American Journal of Emergency Medicine 33 (2015) 129.e1-129.e3

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American Journal of Emergency Medicine

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Case Report

The intractable intra-Abdominal hemorrhage with unknown etiology in a patient with severe hemophilia A?

Abstract

Severe hemophilia patients are more likely to be complicated by intra-articular hemorrhage, subcutaneous hemorrhage, and intra- mascular hemorrhage. Spontaneous intra-abdominal hemorrhage is a rare fatal disease, which is an arterial bleeding of uncertain causes from vessel feeding arteries. In case the spontaneous intra-abdominal hemorrhage is complicated to severe hemophilia patients, the mortality rate increases considerably. We experienced a patient with severe hemophilia A, who made a Full recovery from spontane- ous intra-abdominal hemorrhagic shock by replacement therapy of coagulation factor VII, a noninvasive procedure.

Because of sudden-onset severe abdominal pain, a 60-year-old man with severe hemophilia A was taken to the emergency department in our hospital. Initially, he received a regular replace- ment therapy of coagulation factor VII for possible hemorrhage. His blood pressure, heart rate, and respiratory rate were 70/40 mm Hg, 130 per minute, and 36 per minute, respectively. His extremities were cold. He exemplified an Abdominal distention and a pressure pain at the left lower quadrant. There have been no histories of abdominal trauma. The test for occult blood in his stool was negative.

We found a considerable amount of echo-free space in the abdominal cavity. His hemoglobin concentration was 7.5 g/dL, which decreased from 14 g/dL within a month, and the creatinine level was 2.1 mg/dL as a reflection of hypovolemia. Later, it was clarified that coagulation factor VII activity was less than 1%. The inhibitors for coagulation factor VII have not been detected. Abdominal computed tomographic scanning with intravenous con- trast also revealed a huge amount of intra-abdominal hemorrhage, which was about 70 x 50 x 75 mm in diameter in the presence of an extravascular leakage near the sigmoid colon (Figure a and b). Eventually, spontaneous intra-abdominal hemorrhage with severe hemophilia A was diagnosed.

We administered him recombinant factor VII intravenously and also carried out blood and fresh frozen plasma transfusions. The angiography identified an extravascular leakage from mesenteric arteries close to a sigmoid colon (Figure c and d). After the injection of recombinant factor VII, the extravascular leakage was expeditiously vanished (Figure e), and his vital signs became stabilized. To avoid possible occurrence of radiocontrast nephropathy, we determined to

? Conflict of interest: None declared.

treat him with noninvasive procedure, instead of arterial emboliza- tion. We administered the recombinant coagulation factor VII every 12 hours to keep the coagulation activity higher than 80%, similar to the case in gastrointestinal bleedings with hemophilia [1]. The supplementation of recombinant coagulation factor VII was continued for 14 days, and he was discharged on foot.

Spontaneous intra-abdominal hemorrhage is a rare disease, which is an arterial bleeding of uncertain causes from vessel feeding arteries [2]. It sometimes causes an intestinal ischemia or ileus by hematoma. Although it is unclear whether hemophilia is a risk of spontaneous intra-abdominal hemorrhage, it should be noted that spontaneous intra-abdominal hemorrhage in hemophilia results in severe outcome compared to that in healthy individual [3]. Spontaneous intra- abdominal hemorrhage of hemophilia was reported for the first time in 1976, and to date, 11 cases have been reported by 2009 in the world [2-6]. It occurs most commonly at retroperitoneum (5 cases) and great omentum, minor omentum, mesenterium (one case in each). They were treated with the recombinant coagulation factor VII (11 cases) and there have been no cases of the surgical operation. The spontaneous intra-abdominal hemorrhage may happen also in healthy individual [7]. Spontaneous intra-abdominal hemorrhage in healthy individual was reported for the first time in 1908, and to date, 110 cases have been reported until 1998 [7]. Generally, spontaneous intra-abdominal hemorrhages show a good indication of a surgical procedure. The mortality rate is 8.6% in patients with arterial bleeding of defined origin [8]. On the other hand, in cases that were not detected bleeding sites, the mortality rate was more than 50% [8]. Patients with severe hemophilia are handicapped with invasive procedure; therefore, they would be more fatal than spontaneous intra-abdominal hemorrhage in the healthy cases.

