PB1200 - Clinicopathological spectrum and outcome of patients with acquired coagulation factor inhibitors

Tuesday, June 25, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Dinesh Chandra, MD, DM

Associate Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India

Co-Author(s)

AS

Anu Singh

Senior resident
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Lucknow, Uttar Pradesh, India
RG

Ruchi Gupta

Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India
MS

Manish Kumar Singh

Associate Professor
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Lucknow, Uttar Pradesh, India
KR

Khaliqur Rahman

Additional Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India
SY

Sanjeev Yadav

Associate Professor, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India
RK

Rajesh Kashyap

Professor & Head, Department of Hematology
Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow India
Lucknow, Uttar Pradesh, India

Background: Acquired Coagulation Factor Inhibitors (aCFI) are a rare group of bleeding disorders, seen most commonly against factor VIII (FVIII) and von Willebrand factor (VWF) while inhibitors against other coagulation factors are rare. Hence there is limited data available on their clinical manifestations and management.

Aims: To evaluate the clinical phenotype and treatment outcome of patients with aCFI.

Methods: A retrospective analysis of all patients with aCFI, diagnosed and treated between January 2020 and October 2023, was carried out. Clinical and laboratory details were extracted from electronic medical records. Diagnosis was established based on the clinical presentation, coagulation profile, specific factor assays, inhibitor screening and Bethesda assay. Patients with congenital factor deficiency with inhibitors were excluded.

Results: Twelve patients (seven males and five females) with a median age of 46 years (11–73 years) were diagnosed with acquired CFI. The median delay from first symptoms to diagnosis was 17 days (7-40 days). There were 5 patients of acquired hemophilia A (AHA), two patients of lupus anticoagulant with hypoprothrombinaemia syndrome (LA-HPS), 2 cases of acquired VWD and one each of acquired FX, FV and heparin-like anticoagulant. The degree of bleeding symptoms was variable ranging from asymptomatic to severe bleeds. Patients with AHA and LA-HPS, presented with severe bleeding as compared to other aCFI. An underlying cause for the acquired inhibitor was observed in eight patients while in the rest it was idiopathic. Patients of acquired VWD and FX inhibitors were treated conservatively, while other patients were managed using FFP, bypassing agents (rFVII and/or FEIBA), corticosteroids and/or Rituximab. Four patients did not achieve remission and died due to excessive bleeding.

Conclusion(s): A high index of suspicion and prompt diagnosis is mandated to optimise treatment of patients with aCFI. Immunosuppression to eradicate an inhibitor should be started as early as possible to reduce mortality.