Acquired von Willebrand Disease

Kumar, Shaji; Pruthi, Rajiv K.; Nichols, William L.

doi:10.4065/77.2.181

Cited by 59 publications

(34 citation statements)

References 96 publications

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“…In previous studies, subjects with blood group O have been reported to exhibit 20-30% lower levels of VWF antigen and activity, compared with subjects with non-O blood groups (34)(35)(36)(37). In line with these data, we found an influence of ABO blood groups on plasma VWF and FVIII levels.…”

Section: Discussionsupporting

confidence: 81%

Evaluation of the respective influence of thyroid hormones and TSH on blood coagulation parameters after total thyroidectomy

Yango¹,

Alexopoulou²,

Eeckhoudt

et al. 2011

European Journal of Endocrinology

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Background: Several hemostatic abnormalities have been described in hypothyroidism, such as modification of coagulation proteins and bleeding tendency. Although thyroid hormone deficiency is considered to be responsible for these changes, the underlying mechanisms have not yet been established. Objective: To evaluate the respective influence of peripheral thyroid hormones (free thyroxine) and TSH on blood clotting by assessing coagulation parameters in patients with a history of total thyroidectomy for thyroid cancer, under three different conditions: induced hypothyroidism, euthyroid state, and following recombinant human TSH (rhTSH) administration. Methods: Coagulation parameters (platelet count, fibrinogen, international normalized ratio, prothrombin time, thrombin time, activated partial thromboplastin time (APTT), factor VIII activity ((FVIII:C), as well as von Willebrand factor antigen (VWF:Ag) and VWF activity using collagen binding assay (VWF:CBA)) were measured in patients with severe hypothyroidism following withdrawal of thyroid hormone replacement therapy, and in the same patients with euthyroidism after restoring replacement treatment (group A), and before and after administering rhTSH (group B). Results: FVIII:C, VWF:Ag, and VWF:CBA were significantly decreased (P!0.001), whereas APTT was significantly increased (P!0.001) in patients with severe hypothyroidism compared with patients in the euthyroid state. No changes in clotting parameters were observed in patients who received rhTSH therapy.Conclusion: This prospective study shows that severe short-term hypothyroidism is associated with significantly lower levels of VWF:Ag, VWF:CBA, and FVIII:C. Administration of exogenous TSH has no effect on coagulation parameters. These findings suggest that thyroid hormone deficiency is likely to be the main cause of coagulation alterations in patients with hypothyroidism.

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Section: Discussionsupporting

confidence: 81%

Evaluation of the respective influence of thyroid hormones and TSH on blood coagulation parameters after total thyroidectomy

Yango¹,

Alexopoulou²,

Eeckhoudt

et al. 2011

European Journal of Endocrinology

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“…In the first mechanism, normally synthesized vWF is promptly eliminated from circulation due to the presence of specific or non-specific autoantibodies. While most autoantibodies against vWF are IgG, antibodies from other classes have also been implicated sporadically [16]. Anti-vWF autoantibodies can interact with active domains of the factor responsible for binding to collagen and platelets, without interfering with factor VIII [17][18][19].…”

Section: Pathogenesismentioning

confidence: 99%

Acquired von Willebrand Syndrome

Mital¹

2016

Adv Clin Exp Med

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“…Administration of high-dose intravenous IgG can produce more sustained responses in patients with acquired von Willebrand syndrome, especially when associated with MGUS or lymphoproliferative disorders. [15][16][17] Case 4: Schnitzler Syndrome. A 37-year-old man presented with progressive aching of the joints, especially of his wrists and ankles, and morning stiffness.…”

Section: Report Of Cases and Discussionmentioning

confidence: 99%

Monoclonal Gammopathy of Undetermined Significance: Significant Beyond Hematology

Vanderschueren

Mylle

Dierickx

et al. 2009

Mayo Clinic Proceedings

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Monoclonal gammopathies are frequently encountered in clinical practice, and most have so-called undetermined significance. Current diagnostic criteria for monoclonal gammopathy of undetermined significance (MGUS), previously known as benign monoclonal gammopathy, include presence of a monoclonal immunoglobulin in serum of 30 g/L or less, 10% or less of plasma cells in bone marrow, and absence of clinical manifestations related to the M protein, such as anemia, hypercalcemia, lytic bone lesions, and renal insufficiency. 1,2 MGUS is found in 3% of persons older than 50 years, in 5% of persons older than 70 years, and in up to 7% of patients seeking medical evaluation. The importance of MGUS lies in its well-known potential to progress to multiple myeloma or a related plasma cell disorder at an average rate of 1% per year. However, even small and nonprogressive B-cell clones can have profound consequences that may be less familiar to clinicians. We present illustrative cases of probable immune-mediated disorders strongly associated with MGUS. These nonmalignant disorders are rare, and with such disorders patients may present to a variety of medical specialists. Presence of the M protein may be a valuable clue to the diagnosis. This case series is intended to increase awareness of these disorders and their association with M proteins. RepoRt of Cases and disCussionCase 1: Acquired C1 Inhibitor Deficiency. A 60-year-old woman was referred for investigation of recurrent abdominal pain of 1 year's duration. Seven months before presentation, urgent laparoscopy performed elsewhere was unremarkable other than revealing the presence of ascites. Two weeks before referral, another laparo scopic procedure was performed during a bout of intense abdominal pain. Again, ascites was evident. Acute abdominal pain recurred, and the patient was transferred to our hospital. MGUS, IgG κ monoclonal protein, had been diagnosed 9 years previously; family history was noncontributory. The patient's abdomen was diffusely tender without rebound tenderness or guarding. Results of all routine laboratory tests, including C-reactive protein levels, were normal. Complement assays showed low levels of CH50, C1q, and C4, whereas C3 levels were normal. C1 inhibitor levels were low, both quantitatively (0.12 g/L; reference range, 0.21-0.39 g/L) and functionally (<26%; reference range, 70%-130%).Acquired C1 inhibitor deficiency was diagnosed. During a subsequent attack of abdominal pain, computed tomography confirmed bowel wall edema. At last follow-up, 14 years after the diagnosis, the patient was doing well. With administration of 200 mg of danazol, up to 4 times a day, the C1 inhibitor level and function normalized. Attacks became less frequent and less severe and could be managed with addition of tranexamic acid. The monoclonal gammopathy was not progressive.Discussion. C1 inhibitor is an inhibitor of the first component of the classic complement pathway and of the kinin-generating pathways. When the C1 inhibitor level is deficient, uncontrolled c...

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Acquired von Willebrand Disease

Cited by 59 publications

References 96 publications

Evaluation of the respective influence of thyroid hormones and TSH on blood coagulation parameters after total thyroidectomy

Evaluation of the respective influence of thyroid hormones and TSH on blood coagulation parameters after total thyroidectomy

Acquired von Willebrand Syndrome

Monoclonal Gammopathy of Undetermined Significance: Significant Beyond Hematology

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