Factor VIII deficiency presenti”>Case Report

Contents lists available at ScienceDirect

American Journal of Emergency Medicine

journal homepage: locate/ ajem

Acquired factor VIII deficiency presenting with compartment syndrome?

Abstract

Acquired factor VIII deficiency is a rare disease that has high rates of mortality and morbidity. Although this Bleeding disorder has a classic presentation, its infrequency can make it difficult to identify. We present a case report of an Elderly woman who came to an emergency department with spontaneous bilateral lower extremity compartment syndrome. Although she presented with tachycardia and hypotension, her only laboratory abnormalities were an elevated activated partial thromboplastin time and anemia. She underwent bilateral fasciotomies and had a postoperative course that was complicated by significant incisional bleeding. She was ultimately found to have acquired factor VIII deficiency. Emergency physicians must be familiar with this rare entity because its recognition can avoid serious complications, particularly in those requiring operative management.

Acquired factor VIII deficiency is caused by an autoantibody against factor VIII. This is a rare disease with high rates of morbidity and mortality [1-4]. It has an annual incidence of 1 to 4 per million [2- 4] and a bimodal age distribution. A slight peak in incidence occurs with a female predominance between the ages of 20 and 30 years and a major peak occurring among elderly men and women between the ages of 68 and 80 years [2,4]. This bleeding disorder is often associated with autoimmune diseases, malignancy, pregnancy, drugs, and infections, but most cases are idiopathic [1-4]. Patients classically present with hemorrhages into Soft tissues, mucous membranes, skin, or muscle. Some are found to have an incidental isolated elevated Activated partial thromboplastin time during routine blood work, accompanied by a normal prothrombin time, international normalized ratio, and platelet count [1-4].

A 74-year-old woman with a history of hypertension, diabetes, and coronary artery disease presented to an emergency department (ED) with acute bilateral Lower extremity pain and swelling. She had not sustained any trauma, and she reported that she did not bruise or bleed easily and had no history of deep vein thrombosis. Her initial workup included unremarkable radiographs and bilateral venous duplex Ultrasound studies of the lower extremities. The patient was discharged home with instructions to return if her condition worsened.

Two days later, she returned to the ED, complaining of worsening pain and swelling. Her vital signs were as follows: blood pressure, 85/40 mm Hg; heart rate, 100 beats/min; temperature, 36.8?C; respiratory rate, 20 breaths/min; and oxygen saturation, 100% on room air. Her Left lower extremity was swollen, tender to palpation,

? The authors received no grant or other financial support for this work.

and grossly ecchymotic. Her right thigh had scattered areas of ecchymosis. Her dorsalis pedis and posterior tibilais pulses were diminished but palpable in both legs. Laboratory work revealed a hemoglobin level of 5.6 g/dL (baseline, 11 g/dL), hematocrit 17.9% (baseline, 35%), and an elevated aPTT of 72 seconds. Her international normalized ratio, prothrombin time, and platelet counts were within normal limits. A computed tomography angiogram demonstrated enlarged musculature with adjacent subcutaneous edema, raising concern about intramuscular hemato- mas in the right thigh and left calf with partial occlusion of both posterior tibial arteries (Fig.).

After initial Resuscitative efforts in the ED including transfusion with Packed red blood cells and fresh-frozen plasma , a vascular surgery team was consulted. Compartment pressures in both the right thigh and the left calf were higher than 50 mm Hg, raising a concern for compartment syndrome. The patient was taken to the operating room for emergent bilateral lower extremity fasciotomies. Her postoperative course was complicated by significant bleeding from her incision sites. She was transferred to the medical intensive care

Fig. A CT angiogram of the bilateral lower extremities demonstrates enlarged musculature with adjacent subcutaneous edema, Intramuscular hematomas in the right thigh and left calf, and partial occlusion of both posterior tibial arteries.

0735-6757/$ – see front matter (C) 2013

unit, where she was eventually diagnosed as having acquired factor VIII deficiency. The patient’s bleeding was temporarily controlled with FFP, recombinant factor VII and factor VIII, and aminocaproic acid (Amicar, Xanodyne Pharmaceuticals, Newport, KY). She was eventu- ally transferred to a different facility for novel therapy.

Acquired factor VIII deficiency occurs secondary to autoanti- bodies against factor VIII and, unlike congenital hemophilia, without a genetic inheritance pattern [1-3]. Owing to its rarity and nonspecific presentation, the diagnosis is often delayed, leading to high rates of mortality (8%-22%) [2,3]. It is essential to maintain a high index of suspicion for this disorder in elderly patients who present with nonspecific bleeding complaints and are found to have an isolated elevated aPTT. Although the formal diagnosis is made with a mixing study and a Bethesda study, both of which are not routine laboratory tests requested in the ED, it is important to consider this disease entity and to control the bleeding associated with it [1-4]. After initial stabilization and resuscitation, definitive management includes steps to eradicate the inhibitor and restore factor VIII activity. Bleeding control can be initiated in the ED with FFP, desmopressin, and recombinant factor VII and VIII products in addition to blood transfusion [1-4]. postoperative complications can be lessened if appropriate therapy is given before surgery. Therefore, recognition of acquired factor

VIII deficiency in the ED is particularly important for patients requiring surgical intervention.

Acquired factor VIII deficiency is a rare and lethal disease. It often presents with nonspecific bleeding complaints and an isolated elevated aPTT. Emergency physicians must consider this diagnosis and initiate appropriate therapy so as to minimize the development of complications from ongoing bleeding.

Acknowledgment

The authors acknowledge Linda J. Kesselring, MS, ELS, the technical editor/writer in the Department of Emergency Medicine, University of Maryland School of Medicine, for her contributions as copyeditor of the manuscript.

Thuy V. Pham MD

Department of Emergency Medicine University of Maryland Medical Center, Baltimore, MD

Carina A. Sorenson MD Departments of Emergency Medicine and Internal Medicine University of Maryland Medical Center, Baltimore, MD

Jose V. Nable MD

Department of Emergency Medicine University of Maryland School of Medicine, Baltimore, MD

E-mail address: [email protected] http://dx.doi.org/10.1016/j.ajem.2013.09.022

References

1. Baudo F, Caimi T, De Cataldo F. Acquired haemophilia: diagnosis and treatment of acquired haemophilia. Haemophilia 2010;16(102):102-6.
2. Franchini M, Giuseppe L. Acquired factor VIII inhibitors. Blood 2008;112(2):250-5.
3. Ma AD, Carrizosa D. Acquired factor VIII inhibitors: pathophysiology and treatment. Hematology Am Soc Hematol Educ Program 2006:432-7.
4. Shander A, Walsh C, Cromwell C. Acquired hemophilia: a rare but life-threatening potential cause of bleeding in the intensive care unit. Intensive Care Med 2011; 37(8):1240-9.