scholarly journals Successful treatment with recombinant factor VIIa of therapy-resistant severe bleeding in a patient with acquired von Willebrand disease

2001 ◽  
Vol 66 (4) ◽  
pp. 292-294 ◽  
Author(s):  
Philip W. Friederich ◽  
Peter C. Wever ◽  
Ernest Briët ◽  
Cornelis J. Doorenbos ◽  
Marcel Levi
Keyword(s):  
Successful Treatment ◽  
Recombinant Factor Viia ◽  
Factor Viia ◽  
Von Willebrand Disease ◽  
Severe Bleeding ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand

Concomittant Acquired Von Willebrand Disease and Acquired Factor V Deficiency Causing a Severe Bleeding Diathesis Due To Multiple Myeloma With Amyloidosis

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4788-4788
Author(s):  
Nataliya Melnyk ◽  
Jonathan Harrison
Keyword(s):  
Blood Count ◽  
Case Reports ◽  
Von Willebrand Disease ◽  
Severe Bleeding ◽  
Factor V ◽  
Factor X ◽  
Bleeding Diathesis ◽  
Acquired Von Willebrand Disease ◽  
Factor V Deficiency ◽  
Von Willebrand

Background Acquired coagulopathies are a common problem in Hematology, and are most often due either to medication effect, liver disease, or consumption. Among the uncommon causes of acquired coagulopathy, inhibitory auto-antibodies may develop, either in the setting of autoimmune diseases, in the setting of lymphoproliferative disorders, or as isolated inhibitory immunoglobulins. Uncommonly, the adsorption of coagulation factors from the circulation into the tissues by extracellular deposition of pathologic amyloid results in an acquired factor deficiency, due to clearance of factor from the circulation that exceeds the body's ability to produce factor. When amyoidosis does cause a coagulapathy, it is most often the result of the adsorption of Factor X by the amyloid protein, resulting in an acquired Factor X deficiency. However, there are rare reports of amyloidosis being associated with other factor deficiencies. We report a case of amyloidosis that was associated with a severe bleeding diathesis, with the etiology of the bleeding disorder being due to both acquired Factor V deficiency and concomitant acquired von Willebrand Disease. Case Report A previously healthy 51-year-old gentleman presented to an outside medical center for evaluation and management of recurrent bleeding episodes. The patient had a prior medical history significant only for right ankle trauma in the year 2005, following which he underwent a total of 4 surgical procedures; there was no excessive bleeding complicating the patient's surgeries. He was then in his usual state of health until September, 2012 when he developed onset of severe abdominal pain and was admitted to the outside facility. Following hospitalization for several months at the outside facility, he was admitted to our institution. Physical examination was remarkable for extensive ecchymoses, and for splenomegaly to 18 cm. span by exam, confirmed by imaging. CT scan showed multiple peri-caval and periaortic nodes present up to 1.7 cm in size, with shotty inguinal lymph nodes. A complete blood count showed White blood count 21,600, hemoglobin 8.0 g/dL, hematocrit 24%, platelet count 370,000, Hepatic function studies and renal function studies, as well as electrolytes, were normal on admission. Coagulation studies revealed Prothrombin Time prolonged at 16.8 seconds (normal < 12.7), aPTT prolonged at 44. Mixing patient plasma with equal volume normal plasma corrected both the PT and aPTT. Detailed factor assays showed markedly decreased Factor V activity of 27%; Ristocetin Cofactor activity was markedly decreased at 49%, but von Willebrand antigen was elevated at 213%. Multimer analysis was consistent with Type II vWD (see figure 1). The patient received fresh frozen plasma and Humate P, with transient correction of the bleeding diathesis. This permitted inguinal lymph node biopsy, which documented AL amyloidosis. Extraction of the protein from the lymph node documented AL lambda light chain amyloid (see figure 2). Marrow biopsy documented IgG lambda multiple myeloma. The patient was treated using Bortezumib plus Dexamethasone, and achieved a complete remission, with normalization of the coagulation parameters and factor levels over the following several months. His bleeding diathesis has fully resolved, and Karnofsky performance status improved to 100%. Conclusion Although there are several case reports of acquired von Willebrand disease on the basis of amyloidosis, and several case reports of acquired Factor V deficiency on the basis of amyloidosis, this appears to be the first reported case of both acquired vWD and acquired Factor V deficiency on the basis of amyloidosis. Disclosures: No relevant conflicts of interest to declare.

