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Digestive cancers are the most severe complications of celiac disease (CD), which can lead to death. We can report 12 patients with CD associated with cancer. It is retrospective study of 12 patients with CD complicated by cancers, collected in the department of diseases of the digestive system "Medicine C", from 1995 to 2017. Six patients had lymphoma, three were gastric tumors, two were small intestine tumors, and one had liver tumor. The diagnosis of lymphoma was made at the same time as that of CD in one patient, three months later in two patients, 18 months later in two patients and 8 years later in one patient. Morphological and histological data were in favor of malignant non-Hodgkin's lymphoma with intestinal localization in four patients, with lymph node localization in one patient and one patient with refractory celiac disease type 2 with intraepithelial lymphoma. The treatment consisted of chemotherapy in four patients and corticosteroid therapy with parenteral nutrition in one patient. The evolution was marked by the death of 03 patients and 2 patients were lost without any idea about their evolution. The diagnosis of the gastric tumor was retained after the realization of a upper gastrointestinal endoscopy with biopsies. The histopathologic examination was in favor of a well-differentiated adenocarcinoma in two patients, and of an adenocarcinoma moderately differentiated in one patient. The diagnosis of the well-differentiated neuroendocrine tumor was carried out 30 years after that of CD. The patient underwent a resection of the first two duodenal portions with duodeno-jejunal anastomosis and a colonic resection with termino-terminal anastomosis. The evolution was good with a 2 years follow-up. The second patient with intestinal cancer was a moderately differentiated adenocarcinoma of intestinal origin; the diagnosis was retained after an abdominal CT scan with mass biopsy. The treatment was palliative chemotherapy, with an abdominal scan three months later showed partial response. The hepatocellular carcinoma on healthy liver was diagnosed at the same time as CD on angio-CT scan data and liver biopsy with patient death at the time of diagnosis. Digestive cancers are severe complications of CD requiring early management and follow-up of patients to reduce the rate of death.

MeSH
celiakie * komplikace MeSH
diagnostické techniky gastrointestinální MeSH
gastrointestinální nádory etiologie komplikace MeSH
lidé MeSH
lymfom z T-buněk asociovaný s enteropatií etiologie MeSH
nehodgkinský lymfom etiologie MeSH
Check Tag
lidé MeSH

Článek

Primary intestinal malignant lymphomas associated with Celiakia – A pathologist´s review

Plank, Lukáš, 1951-
Autor Autorita Department of Pathology, Comenius University in Bratislava Jessenius Medical Faculty and University Hospital in Martin and Martin´s Biopsy Center, Ltd., Slovakia

International journal of celiac disease. 2015 ; 3 (2) : 59-68.

ISSN 2334-3427
Medvik
Zdroj

Celiakia represents a prototype of chronic antigenic stimulation and autoimmune dysregulation leading to the accumulation of reactive cytotoxic intraepithelial lymphocytes (IEL) and small intestinal mucosal injury. These lymphocytes possess a potential of transformation, gaining an aberrant phenotype and genotype during their clonal expansion. The development of type I enteropathy –associated T-cell lymphoma (EATL) in patients with celiac disease might represent a gradual multistep transformation process starting from the increasing accumulation of IEL, and developing through the refractory celiac disease I and refractory celiac disease II, the last representing probably a lymphoma in situ. These transformational stages of the progressing celiac disease are showing increasing risk of type I EATL development, and decreasing survival of the patients. The whole process of progressing EATL lymphomagenesis resembles the hyperplasia-adenoma-dysplasia-adenocarcinoma sequence of colorectal carcinomas. Similar to the already more understood multistep developmental process from CD to type I EATL, an overt type II EATL lymphoma might represent a final step in a process of progression from IEL, representing the lymphoma precursors. However, the patients with the type II lymphoma show signs of celiakia neither clinically nor morphologically and the pathogenetical link to CD is missing. The lymphomagenesis of EATL type II seems to utilize activation of other pathways than that of type I. The recent data support the attempts to set apart the type II from the EATL category and to rename it.

