Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia

Alattar, Mona Lisa; Ciccone, Maria; Gaballa, Mahmoud R.; Vitale, Candida; Badoux, Xavier C.; Manoukian, George; Keating, Michael J.; Kroll, Michael H.; Ferrajoli, Alessandra

doi:10.3109/10428194.2015.1037757

Cited by 11 publications

(10 citation statements)

References 9 publications

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“…No T-cell or aggressive B-cell lymphomas were reported, except 2 Richter's transformations of CLL. 44,46 These data highlight the important, but not exclusive, role of IgM monoclonal compounds in the physiopathology of AWS. The rarity of AWS could bias this interpretation.…”

Section: Discussionmentioning

confidence: 83%

“…The immunochemotherapy used in some disseminated indolent lymphomas, such as bendamustine‐rituximab, CVP‐rituximab and CHOP‐rituximab was associated with AWS‐ORRs of 66.7% (4/6) 44–49 , 66.7% (2/3) 50,51 and 75% (3/4), respectively 44,46,52,53 . Older drugs, such as chlorambucil, provided an AWS‐ORR of 50% (2/4), 14,54,55 and more recent drugs, such as, showed an AWS‐ORR of 75% (3/4),47 , 51 including one inefficient combination with rituximab 47 .…”

Section: Resultsmentioning

confidence: 99%

“…The overall response for AWS was from several months to up to two years after the initial improvement of VWF levels and/or clinical bleeding 48,51 . Two articles provided some data on AWS and LRD recurrence 44,51 …”

Section: Resultsmentioning

confidence: 99%

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Acquired von Willebrand syndrome and lymphoid neoplasms: A review of malignancy management, and propositions of practical recommendations

2022

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Introduction Acquired von Willebrand syndrome (AWS) is a rare and potentially life‐threatening bleeding disorder. AWS is primarily associated with lymphocyte‐related disorders (AWS‐LRD), such as lymphoma and IgM monoclonal gammopathy of undetermined significance (MGUS), and plasmocyte‐related disorders (AWS‐PRD), such as non‐IgM MGUS and myeloma. Symptomatic treatments are important to control and prevent bleeding, but AWS‐LRD and AWS‐PRD can only be cured by targeting the responsible clonal cell. No reviews exist on this specific subgroup of AWS. Aim We performed a literature review to help manage these rare cases. Method Thirty‐two AWS‐PRD and 43 AWS‐LRD cases with data on malignancy treatment were reported in 56 articles from the Medline database. Results LRDs were exclusively indolent and primarily associated with IgM monoclonal compounds. LRDs and PRDs may be treated because of severe bleeding symptoms, but severe VWF deficiency did not necessarily correlate with severe bleeding. Immunosuppressive drugs in AWS‐PRD, including rituximab, provided an overall response rate of AWS (AWS‐ORR) of 30% (3/10), including short responses. Anti‐myeloma drugs provided an AWS‐ORR of 71.4% (20/28), with long‐lasting remissions. Bortezomib was the most commonly used drug and provided an AWS‐ORR of 66.7% (6/9), including therapeutic associations with other anti‐myeloma drugs. Autologous and allogeneic stem cell transplantation was performed in eight and two patients, respectively, and some details on the management of AWS during these procedures were provided. Rituximab in AWS‐LRD provided an AWS‐ORR of 60% (3/5), and a chemotherapy + rituximab regimen increased the AWS‐ORR to above 50%. Bleeding syndrome in AWS‐PRD and AWS‐LRD generally improved prior to AWS biological improvement. Conclusion Long term remission of AWS due to lymphoid neoplasms is attainable by treating the underlying clonal cell. Some data and recommendations are provided to help answer difficult questions, including treatment timing, choice of drug, and the timing of evaluations and treatment changes.

