Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding
نویسندگان
چکیده
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.
منابع مشابه
CD5 expression identifies a subset of splenic marginal zone lymphomas with higher lymphocytosis: a clinico-pathological, cytogenetic and molecular study of 24 cases.
BACKGROUND Classically, splenic marginal zone B-cell lymphoma is characterized by the absence of CD5 expression. Cases of apparent splenic marginal zone B-cell lymphoma showing CD5 expression, as diagnosed by blood studies, have been described; however, in the absence of histological evidence, the correct diagnosis of these cases is controversial because of possible confusion with other CD5-pos...
متن کاملSystemic Mastocytosis Associated with Splenic Marginal Zone Lymphoma with Villous Lymphocytes
The development of a second haematological disease during the course of systemic mastocytosis is a well-known phenomenon. In most of the cases, they consist of myelodysplasia or myeloproliferative disorders. The association with lymphoproliferative disorders has also been described, but it is uncommon and the relationship is not well established. We report a patient diagnosed with systemic mast...
متن کاملCoexistence of splenic marginal zone lymphoma with hepatocellular carcinoma: a case report
BACKGROUND Coexistence of splenic marginal zone lymphoma with hepatocellular carcinoma is rare. Although some reports have suggested the possible pathogenic role of HBV, HCV, chronic and persistent antigenic stimulation in lymphoma, their role in causing lymphomas is still unclear. CASE PRESENTATION We describe a hepatocellular carcinoma with concomitant splenic marginal zone lymphoma in a 64...
متن کاملSpontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema.
A 52 year old woman with marginal zone lymphoma developed recurrent episodes of angio-oedema and was found to have C1 esterase inhibitor deficiency. She declined chemotherapy for the lymphoma. Fourteen months after her initial presentation she was found to be in partial remission, and this was confirmed by peripheral blood film and bone marrow examinations. This was associated with normalisatio...
متن کامل