MeSH
celiakie komplikace patologie MeSH
lidé MeSH
lymfom z T-buněk asociovaný s enteropatií * klasifikace patofyziologie patologie MeSH
počet CD4 lymfocytů MeSH
prognóza MeSH
Check Tag
lidé MeSH
Publikační typ
práce podpořená grantem MeSH

Článek

Lymfomy gastrointestinálního traktu – klinicko-patologický přehled
[Lymphomas of gastrointestinal tract – clinico-pathological review]

Transfuze a hematologie dnes. 2013 ; 19 (3) : 140-151.

ISSN 1213-5763
Medvik
Zdroj

Primárně extranodální lymfomy (tzn. lymfomy vyrůstající z mimouzlinových orgánů) tvoří asi 30 % všech nehodgkinských lymfomů (NHL), z nich asi 40 % je diagnostikováno právě v gastrointestinálním traktu (GIT). Primární lymfomy gastrointestinálního traktu tedy tvoří asi 5–10 % ze všech NHL. Jejich výskyt oproti karcinomům je sice výrazně nižší, nicméně lymfom je druhý nejčastější nádor žaludku a střeva. Místem postižení bývá zejména žaludek (v cca 60–75 % případů) a tenké střevo, v ileu, céku a rektu se lymfom objevuje méně. Pokud jde o typy lymfomů, nejčastěji bývá diagnostikován difuzní velkobuněčný B-lymfom (DLBCL) a MALT lymfom (MALT = mucosa associated lymphatic tissue), následován ostatními zralými B a T lymfoproliferacemi. Některé typy NHL primárně se vyskytujících v GIT bývají asociovány s chronickou zánětlivou reakcí v důsledku infekčních agens (Helicobacter pylori, Campylobacter jejuni, EBV), nebo glutenové enteropatie, jako je tomu v případě T-lymfomu sdruženého s enteropatií. Zatímco primární či sekundární postižení GIT u NHL je časté, Hodgkinův lymfom se v této oblasti nachází raritně. Důležitým nástrojem v diagnostice a stanovení rozsahu postižení (stagingu) primárních lymfomů horního GIT je endoskopická ultrasonografie. Strategie léčby závisí na typu lymfomu, rozsahu choroby a etiologii. V případě lokalizovaného MALT lymfomu sdruženého s infekcí Helicobacterem pylori je indikována antibiotická eradikace, která může navodit remisi. Radioterapie je obecně vhodná pro lokalizovaná stadia lymfomů, zejména tam, kde nelze použít antibiotika nebo po selhání antibiotické terapie. V případě pokročilých nebo agresivních lymfomů je indikována imunoterapie, respektive imunochemoterapie. Chirurgická léčba se uplatňuje dominantně v rámci diagnostiky, její místo v léčbě lymfomů GIT je velmi specifické a limitované.

Primary extranodal lymphomas (lymphomas arising from extranodal organs) comprise approximately 30% of all of lymphomas, from which about 40% is diagnosed in gastrointestinal tract (GIT). Primary gastrointestinal lymphomas form nearly 5–10% of all non-Hodgkin's lymphomas. Their frequency is much lower than carcinomas; nevertheless lymphoma is second most frequent tumour of stomach and gut. The most common localization of lymphoma manifestation is stomach (about 60–75% cases) and small bowel; ileum, caecum and rectum are involved less often. Regarding of type, diffuse large B-cell lymphoma (DLBCL) and Mucosa associated lymphoid tissue lymphoma (MALT) are diagnosed most frequently, followed by other mature B- and T-lymphoproliferative diseases. Some of primary GIT lymphomas are associated with a chronic inflammation, in particular of infection (Helicobacter pylori, Campylobacter jejuni, EBV), or gluten enteropathy, how it is in the case of enteropathy associated T-lymphoma. Whereas non-Hodgkin's lymphomas are common in GIT, the impairment of Hodgkin's disease in this localization is rare. The important tool for diagnosis, staging and follow up of upper GIT lymphoma, especially in stomach, is endoscopic ultrasonography. Treatment strategy depends on type of lymphoma, stage and etiology. In principle, localized stages of MALT lymphoma associated with H. pylori infection should be treated by antibiotics with Helicobacter eradication, which can lead to remission. Radiotherapy should be used in localized cases not suitable for antibiotics or with antibiotics failure. Immunotherapy or immunochemotherapy is indicated for the treatment of aggressive or more advanced lymphomas. Surgery is useful in diagnostics but in the treatment of GIT lymphoma has very limited place. .