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Sudden Severe Bleeding in a Patient with Hemochromatosis: Liver Failure or Something Else?

CASE DESCRIPTION

A 51-year-old man with hereditary hemochromatosis presented with hematuria and bleeding gums. He had no history of a bleeding disorder or abnormal bleeding. He was taking chlorthalidone and losartan for hypertension but took no other prescribed medications or over the counter supplements. He was adherent to a therapeutic phlebotomy schedule to control iron overload. Hemoglobin and platelet count were within reference intervals, and coagulation testing showed markedly prolonged clotting times and multiple coagulation factor deficiencies (Table 1).

QUESTIONS

1. What are acquired causes of multiple coagulation factor deficiencies?

2. What parts of this patient's medical history are important to consider?

3. What is the most likely cause of the coagulopathy in this scenario?

The answers are below.

ANSWERS

Acquired conditions causing multiple coagulation factor deficiencies include disseminated intravascular coagulation (DIC), liver disease, and vitamin K deficiency (1). DIC would be unusual with a normal platelet count, and, although cirrhosis is a complication of hemochromatosis (2), factor V should also be decreased (3). This pattern is consistent with vitamin K deficiency, which was confirmed when warfarin, a vitamin K antagonist, was detected by LC-MS/MS in the patient's serum. Warfarin had not been prescribed; rather it was a prescription filling error.

Author Contributions: All authors confirmed they have contributed to the intellectual content of this paper and have met the following 3 requirements: (a) significant contributions 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: No authors declared any potential conflicts of interest.

References

(1.) Schmaier AH, Miller JL. Coagulation and fibrinolysis. In: Henry JB, McPherson RA, Pincus MR. Henry's clinical diagnosis and management by laboratory methods. 2nd ed. Philadelphia (PA): Elsevier Saunders; 2011. p 785-800.

(2.) Pietrangelo A. Hereditary hemochromatosis: Pathogenesis, diagnosis, and treatment. Gastroenterology 2010;139:393-408.

(3.) Tripodi A. Hemostasis abnormalities in cirrhosis. Curr Opin Hematol 2015;22: 406-12.

Nicholas J. Olson, [1] * Mark A. Cervinski, [1,2] and Deborah L. Ornstein [1,2]

[1] Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH; [2] Geisel School of Medicine, Hanover, NH.

* Address correspondence to this author at: Dartmouth-Hitchcock Medical Center, One Medical Center Dr., Lebanon, NC. Fax603-650-7214; e-mail nicholas.j.olson@hitchcock.org.

Received June 6, 2016; accepted June 16,2016.

DOI: 10.1373/clinchem.2016.261941
Table 1. Coagulation studies.

Test          Result      Reference Interval

PT (a)        91.3s       12.5-15.5 s
aPTT          128s        25-35 s
Fibrinogen    495 mg/dL   175-400 mg/dL
Factor II        5%            50%-150%
Factor V         98%           50%-150%
Factor VII       10%           50%-150%
Factor IX        8%            50%-150%

(a) PT, prothrombin time; aPTT, activated partial thromboplastin time.
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Title Annotation:the Clinical Chemist: What Is Your Guess?
Author:Olson, Nicholas J.; Cervinski, Mark A.; Ornstein, Deborah L.
Publication:Clinical Chemistry
Date:Dec 1, 2016
Words:458
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