You Make the Call
Each month in “ You Make the Call ,” we pick a challenging clinical question submitted through the Consult a Colleague program and post the expert ’ s response , but we also want to know what you would do . Send in your response to next month ’ s clinical dilemma and see how your answer matches up to the expert ’ s in the next print issue .
This month , Barbara A . Konkle , MD , discusses bleeding prevention in a patient undergoing spinal surgery .
TRAINING and EDUCATION
Clinical Dilemma :
I am evaluating a 60-year-old man for cervical spine surgery ( disc disease with neurologic symptoms ) who has a prolonged prothrombin time ( PT ). He appears to have mild factor VII ( FVII ) deficiency . His bleeding history is as follows : catheter ablation for atrial fibrillation ( followed by warfarin for 10 months without bleeding ), shockwave lithotripsy ( no bleeding problems ), and tooth extractions about 30 years ago ( followed by bleeding for about 2 days , with no need for reevaluation or hemostasis ). He has no problems with minor lacerations ( e . g ., shaving ) and is physically active in taekwondo . The initial abnormality that prompted referral was a baseline PT of 15.2 seconds , international normalized ratio ( INR ) of 1.3 , with normal partial thromboplastin time . Repeat PT was 13.8 seconds , INR 1.2 , with the following factor levels : FVII 46 percent , factor II 86 percent , factor V 87 percent , and factor X 87 percent . The FVII deficiency literature suggests that surgical bleeding is rare if FVII is above 10 percent . One discussion suggested that 30 percent should be okay , but I am concerned about this being a critical bleeding site .
Expert Opinion
Barbara A . Konkle , MD Associate Chief Scientific Officer and Associate Director , Washington Center for Bleeding Disorders Director , Hemostasis , Platelet Immunology and Genomics Laboratory , Bloodworks Northwest Professor of Medicine / Hematology , University of Washington
In determining the risk of bleeding in a patient with a rare factor deficiency , one must consider the factor level , information regarding bleeding risk for the specific deficiency , and the bleeding risk associated with the planned surgery .
Factor VII ( FVII ) deficiency is a rare bleeding disorder , which in the homozygous or compound heterozygous state has an estimated incidence of 1 in 500,000 individuals . 1 Patients with low FVII levels (< 10 %) commonly have symptoms of mucosal and procedure-related bleeding , and less commonly hemarthroses , hematuria , central nervous system and gastrointestinal bleeding . 1 , 2 The FVII level required for hemostasis is lower than for many other factors , and thus patients can have a FVII level low enough to result in a prolonged prothrombin time , even with a level above what is needed for hemostasis . The FVII hemostatic level generally has been thought to be 10 to 15 percent . The European Network of Rare Bleeding Disorders evaluated the relationship been bleeding and factor activity level in 489 patients with rare bleeding disorders , including 224 with FVII deficiency . 3 In FVII deficiency , a trough level greater than 20 percent rendered people asymptomatic .
The patient described above has a FVII level well above the hemostatic level . Care is warranted given the high bleeding risk of the surgery planned . However , there are risks associated with treatment of FVII deficiency using either fresh frozen plasma or recombinant factor VIIa , and one should avoid risks if there are no data to support benefit . That the patient has an essentially negative bleeding history and did well on warfarin , which would lower the FVII level further , provides assurance that he is not at increased bleeding risk . Given the influence of FVII on the prothrombin time , should he require warfarin therapy in the future , another measure of warfarin activity such as the chromogenic FX assay should be used for monitoring .
REFERENCES
1 . Palla R , Peyvandi F , Shapiro . Rare bleeding disorders : diagnosis and treatment . Blood 2015 ;
125 ( 13 ): 2052-2061 . 2 . Mariani G , Barnardi F . Factor VII deficiency . Semin Thromb Hemost 2009 ; 35 ( 4 ): 400-406 . 3 . Peyvandi F , Palla R , Menegatti M , et al . Coagulation factor activity and clinical bleeding severity in rare bleeding disorders : results from the European Network of Rare Bleeding Disorders . J Thromb Haemost . 2012 ; 10 ( 4 ): 615-621 .
Next Month ’ s Clinical Dilemma :
I am treating a 67-year-old man who is status / post a liver transplant four months ago . He had transfusion requirements one year prior to transplant , which have now increased . Absolute neutrophil count is variable ( 700- 6,000 / mm 3 ). Bone marrow biopsy is hypercellular ( 70 %) with myeloid left shift , but no dysplasia . Flow is negative . Myelodysplastic syndromes FISH panel is negative . Cytogenetics are normal . Myeloid next-generation sequencing shows the following mutations : TET2 ( 15-35 %), ASXL1 ( 40-50 %), and ZRSR2 ( 80-85 %). So , technically he has clonal cytopenias of undetermined significance ( CCUS ). His erythropoietin level is 680 mU / mL . What therapeutic options are available to reduce his transfusion requirement ? ●
Consult a Colleague Through ASH
Consult a Colleague is a service for ASH members that helps facilitate the exchange of information between hematologists and their peers . ASH members can seek consultation on clinical cases from qualified experts in 11 categories :
• Anemias
• Hematopoietic cell transplantation
• Hemoglobinopathies
• Hemostasis / thrombosis
• Lymphomas
• Lymphoproliferative disorders
• Leukemias
• Multiple myeloma & Waldenström macroglobulinemia
• Myeloproliferative neoplasms
• Myelodysplastic syndromes
• Thrombocytopenias
Assigned volunteers (“ colleagues ”) will respond to inquiries within two business days ( either by email or phone ).
Have a puzzling clinical dilemma ? Submit a question , and read more about Consult a Colleague volunteers at hematology . org / Clinicians / Consult . aspx or scan the QR code .
* If you have a request related to a hematologic disorder not listed here , please email your recommendation to ashconsult @ hematology . org so it can be considered for addition in the future .
DISCLAIMER : ASH does not recommend or endorse any specific tests , physicians , products , procedures , or opinions , and disclaims any representation , warranty , or guaranty as to the same . Reliance on any information provided in this article is solely at your own risk .
32 ASH Clinical News June 2018