Successful treatment of severe bleeding with recombinant factor VIIa after kidney transplantation

2004 ◽  
Vol 30 (6) ◽  
pp. 1232-1234 ◽  
Author(s):  
S. E. M. J. Gielen-Wijffels ◽  
W. N. K. A. van Mook ◽  
S. van der Geest ◽  
G. Ramsay
Keyword(s):  
Kidney Transplantation ◽  
Successful Treatment ◽  
Recombinant Factor Viia ◽  
Factor Viia ◽  
Severe Bleeding

scholarly journals Successful treatment using recombinant factor VIIa for severe bleeding post cardiopulmonary bypass

2003 ◽  
Vol 50 (6) ◽  
pp. 599-602 ◽  
Author(s):  
Viren N. Naik ◽  
C. David Mazer ◽  
David A. Latter ◽  
Jerome M. Teitel ◽  
Gregory M. T. Hare
Keyword(s):  
Cardiopulmonary Bypass ◽  
Successful Treatment ◽  
Recombinant Factor Viia ◽  
Factor Viia ◽  
Severe Bleeding ◽  
Post Cardiopulmonary Bypass

Characterization of a myeloma patient with a life-threatening hemorrhagic diathesis: presence of a lambda dimer protein inhibiting shear-induced platelet aggregation by binding to the A1 domain of vonWillebrand factor

2005 ◽  
Vol 93 (05) ◽  
pp. 889-896 ◽  
Author(s):  
Atsushi Shinagawa ◽  
Michael Berndt ◽  
Shin Kaneko ◽  
Kazumi Suzukawa ◽  
Yuichi Hasegawa ◽  
...  
Keyword(s):  
Platelet Aggregation ◽  
Von Willebrand Disease ◽  
Platelet Glycoprotein ◽  
Severe Bleeding ◽  
Hemorrhagic Diathesis ◽  
Bleeding Tendency ◽  
High Shear ◽  
Acquired Von Willebrand Disease ◽  
A1 Domain ◽  
Von Willebrand

SummaryWe have identified a patient with IgD λ-type multiple myeloma who was characterized by a severe bleeding tendency, especially after puncture of arterial vessels. Both the bleeding time (>25 min) and activated partial thromboplastin time (APTT) were prolonged. To clarify the underlying pathogenesis, we purified the APTT-prolonging activity from the patient’s serum. The purified protein was a highly negatively-charged homodimer of the λ light chain. The λ dimer protein (M-protein) inhibited ristocetin-and high shear-induced platelet aggregation, dependent on platelet glycoprotein Ibα (GPIbα), but not epinephrine-, collagen-, ADP-, thrombin-, or botrocetin-induced platelet aggregation. The λ dimer protein inhibited the binding of platelets to immobilized or ristocetin-treated von Willebrand factor (VWF). Furthermore, a 39/34 kD fragment of VWF encompassing the A1 domain specifically bound to the immobilized λ dimer protein in the presence of ristocetin, suggesting that the λ dimer protein directly binds to the A1 domain of VWF. To help elucidate the binding site within the A1 domain, binding of ristocetin-treated VWF to the immobilized λ dimer protein was assayed in the presence of various anti-A1 domain monoclonal antibodies. Based on these data, we conclude that the λ dimer protein binds to the region of the A1 domain composed of helices α3 and α4 and thus interferes with VWF-GPIbα interaction. The existence of a protein that inhibits high shear-induced platelet aggregation in acquired von Willebrand disease (VWD) has only rarely been reported. The results suggest that the hemostatic function in arteries with high shear force is profoundly disrupted if the binding of GPIbα to VWF is abrogated, supporting the relevance of shear-induced VWF interaction with GPIbα in the initiation of the hemostatic process.

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