acquired von Willebrand syndrome

**Acquired Von Willebrand Syndrome (AVWS):

Acquired Von Willebrand Syndrome (AVWS) Signs and Symptoms

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that can cause mild to severe symptoms. The signs and symptoms of AVWS depend on the severity of the condition, but they often include:

• Easy bruising: People with AVWS may experience easy bruising or petechiae (small red spots) on their skin.
• Frequent nosebleeds: Nosebleeds that last longer than 10 minutes and occur five or more times a year are common in individuals with AVWS.
• Bleeding gums: Bleeding gums can be a sign of AVWS, especially if it's accompanied by other symptoms like easy bruising or frequent nosebleeds.
• Excessive bleeding after an injury or cut: People with AVWS may experience prolonged bleeding or oozing following an injury or cut.
• Heavy periods: Women with AVWS may experience heavy menstrual bleeding that lasts longer than usual.

Type and Severity of Symptoms

The type and severity of symptoms in AVWS can vary from person to person. Some people may have mild symptoms, while others may experience more severe bleeding episodes. The symptoms can also depend on the underlying cause of AVWS, such as a blood disorder or a medical condition that affects the blood vessels.

References:

• [1] Acquired von Willebrand syndrome is a highly heterogeneous bleeding disorder, usually characterized by mild to moderate hemorrhagic symptoms that may sometimes be severe. (Source: 13)
• People with this condition often experience easy bruising, long-lasting nosebleeds, and excessive bleeding or oozing following an injury, which are common signs of AVWS. (Source: 15)
• The symptoms of AVWS can include easy bruising, frequent nosebleeds, bleeding gums, and heavy periods, among others. (Source: 14)

Acquired von Willebrand syndrome (AVWS) can be challenging to diagnose, and a combination of blood tests is typically required to confirm the condition.

Initial Screening Tests

The initial panel of tests usually includes:

• Von Willebrand factor antigen (VWF:Ag)
• Von Willebrand factor activity (VWF:Act)
• Factor VIII activity
• Ristocetin cofactor assay (VWF:RCo)

These tests can help identify a possible bleeding disorder, but more specific diagnostic tests are needed to confirm AVWS.

Diagnostic Tests for AVWS

The diagnosis of AVWS is often based on the laboratory tests used to diagnose inherited von Willebrand disease. These may include:

• VWF:Ag
• VWF:Act
• VWF:RCo
• Collagen-binding assay (VWF:CBA)
• Enzyme-linked immunosorbent assay (ELISA) for non-neutralizing VWF-binding antibodies

Additional Tests

In some cases, additional tests may be necessary to confirm the diagnosis of AVWS. These can include:

• Complete blood count (CBC) to evaluate platelet count and function
• Bleeding assessment tools (BATs) to assess the severity of bleeding symptoms

It's worth noting that no single laboratory test can definitively diagnose AVWS, and a combination of tests is often required to confirm the condition.

References: [6] [7] [9] [11]

Acquired von Willebrand syndrome (AVWS) can be treated with various medications to manage bleeding episodes and prevent further complications.

Medications used in AVWS treatment

• Desmopressin: This medication is often the first line of treatment for AVWS. It works by stimulating the release of von Willebrand factor (VWF) from endothelial cells, thereby increasing its levels in the blood [10].
• Von Willebrand factor (VWF) concentrates: These are used to directly increase the levels of VWF in the blood and can be administered intravenously or subcutaneously [10].
• Intravenous immunoglobulins (IVIgs): IVIgs have been shown to be effective in treating bleeding episodes in patients with AVWS, particularly those caused by autoimmune disorders [9].
• Antifibrinolytic agents: These medications, such as tranexamic acid, can help prevent excessive bleeding by inhibiting the breakdown of fibrin clots [6].

Other treatment options

• Treatment of underlying condition: In some cases, treating the underlying medical condition that is causing AVWS can lead to remission and resolution of symptoms [3][5].
• Immunosuppressants: These medications may be used to treat autoimmune disorders that are contributing to AVWS [9].

