD from induction through HSCT. A patient in his 20s with an IFD is diagnosed with B-ALL and receives induction chemotherapy, including dose-reduced pegaspargase (PEG-Asp), blinatumomab consolidative therapy and HSCT for post-induction measurable residual disease (MRD) positivity. Management involved prophylactic anticoagulation (AC) during induction and pharmacokinetic-guided fibrinogen replacement. Complications were iatrogenic intracranial hypotension and acute catheter-associated proximal deep vein thrombosis managed with therapeutic AC. One year post-HSCT, he remained MRD-negative with 100% donor chimerism. This case highlights a management strategy for patients with IFD requiring chemotherapy and/or HSCT and addresses a critical gap in knowledge.
PEREZ-MELENDEZ et al. (Mon,) studied this question.