Autoimmune Manifestations in Patients With Waldenström Macroglobulinemia

Agranulocytosis Associated with Waldenström Macroglobulinemia

“…The most common clinical manifestations of WM are anemia (38%), B symptoms (23%), abnormal bleeding (23%), and neurological symptoms (22%). WM is known to cause a variety of autoimmune hematologic disorders, such as autoimmune hemolytic anemia, immune thrombocytopenia, cold agglutinin disease, cryoglobulinemia, and antiphospholipid syndrome [1]. Although neutropenia has never been reported as an immune manifestation of WM, it was still considered a possible etiology in the present case.…”

Multiple cystic lung lesions and autoimmune thrombocytopaenia developing after chemotherapy for pulmonary indolent B-cell lymphoma with plasmacytic differentiation

“…Although the marrow megakaryocytes were not increased as would normally be expected in immune thrombocytopaenia, both the elevated level of PA-IgG and the failure of platelet transfusions to increase the platelet count were consistent with autoimmune thrombocytopaenia. The association between lymphoid neoplasm with monoclonal protein and autoimmune disease may indicate that some monoclonal proteins in lymphoid neoplasms possess antigen-binding activity directed against autologous antigens 6. Among reports of autoimmune thrombocytopaenia in the course of WM, one patient had a confirmed PA IgM, thought to be an IgM monoclonal protein 19.…”

“…That patient had an elevation of PA-IgG without IgG monoclonal gammopathy, suggesting that autoimmune thrombocytopaenia was not directly related to the production of monoclonal IgM but to the presence of PA-IgG antibodies not from a malignant clonal expansion 21. Another hypothesis to explain the relationship between lymphoid neoplasm and autoimmune disease suggests that lymphoid neoplasm involves defective elimination of dysfunctional and autoreactive lymphocytes, allowing those aberrant lymphocytes to produce autoimmune manifestations 6. This hypothesis may apply to the occurrence of autoimmune disease in our patient, namely, the thrombocytopaenia and pulmonary cysts developing even after the lymphoma tumour burden was decreased by chemotherapy.…”

“…Indolent B-cell lymphomas with plasmacytic differentiation frequently pose diagnostic challenges 2. Additionally, these are often associated with various autoimmune diseases 3–6…”

Management of refractory autoimmune hemolytic anemia via allogeneic stem cell transplantation

“…AIHA is found in 10-20% of those with WM. 7 However, in contrast to the severe, refractory hemolysis seen in this patient, the hemolysis observed in association with chronic lymphoproliferative disorders is typically mild and chronic. 8 The case presented here is unique owing to the lack of association between the pace of hemolysis and burden of WM, the lack of an identifiable autoantibody, and the patient's lack of response to multiple lines of therapy used in WM and AIHA.…”