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1.
BMJ Case Rep ; 16(12)2023 Dec 28.
Artigo em Inglês | MEDLINE | ID: mdl-38154873

RESUMO

Composite lymphoma is defined as two or more lymphomas with distinct morphological and immunophenotypical characteristics synchronously diagnosed at the same anatomical site. Composite lymphoma is rare, and the most common combination is follicular lymphoma (FL) associated with diffuse large B cell lymphoma, followed by FL associated with classic Hodgkin's lymphoma (HL). Histologically, composite lymphomas display a mixed pattern or distinct zonal distribution of each lymphoma component. Composite lymphoma poses a diagnostic challenge, especially when two lymphoma components are mixed in the same lymph node. Here, we report a case of composite HL and FL 11 years after initial and repeat biopsies consistent with FL in a man in his 70s emphasising the importance of repeat biopsy in lymphoma diagnosis.


Assuntos
Linfoma Composto , Doença de Hodgkin , Linfoma Folicular , Linfoma Difuso de Grandes Células B , Masculino , Humanos , Linfoma Folicular/diagnóstico , Linfoma Folicular/patologia , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/patologia , Linfoma Composto/diagnóstico , Recidiva Local de Neoplasia , Linfoma Difuso de Grandes Células B/patologia
2.
J Clin Med ; 11(23)2022 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-36498588

RESUMO

POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. Even though the pathophysiology is poorly understood, recent studies suggest that both clonal and polyclonal plasmacytosis leading to the production of pro-inflammatory cytokines and angiogenic mediators play the central role. These mediators including vascular endothelial growth factor (VEGF) are the driving forces of the syndrome. The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. Due to the presence of neuropathy, it can be confused with chronic inflammatory demyelinating polyradiculopathy (CIDP), and if thrombocytosis and splenomegaly are present, it can be confused with myeloproliferative neoplasms. Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. For patients with localized bony disease, radiotherapy has proven to be very effective. Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.

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