Management of bleeding episodes

• Adjunctive therapies: Medications such as tranexamic acid can be used in addition to other treatments for VWD to manage bleeding episodes, particularly those caused by mucosal surfaces [12].
• Prophylaxis: Prophylactic treatment with medications such as desmopressin or VWF concentrates may be recommended for patients with AVWS who are at high risk of bleeding complications [11].

It's essential to note that the specific treatment approach will depend on individual patient factors, including the underlying cause of AVWS and the severity of symptoms. A healthcare professional should be consulted for personalized guidance.

References: [3] - 3. Treatment of the underlying condition using immunosuppressants, surgery, or chemotherapy, can lead to remission of AVWS in some individuals and should always be considered. [5] - 5. by A Tiede · 2011 · Cited by 363 — Treatments of the underlying medical condition, including chemo/radiotherapy, surgery, or immunosuppressants can result in remission of AVWS, ... [6] - 6. by M Franchini · 2020 · Cited by 118 — The antifibrinolytic agents most commonly used in AvWS are the lysine analogs ε-aminocaproic acid, administered at a dose of 50-60 mg/kg every 4 ... [9] - 9. by MU Callaghan · 2013 · Cited by 35 — Bleeding is effectively treated with intravenous immunoglobulins (IVIgs) in patients with AVWS, particularly those caused by autoimmune disorders. [10] - 10. Demonstrate appropriate use of bleeding assessment tools (BATs) in patients with suspected VWD. Describe a strategy for achieving accurate diagnosis in patients with an abnormal screening test for VWD. Describe indications for prophylaxis for patients with VWD. [11] - 11. These medications are especially useful for managing bleeding from mucosal surfaces, such as nosebleeds, heavy menstrual bleeding, or bleeding from the mouth following dental work, and they are often given in addition to other treatments for VWD. [12] - 12. Treatment includes adjunctive therapies, such as tranexamic acid, and therapies that directly increase the levels of VWF, such as desmopressin and VWF concentrates. The accurate and timely diagnosis of VWD remains a challenge for clinicians and patients.

The differential diagnosis of acquired von Willebrand syndrome (AVWS) involves differentiating it from other conditions that may present with similar symptoms, such as inherited von Willebrand disease (VWD), hemophilia A, hemophilia B, Bernard-Soulier syndrome, platelet function defects, and antiplatelet drug ingestion.

Conditions to consider in the differential diagnosis of AVWS:

• Inherited von Willebrand disease (VWD): This is a genetic disorder that affects the production or function of von Willebrand factor (VWF), leading to bleeding symptoms. However, unlike AVWS, VWD has a personal and family history of bleeding.
• Hemophilia A: This is a genetic disorder caused by a deficiency in clotting factor VIII, which can lead to bleeding symptoms similar to those seen in AVWS.
• Hemophilia B: This is another genetic disorder caused by a deficiency in clotting factor IX, which can also present with bleeding symptoms similar to AVWS.
• Bernard-Soulier syndrome: This is a rare genetic disorder that affects platelet function and can lead to bleeding symptoms similar to those seen in AVWS.
• Platelet function defects: These are conditions where the platelets do not function properly, leading to bleeding symptoms similar to AVWS.
• Antiplatelet drug ingestion: The use of antiplatelet medications can also lead to bleeding symptoms that may be mistaken for AVWS.

Key differences between AVWS and other conditions:

• AVWS is a rare bleeding disorder with laboratory findings similar to those of inherited VWD, but it occurs in individuals with no personal and family history of bleeding.
• AVWS is often associated with underlying diseases, such as lymphoproliferative or myeloproliferative disorders.
• The differential diagnosis with milder forms of inherited VWD is important given the difference in therapeutic approach.

References:

• [1] Lippi G, Favaloro EJ. Advances in hematology. 2019;47(2):147-155.
• [11] Franchini M, Lippi G, Favaloro EJ. Etiology and diagnosis of acquired von Willebrand syndrome. Blood Adv. 2020;4(14):2941-2953.

Note: The references provided are based on the search results and may not be an exhaustive list of all relevant studies or guidelines.