In summary, we achieved a good clinical course by noninvasive

procedure to a hemorrhagic shock case with severe hemophilia caused by spontaneous intra-abdominal hemorrhage. In the process of reach- ing into hemostasis, the elevation of abdominal pressure is a critical factor. Some cases may control this fatal state by conservative medical management as the recombinant coagulation factor VII replacements. As patients with severe hemophilia are disadvantaged for emergent surgical procedure, nonsurgical procedure would be anticipated for spontaneous intra-abdominal hemorrhage.

Acknowledgments

The authors deeply appreciate Yoshiaki Azama, MD, Department of Radiology, University of Ryukyus Hospital, for trying to administer Arterial embolization in the present case. All authors have reviewed the manuscript.

0735-6757/(C) 2014

129.e2 K. Morichika et al. / American Journal of Emergency Medicine 33 (2015) 129.e1-129.e3

Kazuho Morichika, MD Takeaki Tomoyose, MD, PhD

Yukiko Nishi, MD Sawako Nakachi, MD, PhD

Division of Endocrinology, Diabetes and Metabolism Hematology, Rheumatology (Second Department of Medicine) Graduate School of Medicine, University of the Ryukyus

Nakagami, Okinawa, Japan

Takuya Fukushima, MD, PhD Laboratory of Hematoimmnology, School of Health Sciences Faculty of Medicine, University of the Ryukyus

Hiroaki Masuzaki, MD, PhD Division of Endocrinology, Diabetes and Metabolism Hematology, Rheumatology (Second Department of Medicine) Graduate School of Medicine, University of the Ryukyus

Nakagami, Okinawa, Japan E-mail address:[email protected]

http://dx.doi.org/10.1016/j.ajem.2014.06.038

References

1. Hemophilia of Georgia USA. Protocols for the treatment of hemophilia and von Willebrand Disease. Montreal: World Federation of Hemophilia; 2008.
2. Jones JJ, Kitchens CS. Spontaneous intra-abdominal hemorrhage in hemophilia. Arch Intern Med 1984;144(2):297-300.
3. Yoshimi A, Takahashi T, Motokura T, Yatomi Y, Chiba S, Kurosawa M. Hemoper- itoneum due to Spontaneous rupture of the left gastroepiploic artery in a patient with hemophilia A. Ann Hematol 2009;88:811-2.
4. Tsuchimochi N, Nagafuji K. Spontaneous lesser sac haematoma in a haemophiliac. Br J Haematol 2004;126:287.
5. Santagostino E, Morfini M, Rocino A, Baudo F, Scaraggi FA, Gringeri A. Relationship between factor VII activity and clinical efficacy of recombinant factor VIIa given by continuous infusion to patients with Factor VIII inhibitors. Thromb Haemost 2001;86(4):954-8.
6. Adelman MI, Gishen P, Dubbins P, Mibashan RS. Localised intramesenteric haemorrhage-a recognisable syndrome in haemophilia? Br Med J 1979;15 (9):642-3.
7. Carmeci C, Munfakh N, Brooks JW. Abdominal apoplexy. South Med 1998;91:273-4.
8. Reilly EF, Blansfield JA, Zebley DM. Spontaneous colonic mesenteric hemorrhage: report of an unusual case of abdominal apoplexy. Dis Colon Rectum 2005;48 (7):1484-6.

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   Figure. a, Computed tomographic scanning with intravenous contrast revealed a massive amount of intra-abdominal fluid. b, The extravascular leakage was identified near the sigmoid colon. Surrounding intestine was considerably edematous. c, The extravascular leakage from mesenteric artery was identified by the angiography surrounded by arrows. d, Three-dimensional Digital subtraction angiography revealed arterial bleeding at the same place observed by the computed tomographic scanning. e, After the injection of recombinant factor VII, the extravascular leakage was expeditiously vanished.