Remission of acquired von Willebrand syndrome after successful treatment of gastric MALT lymphoma

Biondo, Francesca; Matturro, Angela; Santoro, Cristina; Leporace, Anna Paola; D’Elia, Gianna Maria; Baldacci, Erminia; Pignoloni, Patrizia; Pulsoni, Alessandro; Foà, Robin; Mazzucconi, Maria Gabriella

doi:10.1111/j.1365-2516.2011.02681.x

Cited by 6 publications

(10 citation statements)

References 7 publications

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“…Serendipitous remissions of AVWS after rituximab treatment for two cases of low‐grade lymphoma and a single case of Type III cryoglobulinemia have been reported . Furthermore, given the efficacy of rituximab for CLL‐associated autoimmune hemolytic anemia or autoimmune thrombocytopenia, the success in our case is not surprising .…”

Section: Discussionmentioning

confidence: 52%

Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling

Kanakry

Gladstone

2012

Transfusion

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Section: Discussionmentioning

confidence: 52%

Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling

Kanakry

Gladstone

2012

Transfusion

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“…Our patient was diagnosed with a mantle cell lymphoma, a rare subtype of B-cell non-Hodgkin lymphoma. The relationship between AVWS and non-Hodgkin lymphoma has earlier been described in several case reports, mainly in patients with a mucosa-associated lymphoid tissue (MALT) lymphoma [ 3 , 4 ]. To our best knowledge, the association between AVWS and mantle cell lymphoma was never reported previously.…”

Section: Discussionmentioning

confidence: 99%

“…Resolution of AVWS after initiation of therapy has indeed been described in several patients with a lymphoma [ 3 , 4 ]. However, a case of AVWS in a patient with a small lymphocytic lymphoma illustrates that successful management of the underlying condition not always results in remission of the coagulopathy [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma

Maas

Gorkom

Gianotten

et al. 2018

Case Reports in Hematology

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We present a rare case of acquired von Willebrand syndrome (AVWS) caused by a mantle cell lymphoma. A 61-year-old male suffered from recurrent bleeding symptoms since a few months. Initially, physical examination was normal. von Willebrand factor antigen (VWF:Ag) level and factor VIII activity (FVIII:C) were low (0.31 IU/ml and 0.43 IU/ml, resp.). Ristocetin cofactor activity (VWF:RCo) was 0.09 IU/ml, and collagen binding activity (VWF:CB) was 0.10 IU/ml. VWF:RCo/VWF:Ag ratio was 0.29, and RIPA value was normal. Highest molecular weight VWF multimers were absent. A diagnosis of von Willebrand Disease (VWD) type 2A was made. However, no genetic mutation was found. No inhibitory antibodies against VWF or factor VIII were detected. A few months later, cervical, axillary, and inguinal lymphadenopathy was found on physical examination. A CT scan confirmed multiple enlarged lymph nodes, and a clonal B-cell population matching a mantle cell lymphoma was detected in the bone marrow. Chemoimmunotherapy resulted in a very good partial remission and concomitantly in a rapid decrease of bleeding problems and complete normalization of FVIII:C and VWF:Ag. The diagnosis of AVWS cannot be rejected by negative mixing studies due to difficulties in the detection of autoantibodies and because of a highly heterogeneous pathogenesis of AVWS. When the suspicion of AVWS is high, an extensive investigation should be performed to find the underlying cause.

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“…In the present case, although SMZL was quite advanced, the patient refused to receive conventional chemotherapy and instead chose rituximab monotherapy. In the literature, rituximab has not been effective in AVWS associated with MGUS (15-17) or with lymphoplasmacytic lymphoma (18), although it has been effective in AVWS associated with B cell lymphoma (MALT lymphoma) (10,11), monoclonal B cell lymphocytosis (19), essential mixed cryoglobulinemia (20), and SLE (21,22). Although the lymphadenopathy in our case showed mild shrinkage, the AVWS was not resolved.…”

Section: Discussionmentioning

confidence: 99%

Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding

et al. 2017

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An 85-year-old woman underwent emergent splenectomy due to left abdominal pain and active bleeding in a massively enlarged spleen. The histological diagnosis was splenic marginal zone lymphoma (SMZL). A prolonged activated partial thromboplastin time (APTT) was noted, and additional tests led to the diagnosis of type 2A-like acquired von Willebrand syndrome (AVWS). An APTT cross mixing test ruled out the presence of inhibitors. She received eight courses of rituximab monotherapy. The coagulation data showed no improvement, possibly because the lymphoma showed a poor response to the treatment. AVWS rarely causes bleeding in solid organs. This is the first case of SMZL with AVWS diagnosed via splenic bleeding.

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Remission of acquired von Willebrand syndrome after successful treatment of gastric MALT lymphoma

Cited by 6 publications

References 7 publications

Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling

Maintaining hemostasis in acquired von Willebrand syndrome: a review of intravenous immunoglobulin and the importance of rituximab dose scheduling

Acquired von Willebrand Disease Associated with Mantle Cell Lymphoma

Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding

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