Localized gastric diffuse large B-cell lymphoma and Hodgkin's lymphoma as secondary neoplasms in two patients with chronic lymphocytic leukemia.
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Biomedical subjects
Monika Prochorec-Sobieszek
Publications and source records attributed to Monika Prochorec-Sobieszek.
Assessment of the inflammatory process by endomyocardial biopsy in patients with dilated cardiomyopathy based on pathological and immunohistochemical methods.
INTRODUCTION: Myocarditis may lead to dilated cardiomyopathy (DCM) in immunogenetically predisposed individuals. The diagnosis of myocardial inflammation is currently based on histopathological and immunohistochemical methods. Previous studies indicate that inflammatory cardiomyopathy occurs in approximately 50% of patients with DCM. AIM: The goal of the study was to assess the inflammatory process in patients with DCM by endomyocardial biopsy using histopathological and immunohistochemical methods. METHODS: Endomyocardial biopsy specimens was examined using routine histopathological methods and immunochemical staining for T lymphocytes (CD3(+), n=84), major histocompatibility complex I (HLA ABC, n=48) and II (HLA DPQR, n=84) antigens and the adhesion molecules ICAM-1 (n=51) and VCAM-1 (n=48) in 84 patients (69 male, 15 female; mean age 35.0+/-10.5 years) with angiographically-confirmed DCM. Familial disease occurrence was noted in 14 (16.7%) patients. Cardiac samples obtained from 18 patients who died of non-cardiovascular causes were used as a control group. RESULTS: Myocarditis was diagnosed, according to the Dallas criteria, in 8 (9.5%) patients. The frequency of inflammatory cardiomyopathy, defined as the presence of >2 CD3(+) T lymphocytes per high-power field (hpf) in myocardial biopsy, was 14.3%. When broader criteria were applied (presence of >2.0 CD3(+) lymphocytes/hpf and/or 1.5 CD3(+) lymphocytes/hpf in multiple foci and increased expression of class I/II HLA), inflammatory cardiomyopathy was diagnosed in 32.1% of patients. Inflammatory activation of the endothelium, indicated by increased expression of at least three adhesion molecules (class I and II HLA, ICAM-1, VCAM-1), was present in 22 (45.8%) patients. The expression of HLA DPQR, HLA ABC and ICAM-1 was observed on the endothelium of capillaries and larger vessels, interstitial cells, and the surface of activated lymphocytes; immunohistochemical reactions were diffuse. In patients with markedly elevated expression of the aforementioned adhesion molecules, the expression was also present on cardiomyocyte cell membranes. VCAM-1 was restricted to the endothelium of individual small veins. The control group did not demonstrate any signs of myocarditis, inflammatory cardiomyopathy or inflammatory endothelial activation. CONCLUSIONS: The application of immunohistochemical methods to myocardial biopsy in order to identify the inflammatory cell phenotype and the presence of adhesion molecules permits the diagnosis of inflammatory cardiomyopathy in 14% or 32% of patients, depending on the criteria used, while conventional pathology allows for this diagnosis in 9% of patients. The observed frequency of inflammatory cardiomyopathy, defined as the presence of >2 CD3(+) T lymphocytes/hpf in the myocardium, was lower (14%) than in previous studies, while the frequency of inflammatory endothelial activation was similar (45%).
Adult↗
[Clinico-pathological collations in borreliosis].
Borreliosis is an infectious disease caused by spirochetal microorganisms Borrelia burgdorferi transmitted by ticks. Due to versatile clinical symptomatology and many pathogenetic aspects not explained yet, diagnosis of this disease is often very difficult. Borreliosis may affect various human organs and systems such as movement system, nervous system (central and peripheral), skin, heart and vessels. One of the diagnostic methods for detection of microbial organisms in tissues is the histochemical staining by Warthin-Starry. Very important problem connected with this illness is the relationship between borrelial antigens and autoimmunity.
Humans↗
Transformation in lymphomas--morphological, immunophenotypic and molecular features.
During the course of lymphoma, a clinically more aggressive process with different morphology may develop, referred to as lymphoma transformation. Clonal relationship and pathogenic mechanism of this process are widely debated. The aim of the study was to evaluate morphology, immunophenotype (including EBV status) and clonal relationship in nine cases of lymphoma transformation. Among the six patients with low grade B-cell lymphomas three transformed into high grade B-cell lymphomas (two into diffuse large B-cell lymphoma, one into Burkitt lymphoma) and three into Hodgkin lymphoma. Three other patients with Hodgkin lymphoma presented with transformation into diffuse large B-cell lymphoma in two patients and peripheral T-cell lymphoma in one patient. In all cases there was a sudden clinical change as well as change in morphology and phenotype. In five of the nine patients studied EBV-LMP1 was demonstrated by immunohistochemistry in large transformed lymphoma cells. In two cases molecular studies revealed a different pattern of immunoglobulin gene rearrangement in the large transformed cells as compared to the small cells of primary indolent lymphoma. Thus, they represented secondary, arising de novo neoplasm.
