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Prolonged Bleeding in a 34-Year-Old Man following Oral Surgery.

CASE DESCRIPTION

A 34-year-old healthy man underwent oral surgery and returned multiple times thereafter with recurrent bleeding from the surgical site. Over the course of 1 month and several visits, the bleeding was stopped temporarily with sutures and cauterization but recurred after each intervention. He had a history of excessive bleeding after dental extractions and developed a hematoma after a vasectomy. There was no family history of a bleeding disorder. Relevant coagulation studies are summarized in Table 1.

QUESTIONS TO CONSIDER

1. What is your differential diagnosis?

2. What tests help differentiate among these possibilities?

3. What is the inheritance pattern of this patient's disorder?

The answers are below.

ANSWERS

Low factor VIII (FVIII) [2] concentrations are associated with hemophilia A, type 2N von Willebrand disease (vWD), and combined factor V/VIII (CFV/VIII) deficiency. CFV/VIII deficiency is unlikely since both the prothrombin time (PT) and partial thromboplastin time (PTT) are not prolonged (1). The vWF: factor VIII binding assay distinguishes between hemophilia A and type 2N vWD (2, 3). In this case it was undetectable, diagnostic for type 2N vWD, an autosomal recessive form of vWD with normal platelet adhesion but low FVIII concentration due to rapid clearance (4).

Author Contributions: All authors confirmed they have contributed to the intellectual content of this paper and have met the following 3 requirements: (a) significant contribution to the conception and design, acquisition of data, or analysis and interpretation of data; (b) drafting or revising the article for intellectual content; and (c) final approval of the published article.

Authors' Disclosures or Potential Conflicts of Interest: Upon manuscript submission, all authors completed the author disclosure form. Disclosures and/or potential conflicts of interest:

References

(1.) Zheng C, Zhang B. Combined deficiency of coagulation factors Vand VIII: an update. Semin Thromb Hemost 2013; 39:613-20.

(2.) Mazurier C, Dieval J, JorieuxS, Delobel J, Goudemand M.A new von Willebrand factor (vWF) defect in a patient with factor VIII (FVIII) deficiency but with normal levels and multimeric patterns of both plasma and platelet vWF. Characterization of abnormal vWF/FVIII interaction. Blood 1990; 75:20-6.

(3.) Zhukov O, Popov J, Ramos R, Vause C, Ruden S, Sferruzza A, et al. Measurement of von Willebrand factor-FVIII binding activity in patients with suspected von Willebrand disease type 2N: application of an ELISA-based assay in a reference laboratory. Haemophilia 2009; 15:788-96.

(4.) Nichols WL, Hultin MB, James AH, Manco-Johnson MJ, Montgomery RR, Ortel TL, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008; 171-232.

Nicholas J. Olson, [1] * Zahra Shajani-Yi, [1] Mark A. Cervinski, [1] and Deborah L. Ornstein [1]

[1] Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center, Lebanon, NH; and Geisel School of Medicine, Hanover, NH.

* Address correspondence to this author at: Dartmouth-Hitchcock Medical Center, One Medical Center Dr., Lebanon, NH, 03756. Fax 603-650-7214; e-mail nicholas.j.olson@hitchcock.org.

Received July 14, 2016; accepted July 26, 2016.

DOI: 10.1373/clinchem.2016.264069

[2] Nonstandard abbreviations: FVIII, factor VIII; vWD, von Willebrand disease; CFV/VIII, combined factor V/VIII deficiency; PT, prothrombin time; PTT, partial thromboplastin time.
Table 1. Coagulation studies.

Test                      Result   Reference interval

PT                         14.3      12-15 s
INR (a)                     1.1     0.9-1.1
PTT                        37        25-35 s
Thrombin time              15        15-20 s
Fibrinogen                331       175-450 mg/dL
FVIII activity            23%      50%-150%
vWF antigen               52%          >50%
vWF activity              64%      50%-150%
vWF:FVIII binding assay    <0.12   0.73-1.42
ABO group                   O

(a) INR, international normalized ratio.
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Title Annotation:the Clinical Chemist: What Is Your Guess?
Author:Olson, Nicholas J.; Shajani-Yi, Zahra; Cervinski, Mark A.; Ornstein, Deborah L.
Publication:Clinical Chemistry
Date:Dec 1, 2016
Words:596
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