Hematologic malignancies and hematopoietic stem cell transplantation.

Paraneoplastic neurologic syndromes are rarely associated with hematologic malignancies. In their rarity, lymphomas are the diseases with more frequent paraneoplastic neurologic syndrome. High-risk antibodies are absent in most lymphoma-associated paraneoplastic neurologic syndromes, with the exception of antibodies to Tr/DNER in paraneoplastic cerebellar degeneration, mGluR5 in limbic encephalitis, and mGluR1 in some cerebellar ataxias. Peripheral nervous system paraneoplastic neurologic syndromes are rare and heterogeneous, with a prevalence of demyelinating polyradiculoneuropathy in non-Hodgkin lymphoma. Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) is a rare, paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis is based on defined criteria, and vascular endothelial growth factor (VEGF), not an antibody, is considered a reliable diagnostic marker that also mirrors therapy response. As with the paraneoplastic neurologic syndromes in solid tumors, therapies rely on cancer treatment associated with immunomodulatory treatment with better response in PNS with antibodies to surface antigens. The best outcome is generally present in Ophelia syndrome/limbic encephalitis with anti-mGluR5 antibodies, with frequent complete recovery. Besides patients with isolated osteosclerotic lesions (where radiotherapy is indicated), hematopoietic stem-cell transplantation is the therapy of choice in patients with POEMS syndrome. In the paraneoplastic neurologic syndromes secondary to immune checkpoint inhibitors, discontinuation of the drug together with immunomodulatory treatment is recommended.

Eritema multiforme como manifestación paraneoplásica de un adenocarcinoma de colon / Erythema multiform as a paraneoplastic manifestation of a colon adenocarcinoma

RESUMEN: El eritema multiforme es una enfermedad autoinmune vesicular-ampollosa y autolimitada, se presenta de forma aguda afectando la piel y mucosa. En 2015, Lee E. y Freer J., incluyeron esta enfermedad dentro de los síndromes dermatológicos paraneoplásicos, pero actualmente no existen muchas publicaciones de esta entidad como manifestación a una neoplasia subyacente. El objetivo de esta investigación es presentar el eritema multiforme como una manifestación paraneoplásica inusual. En este caso una paciente de 64 años de edad que cursaba con adenocarcinoma de colon exhibió el eritema multiforme oral; cuyas manifestaciones bucales involucionaron gracias a la farmacoterapia local y sistémica en conjunto con la excéresis de la lesión cancerosa en el colon. Es importante reconocer la presencia de eritema multiforme como una manifestación paraneoplásica cuando no se exhiben los factores etiológicos frecuentes de esta patología y, por lo tanto, centrarse en la búsqueda subyacente de neoplasias malignas que no son muy evidentes.

ABSTRACT: Erythema multiforme is a self-limiting, blistering, autoimmune disease that presents acutely, affecting the skin and mucosa. In 2015, Lee E. and Freer J. included this disease in paraneoplastic dermatological syndromes, but there are currently not many publications about this entity as a manifestation of an underlying malignancy. The objective of this research is to present the erythema multiforme as an unusual paraneoplastic manifestation. In this case, a female 64-year-old patient with colon adenocarcinoma exhibited oral multiform erythema; whose oral manifestations regressed thanks to local and systemic pharmacotherapy in conjunction with the exeresis of the cancerous lesion in the colon. It is important to recognize the presence of erythema multiforme as a paraneoplastic manifestation when the frequent etiological factors of this pathology are not exhibited and, therefore, to focus on the underlying search for malignant neoplasms that are not very evident.

[Case of multiple systemic (extrahepatic) manifestations of chronic HCV infection (analysis of the literature and own observations). Case report].

The article presents a description of a patient with chronic HCV infection and multiple extrahepatic manifestations, which manifested in dynamics and were recorded with a different sequence during 15 years of follow-up. In the patient we observed, the most frequently recorded extrahepatic manifestations were verified: porphyria cutanea tarda, mixed cryoglobulenemia, and utoimmune thyroiditis. Chronic HCV infection is often diagnosed in the presence of psoriasis was assessed as a paraneoplastic disease.

Tumour TIF1 mutations and loss of heterozygosity related to cancer-associated myositis.

Objectives: To analyse the influence of genetic alterations and differential expression of transcription intermediary factor 1 (TIF1) genes in the pathophysiology of cancer-associated myositis (CAM). Methods: Paired blood and tumour DNA samples from patients with anti-TIF1γ-positive CAM and from controls were analysed by whole-exome sequencing for the presence of somatic mutations and loss of heterozygosity (LOH) in their TIF1 genes. The genesis and maintenance of the autoimmune process were investigated immunohistochemically by studying TIF1γ expression in the different tissues involved in CAM (skin, muscle and tumour) based on the immunohistochemical H-score. Results: From seven patients with anti-TIF1γ-positive CAM, we detected one somatic mutation and five cases of LOH in one or more of the four TIF1 genes compared with just one case of LOH in tumours from TIF1γ-negative myositis patients (86% vs 17%; P = 0.03). Compared with type-matched control tumours from non-myositis patients, TIF1γ staining was more intense in tumours from anti-TIF1γ-positive patients (H-score 255 vs 196; P = 0.01). Also, TIF1γ staining in muscle was slightly more intense in anti-TIF1γ-positive than in anti-TIF1γ-negative myositis (H-score 22 vs 5; P = 0.03). In contrast, intense TIF1γ staining was detected in the skin of both myositis and control patients. Conclusion: Tumours from paraneoplastic anti-TIF1γ-positive patients showed an increased number of genetic alterations, such as mutations and LOH, in TIF1 genes. These genetic alterations, in the context of a high expression of TIF1γ in the tumour, muscle and skin of these patients may be key to understanding the genesis of paraneoplastic myositis.

Sweet´s syndrome and pancreatic neoplasm: an atypical association

El síndrome de Sweet es una dermatosis inflamatoria poco común, que se ha asociado a tumores malignos, principalmente de tipo hematológico. Presentamos un caso clínico de síndrome de Sweet asociado con una rara neoplasia pancreática, siendo uno de los pocos casos reportados en la literatura médica acerca de esta asociación.

Sweet's syndrome is an uncommon inflammatory dermatosis, which has been associated with malignant tumors, mainly of hematological type. We report a clinical case of Sweet syndrome associated with a rare pancreatic neoplasm, which is one of the few cases reported in the medical literature about this association.