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Section: Discussionmentioning

confidence: 83%

Section: Resultsmentioning

confidence: 99%

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Acquired von Willebrand syndrome and lymphoid neoplasms: A review of malignancy management, and propositions of practical recommendations

2022

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“…Acute bleeding episodes can be managed with the administration of human plasma derived von Willebrand factor. Immunosuppression with steroids and rituximab has also been successfully utilized to manage these patients 50 …”

Section: Autoimmune Complicationsmentioning

confidence: 99%

“…Immunosuppression with steroids and rituximab has also been successfully utilized to manage these patients. 50 Another rare cause of unexplained bleeding in patients with CLL has acquired hemophilia A, which is caused by autoantibodies targeting factor VIII. 51 Acute bleeding episodes in these patients should be treated with either factor VIII concentrates or bypass agents, depending on factor VIII inhibitor titers.…”

Section: Acquired Von Willebrand Disease and Acquired Hemophilia Amentioning

confidence: 99%

Unusual complications in the management of chronic lymphocytic leukemia

Gordon

Ferrajoli

2022

American J Hematol

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Chronic lymphocytic leukemia (CLL) is a common, indolent disease that typically presents with a proliferation of mature, immunologically dysfunctional CD5+ B-cells which preferentially occupy the bone marrow, peripheral blood and lymphoid organs.Immune dysfunction leads to an increase in autoimmune diseases which occur in approximately 10% of patients with CLL. Autoimmune cytopenias are the most common, but other organs may be affected as well. The treatment of these conditions typically depends on the extent of CLL and severity of symptoms, but generally consists of CLL-directed therapies, immunosuppression or both. CLL may also infiltrate extranodal sites in the body. Symptomatic extranodal CLL or extranodal disease which threatens normal organ function is an indication for initiation of CLL-directed therapy. The following review summarizes autoimmune and extranodal complications that can occur in patients with CLL and our suggested approach to their treatment.

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Bleeding and Clotting Disorders

Napeñas¹,

Patton²

2021

Burket's Oral Medicine

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Dental health care workers are increasingly called upon to provide quality dental care to individuals whose bleeding and clotting mechanisms have been altered by inherited or acquired diseases. This provides an opportunity for the dentist who is trained in the recognition of oral and systemic signs of altered hemostasis to assist in the diagnosis of the underlying condition. A number of dental procedures result in the risk of bleeding that can have serious consequences, such as severe hemorrhage or possibly death, for the patient with a bleeding disorder. Safe dental care may require consultation with the patient's physician, systemic management, and dental treatment modifications.Of the inherited coagulopathies, von Willebrand's disease (vWD) is the most common. It results from deficiency of von Willebrand's factor (vWF) and affects about 0.8 to 1% of the population. 1 Hemophilia A, caused by coagulation factor (F) VIII deficiency, is the next most common, followed by hemophilia B, a F IX deficiency. The age-adjusted prevalence of hemophilia in six surveillance states in 1994 was 13.4 cases in 100,000 males (10.5 for hemophilia A and 2.9 for hemophilia B). 2 Application to the US population resulted in an estimated national prevalence of 13,320 cases of hemophilia A and 3,640 cases of hemophilia B, with an incidence rate of 1 per 5,032 live male births. Hemophilia A was predominant, accounting for 79% of all hemophiliacs, and prevalence of disease severity was 43% severe (< 1% F VIII), 26% moderate (1-5% F VIII), and 31% with mild (6-30% F VIII) disease. 2 Acquired coagulation disorders can result from drug actions or side effects, or underlying systemic disease. A stratified household sample of 4,163 community residents aged 65 years or older living in a five-county area of North Carolina revealed 51.7% to be taking one or more medications (aspirin, warfarin, dipyridamole, nonsteroidal anti-inflammatory drugs

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Bleeding diathesis associated with acquired von Willebrand Syndrome in three patients with chronic lymphocytic leukemia

Cited by 11 publications

References 9 publications

Acquired von Willebrand syndrome and lymphoid neoplasms: A review of malignancy management, and propositions of practical recommendations

Acquired von Willebrand syndrome and lymphoid neoplasms: A review of malignancy management, and propositions of practical recommendations

Unusual complications in the management of chronic lymphocytic leukemia

Bleeding and Clotting Disorders

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