PB0574 - Severe Bleeding in an Acquired Factor XIII Deficiency in an Otherwise Healthy Patient

Monday, June 26, 2023

18:30 – 19:30 ET

Room: Exhibition

Co-Author(s)

BS

Bianca Santonastaso

Laboratory Technician
Department of Pathology and Laboratory Medicine, Transfusion Medicine Unit, University of Rochester Medical Center, Rochester, New York, USA
Rochester, New York, United States
HS

Hannah Spector

Laboratory Supervisor
Department of Pathology and Laboratory Medicine, Transfusion Medicine Unit, University of Rochester Medical Center, Rochester, New York, USA
Rochester, New York, United States
CC

Christine Cahill

Nurse Coordinator of the Patient Blood Management Program
Department of Pathology and Laboratory Medicine, Transfusion Medicine Unit, University of Rochester Medical Center, Rochester, New York, USA
Rochester, New York, United States
FA

Frank Akwaa

Hematologist
Internal Medicine Department, Hematology Unit, University of Rochester Medical Center, Rochester, New York, USA
Rochester, New York, United States
JI

Jainulabdeen J. Ifthikharuddin

Oncologist
Internal Medicine Department, Hematology Unit, University of Rochester Medical Center, Rochester, New York, USA
Rochester, New York, United States
GG

Gahyun Gim

Hematology and Medical Oncology Fellow
Internal Medicine Department, Hematology Unit, University of Rochester Medical Center, Rochester, New York, USA
Rochester, New York, United States

Presenting Author(s)

Majed A. Refaai, MD

Professor
Department of Pathology and Laboratory Medicine, Transfusion Medicine Division, Hemostasis and Thrombosis Unit, University of Rochester Medical Center
Rochester, New York, United States

Background: Factor XIII (FXIII) catalyzes the formation of covalent bonds between fibrin monomers, stabilizes fibrin clot, and protects it from premature fibrinolysis. FXIII deficiency, which is extremely rare, impairs clot stability and increases bleeding tendency.

Aims: Case Report

Methods: A 76-year-old male with Grave’s Disease, asthma, and hypertension presented to ED with exertional fatigue, pain across his left flank, and ecchymosis. No new medical conditions or medications were recognized. Work-up revealed extensive left flank hematoma on CT chest and abdomen, acute anemia, normal basic coagulation studies with unexplained markedly reduced FXIII levels (Table).

Results: He received 2 doses of cryoprecipitate and 2 units of red blood cells (RBC). However, he continued to develop new expanding ecchymosis and hematoma on the left back side. Bleeding subsequently improved, and he was discharged on aminocaproic acid and prednisone for presumed FXIII inhibitor.

Three days later he was readmitted with weakness, fatigue, swelling and numbness in his latissimus dorsi and lateral right thigh, and ecchymosis and hematoma on the abdominal wall. He then developed a bilateral hemothorax and arterial bleed in his thigh. He underwent an angiogram with embolization procedure, received 2 RBCs, 3 doses of cryoprecipitate, tranexamic acid (TXA), and multiple FXIII concentrate infusions (40U/Kg). Over the next few days, bleeding was controlled with FXIII levels maintained between 50-60% with intermittent FXIII concentrate infusions (Figure). He was started on weekly Rituximab along with weekly concentrated FXIII replacement therapy in addition to prednisone and steroids.

Conclusion(s): FXIII level of >5% is well supported in the literature to be hemostatically adequate; severe bleedings are reported at levels of < 1-3%. Although not identified serologically, the presence of FXIII inhibitor in this case is obvious clinically. This is indicated by significant bleeding, persistently low FXIII levels, suboptimal response to cryoprecipitate and FXIII concentrate replacement therapy, and the favorable response to immunotherapy.