# A nurse is assessing a 12-year-old child with suspected von Willebrand's disease. Which assessment finding would be most characteristic of this condition?

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## 문제

A nurse is assessing a 12-year-old child with suspected von Willebrand's disease. Which assessment finding would be most characteristic of this condition?

The nurse is evaluating a pediatric patient for bleeding disorders.

## 보기

1. Petechiae and purpura on the trunk and extremities
2. Prolonged bleeding after dental procedures or minor cuts **✔ 정답**
3. Deep muscle hematomas and joint bleeding
4. Severe nosebleeds requiring emergency intervention

**정답: 2**

## 해설

Von Willebrand's disease is characterized by prolonged bleeding from mucosal surfaces and after minor trauma due to defective platelet adhesion and aggregation.

Von Willebrand disease is the most common inherited bleeding disorder, affecting about 1% of the population. It is caused by a deficiency or dysfunction of von Willebrand factor (vWF), a protein essential for normal blood clotting.

Von Willebrand factor performs two important functions. First, it helps platelets adhere to damaged blood vessel walls, and second, it acts as a carrier protein for factor VIII, which is needed for the clotting process. When this factor is deficient or does not function properly, platelet aggregation and adhesion are impaired, leading to prolonged bleeding time.

The characteristic bleeding pattern of von Willebrand disease is mucocutaneous bleeding. This refers to bleeding that occurs from mucosal and skin surfaces. Patients commonly experience prolonged bleeding after dental procedures, tooth extractions, minor cuts, or surgery. This is because the defective von Willebrand factor cannot adequately support platelet plug formation at sites of vascular injury.

Bleeding in this disorder tends to be persistent rather than severe, and is superficial rather than deep tissue bleeding. Female patients may experience heavy menstrual bleeding, and pediatric patients may present with frequent nosebleeds, easy bruising, and prolonged bleeding from minor wounds.

In nursing assessment, it is important to obtain a detailed bleeding history regarding prolonged bleeding after dental treatment, surgery, or minor trauma. Since this is a hereditary condition, family history is also an important assessment element.

## 심화 해설

Correct Answer: 2. Prolonged bleeding after dental procedures or minor cuts

Rationale

Von Willebrand disease (VWD) is the most common inherited bleeding disorder, characterized by a deficiency or dysfunction of von Willebrand factor (VWF) [1][2]. VWF plays a critical role in hemostasis by mediating platelet adhesion and aggregation at sites of vascular injury, and it also serves as a carrier protein for factor VIII, protecting it from proteolytic degradation [2][3].

Because the primary defect in VWD involves platelet plug formation—the first phase of hemostasis—the clinical hallmark of this condition is mucocutaneous bleeding [2]. This includes easy bruising, epistaxis, gingival bleeding, and notably, prolonged oozing from superficial wounds. The characteristic finding of prolonged bleeding after dental procedures or minor cuts directly reflects the impaired platelet adhesion mechanism, where an inadequate VWF-mediated bridge between platelets and the exposed subendothelium results in a failure to form a stable platelet plug [2]. This is why option 2 is the most characteristic assessment finding.

Analysis of Incorrect Options

Option 1: Petechiae and purpura on the trunk and extremities.

While mucocutaneous bleeding such as easy bruising is common in VWD, petechiae are more characteristic of thrombocytopenia or platelet dysfunction disorders. In VWD, the bleeding pattern typically involves larger ecchymoses and mucosal surface bleeding rather than pinpoint capillary hemorrhages. The case reports describe presentations like profuse epistaxis, gingival bleeding, and menorrhagia, not primarily petechial rashes [3][4].

Option 3: Deep muscle hematomas and joint bleeding.

Deep tissue bleeding, including intramuscular hematomas and hemarthrosis (joint bleeding), is the hallmark of coagulation factor deficiencies such as hemophilia A (factor VIII deficiency) or hemophilia B (factor IX deficiency). In VWD, the secondary deficiency of factor VIII can occur because VWF stabilizes factor VIII; however, unless the VWF deficiency is severe (as in Type 3 VWD), the factor VIII level is usually not low enough to cause spontaneous deep tissue or joint bleeding [3]. The mucocutaneous pattern is the dominant feature.

Option 4: Severe nosebleeds requiring emergency intervention.

Epistaxis is a very common symptom in VWD and can be profuse, especially in pediatric patients with severe forms of the disease [3]. However, the question asks for the most characteristic finding. While severe epistaxis can occur, it is not as universally and specifically indicative of a primary platelet adhesion defect across all types and severities of VWD as the prolonged oozing from a standardized challenge like a dental extraction or minor laceration. The prolonged bleeding after minor cuts is a classic, pathognomonic presentation that directly demonstrates the failure of primary hemostasis, making it a superior discriminator for a screening assessment question. Furthermore, chronic iron deficiency anemia secondary to long-standing menorrhagia is frequently an initial manifestation in undiagnosed individuals, reinforcing that the bleeding is often slow, persistent, and mucosal, rather than always acutely emergent [4].References (research sources)

- [1]Spontaneous intradural extramedullary hematoma after mild exercise in Von Willebrand disease: A rare clinical presentation and literature review.Research articleBukhari A, Lagaras A, Dherijha M, Malik A, Shahid A. (2026) · DOI: 10.25259/sni_1357_2025

- [2]Concomitant acquired and inherited von Willebrand disease: A challenging bleeding disorder.Research articleSareen NJ, Friedman KD, Sullivan MJ, De Sancho MT. (2026) · DOI: 10.1111/trf.70251

- [3]Type 3 Von Willebrand disease: two clinical cases of a rare disorder.Research articleBelcadi Abassi K, Larbi Ouassou K, Radi A, Laaraje A, Babour S, Ait Hmadouch S, Hassani A, Khorassani MEL, Abilkassem R. (2026) · DOI: 10.1093/omcr/omag037

- [4]Chronic Iron Deficiency Anemia as the Initial Manifestation of Undiagnosed Von Willebrand Disease in a Woman With Long-Standing Menorrhagia: A Case Report.Case reportRafique S, Rafiq I. (2026) · DOI: 10.7759/cureus.108255

## 임상 시나리오

Recognizing von Willebrand Disease Bleeding PatternsDistinguishing Mucocutaneous from Deep Tissue Hemorrhage
The primary defect in von Willebrand disease (VWD) is impaired platelet plug formation, leading to hallmark mucocutaneous bleeding. Assess for prolonged oozing after dental extractions, minor cuts, or easy bruising.

In contrast, hemophilia A or B involves coagulation factor deficiencies (VIII or IX) and presents with deep tissue bleeding such as hemarthrosis and intramuscular hematomas. This distinction is critical for diagnostic workup.

CautionDo not rely solely on epistaxis severity; recurrent, prolonged bleeding from superficial wounds is more specific to VWD than severe, isolated nosebleeds.

## 핵심 개념

- **von Willebrand factor (VWF)** — A plasma glycoprotein that mediates platelet adhesion to injured vessel walls and carries factor VIII to prevent its degradation.
- **Mucocutaneous bleeding** — Bleeding from mucosal surfaces (nose, gums, GI tract) and skin, characteristic of platelet plug formation defects like VWD.
- **Hemostasis phases** — Primary hemostasis involves platelet plug formation (VWF-dependent); secondary hemostasis involves the coagulation cascade (factor-dependent).
- **Hemophilia** — An inherited bleeding disorder from factor VIII or IX deficiency, marked by deep tissue and joint bleeding, not primarily mucocutaneous.
- **Platelet adhesion** — The initial step of primary hemostasis where platelets bind to exposed subendothelial collagen via VWF bridges.

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