Few reports describe the clinical course and acute-care management of patients with recurrent multi-antibody paraneoplastic encephalitis. We describe a rare case of a patient having thymoma with multiple paraneoplastic syndromes who was found to have antibodies to α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) followed by N-methyl-d-aspartate (NMDA) receptor in the setting of residual thymic tissue. He initially presented to the hospital with severe, rapidly progressive encephalitis with simultaneous antibodies to AMPA and voltage-gated potassium channel complex receptor. Brain magnetic resonance imaging revealed scattered white matter hyperintensities and an enhancing lesion adjacent to the left caudate. Computerized tomography showed an anterior mediastinal mass that was resected and revealed to be a thymoma. He was refractory to treatment with intravenous immunoglobulin, high-dose steroids, and plasmapheresis. He was then started on monthly cyclophosphamide. After 3 cyclophosphamide infusions, he began to show improvement in his alertness, ability to speak, and capacity to follow commands. One month later, he was readmitted to the hospital for new and unusual behavioral outbursts and agitation. He was found to have new anti-NMDA receptor antibodies in his cerebrospinal fluid in the setting of residual hyperplastic thymic tissue that required another resection. He was treated with rituximab and then cyclophosphamide (due to an infusion reaction with rituximab) with positive outcomes. The presence of multiple antibodies may be associated with poor prognosis, requiring prompt recognition and aggressive immunosuppressive treatment. New neurological symptoms should prompt a search for residual pathologic tissue or tumor recurrence causing new autoantibodies and additional paraneoplastic syndromes.
Mucormycosis is an invasive, rapidly progressive, life-threatening fungal infection, with a propensity for diabetic, immunosuppressed, and trauma patients. The classic rhinocerebral variation is most common in diabetic patients. While the cutaneous form is usually caused by direct inoculation in immunocompetent patients. Cutaneous mucormycosis manifests in soft tissue and risks involvement of underlying structures. Tibial osteomyelitis can also occur secondary to cutaneous mucormycosis but is rare. Limb salvage is typically successful after lower extremity cutaneous mucormycosis even when the bone is involved. Herein, we report two cases of lower extremity cutaneous mucormycosis in diabetic patients that presented as acute worsening of chronic pretibial ulcers. Despite aggressive antifungal therapy and surgical debridement, both ultimately required amputation. Such aggressive presentation has not been reported in the absence of major penetrating trauma, recent surgery, or burns.
Diffuse large B-cell lymphoma (DLBCL) is characterized by medium- to large-sized neoplastic cells that express a wide range of B-cell markers including CD19, CD20, CD22, and CD79a. Also, as this is a hematopoietic malignancy, there is expression of the leukocyte common antigen CD45. Lack of CD20 expression occurs in a specific rare heterogeneous subgroup of DLBCL including primary effusion lymphoma, plasmablastic lymphoma, ALK-positive large B-cell lymphoma, and large B-cell lymphoma arising in HHV8+ multicentric Castleman disease. In this article, we report a rare case of CD20- and CD45-negative Epstein-Barr virus-positive DLBCL in which the entities listed above were ruled out, thereby posing a significant diagnostic challenge. Arriving at the correct diagnosis of Epstein-Barr virus-positive DLBCL was supported by immunoreactivity for the B-cell transcription factor Oct-2 and the pan-B cell marker CD79a.
Lymphadenopathy in chronic myeloid leukemia (CML) is usually due to extramedullary involvement with accelerated or blast phases of the disease. The occurrence of non-Hodgkin lymphoma (NHL) as a synchronous malignancy with CML is rare. We report a case of a 73-year-old male who presented with dyspnea and right-sided lower extremity edema in the setting of leukocytosis. Bone marrow evaluation indicated a chronic phase chronic myeloid leukemia (CML), confirmed by molecular testing. Imaging of the chest for persistent dyspnea revealed supraclavicular and mediastinal lymphadenopathy. Biopsy of the cervical node showed expanded lymphoid follicles with atypical germinal centers that were positive for CD10, BCL-2, and BCL-6, consistent with follicular lymphoma (FL). Nodal PCR demonstrated clonal IGH and IGK gene rearrangements, and FISH analysis was positive for IGH-BCL-2 fusion. Together, these tests supported the diagnosis of FL. Additionally, the lymph node showed paracortical expansion by maturing pan-hematopoietic elements, no blastic groups, and positive RT-PCR analysis for BCR-ABL1, indicating concomitant involvement by chronic phase-CML. To our knowledge, this is the first reported case of a patient with a concurrent diagnosis of CML and FL.
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