The importance of a multi-disciplinary approach in a severe form of Ahemophilia case

Matei Amalia , Badelita Sorina , Crisan Manuela , Valentina Uscatescu , Mihalcea Razvan , Botnariuc Inga, Conf. Dr. Coriu Daniel Institutul Clinic Fundeni

Patients profile:
50-year old male Retired From a rural area

Patients profile:
A patient with a history of: - Severe A Hemophilia - Type 2 Diabetes mellitus - High blood pressure - Fracture of the right femoral diaphysis previously stabilized through Ender and plaster of Paris Arad County Hospital

Present medical history

Presents with upper gastrointestinal tract bleeding.
After a fall, the patient suffered a bifocal right femoral diaphysis fracture, followed after 48 hours by an upper GI tract bleeding. He received local hemostatic treatment during OGD (epinephrine) and an clips application directly on the ulcer scar

 Due to the persistence of the upper GI bleeding and of the high serum concentration of anti-F VIII inhibitors (6,73-11,248,5UB), patient is referred to Timisoara County Hospital for adequate treatment. Here he received hemostatic treatment and substitution therapy (FFP, cryoprecipitate, Novoseven- F VIIa?, recombinant F VIII), systemic therapy with proton-pump inhibitors.

Due to the local limited resources and funding, his upper GI tract hemorrhage couldnt be stopped.
The patient was referred to the Fundeni Clinical Institute for adequate treatment.

 Physical examination: - altered general status - important skin pallor - naso-gastric catheter with >1000 mL of bloody fluid, associated with more than10 black stools - right leg plaster of Paris

 Laboratory findings: - HGB=8,1g/dl ;HCT=24,6%, MCV=90,1fl

,WBC=20860/mmc PLT=200.000/mmc,(Bl1,ProM1,Mi1,Mt1,N8,S68, E1,B1,L10, M8) - Peripheral blood smear showed: moderate hypocromia and moderate poikilocytosis

ALT= 30 U/l; AST=31 U/l ; GGT=42u/l; ALP=37 U/l; TBil = 1 mg/dl Creat=0,49 mg/dl ;BUN=66,4mg/dl Gluc=285,4 mg/dl; LDH =423 U/l Uric Acid=1,8 mg/dl Cl=95,8 mmol/l; K= 3,9 mmol/l Na=133mmoL/l Ferritin=135,9 ng/ml(Normal ranges 25-280ng/ml)

 Coag panel: PT=14,9 sec( Normal values=12-16sec)

APTT= 91.9sec-110sec-161.3-92.6sec90.1sec-87.8sec (Normal values =22-33sec) INR=1.31(Normal values =0.85-1.3) Fibrinogen=222.4mg/dl-197mg/dl-267mg/dl

 Factor VIII (baseline level) < 1%

AntiFVIII:c inhibitors = 50 UB Fibrin degradation products ++

D-Dimers=1,75 microg/dl

 First OGD: normal esophagus, stomach filled with a large quantity of fresh blood, from a 7cm ulcer over the gastric angle Forrest II C. Diffuse bleeding due to hemorhagic gastritis.
Multiple clips and a tri-clip application followed by continuous perfusion of PPIs.

 Due to active gastric bleeding and because of the high plasma concentration of anti F VIII inhibitors= 50 BU, we decided to administer FEIBA and recombinant F VII concentrate.

Due to persistent hemorrhage we have decided to start this patient on recombinant F VIII. After recombinant F VIII treatment, the GI bleeding stopped and a decrease in the number of melenic stools were shown.

 Over the next few days, the general status worsened and did not showed any improvements in the Coag. Panel (prolonged aPTT, decreased Fibrinogen). At this moment a differential diagnosis regarding his right femoral fracture appeared: local hematoma vs. local septic complication. The orthopedic consult indicated that there was a multi-focal right femoral fracture (intercondilian, supracondilian, diaphysal and 1/3 distal femoral fracture).

 Patient is transferred to the Orthopedic Clinical Hospital Bucharest, where he undergoes minimal invasive surgery - Ender rods osteosynthesis.
Good post-op evolution, with an optimal plasmatic concentration of F VIII (19% before the intervention and 21% after the procedure).

 10 days after the surgery patient presented: - headaches - vomiting - amaurosis (both eyes) with sudden onset and rapid progressive worsening.

 Neurological consult showed: - altered mental status, drowsiness - right eye mydriasis - left photomotor reflex + - bilateral Babinski reflex + - meningeal signs+, stiff neck+ - left hemiparesis Cerebral CT scan: - voluminous right hemisphere subdural hematoma associated cerebral edema and high risk of brain herniation.

 Patient was transferred to Bagdasar-Arseni Emergency Hospital, where he was admitted to the Neurosurgery Department, and the subdural hematoma was drained.

Neurologic post-op evolution was favorable.

 He was transferred back to Fundeni Hematology Department. The last OGD showed no signs of active bleeding in the upper GI tract. Patients general status improved over the next few days, without any other bleeding episode.

Conclusions
A 50 year old male with severe form of Ahemophilia associated with multiple severe complications: - type 2 diabettes mellitus - right femoral multi-focal fracture, - multiple gastric ulcers - right subdural hematoma

Conclusions
The need for a multi-disciplinary teamHemathology, Neurosurgery, neurology, Diabetes, ICU, Cardiology, Orthopedics. An increased need for intensive treatment (packed red blood cells, FFP, cryoprecipitate)- 130.000 UI recombinant F VIII, 45 FEIBA.. vials and 50 recombinant F VII vials.

Conclusions
High costs who were covered mainly by the hospital local budget, and in a small proportion by the National Hemophilia Program.

Thank you!