Adult↗
Clinical and morphological diagnosis of cardiac AL amyloidosis: a review of findings and difficulties as illustrated in one case.
BACKGROUND: Primary amyloid light-chain (AL) amyloidosis results from plasma cell dyscrasia. Fibrillar deposits of monoclonal immunoglobulin light chains are present in various organs, especially in the heart. The patients suffer from fatal cardiac dysfunction. CASE REPORT: We report the case of a 54-year-old man with symptoms of cardiac arrhythmia which was refractory to conventional treatment and ablations. At a later stage of his disease, the diagnosis of AL amyloidosis associated with monoclonal gammapathy of uncertain significance was established. Echocardiography, electrocardiography, serum immunofixation studies, abdominal fat aspirate biopsy, and bone marrow biopsy indirectly confirmed the diagnosis of cardiac amyloidosis. The patient died. CONCLUSIONS: The diagnosis of AL cardiac amyloidosis is difficult, the prognosis always serious, the available therapy limited, and the mortality high.
Amyloidosis↗
Cardiac amyloidosis diagnosed by endomyocardial biopsy. Clinical, histopathological, immunohistochemical and ultrastructural studies.
BACKGROUND: The heart is often involved by primary (AL) and familial transthyretin-related (ATTR) amyloidosis. Endomyocardial biopsy is a valuable diagnostic method, useful in detection and recognition of the type of amyloid. AIM: The aim of the study was to determine the type of amyloid deposits found in endomyocardial biopsies, using histochemical, immunohistochemical and ultrastructural methods. The correlations between morphological and clinical parameters were evaluated. METHODS AND RESULTS: During 1999-2004, endomyocardial biopsy was performed in 41 patients hospitalized in our institution due to heart failure of unknown etiology. Amyloid deposits were revealed in 3 patients, in 2 of them the intraoperative specimens were also taken. The deposits were located in myocardial interstitium as well as in the wall of small vessels. One patient showed deposits in the atrial and pericardial walls. Electron microscopy study revealed amyloid fibers adhering to the basement membranes of the myocardial cells and small vessels. Immunohistochemical staining determined two types of amyloid. A woman aged 42 showed transthyretin-related amyloidosis, most probably hereditary. A woman aged 69 exhibited the light chains amyloidosis (AL-lambda). In contrast, diagnosis was not certain (most probably ATTR) in a 35 year old woman. Essential involvement of the pericardium was observed in two younger patients. They underwent pericardiotomy. The survival time (from diagnosis to death) was one year in the patient with amyloidosis ATTR, 17 days in the patient with the AL type, and 2 years in the patient with amyloidosis of undetermined type. CONCLUSIONS: Cardiac amyloidosis is associated with poor prognosis. Better recognition of this disease may allow the early diagnosis and institution of modern therapy.
Adult↗
[Lymphoproliferative disorders in Sjögren's syndrome].
INTRODUCTION: Sjögren's syndrome [SS] is an autoimmune disease that mainly affects the exocrine glands. B-cell lymphoproliferation is a characteristic feature of this syndrome and the lesion may range from benign to malignant. MATERIAL AND METHODS: After a systematic search of Pubmed we reviewed literature regarding the histopathology, pathophysiology and clinics of lymphoproliferation in SS. RESULTS: Patients with Sjögren's syndrome [SS] have over 40-fold increased risk of the development B-cell non-Hodgkin's lymphoma. Most cases of lymphomas complicating the course of SS arise in mucosal extranodal sites, especially in the salivary gland, and are classified as low grade marginal zone B-cell lymphoma with long-term survival. The main problem in salivary lymphoproliferation in Sjögren's syndrome consists in the difficulties in the differential diagnosis of lymphoma. Genotypic studies have documented the rearrangement of immunoglobulin genes across the full spectrum of lymphoid infiltrates in the salivary gland including cases regarded as reactive lymphoepithelial sialadenitis [LESA], borderline cases with halos of monocytoid cells surrounding epimyoepithelial islets, and cases with fully developed marginal zone lymphoma [MZL]. Thus, the simple detection of B-cell clonality cannot be used as a criterion for the diagnosis of B-cell malignancy. Broad strands of monocytoid B-cells that surround and invade epimyoepithelial islets and monotypic immunoglobulin expression detected by immunohistochemistry are an essential feature for the histopathological diagnosis of MZL. The pathophysiology of lymphoma in SS remains still unknown. Viral infection, hyperstimulation of B cells, disregulation in the process of apoptosis, and unknown oncogenes are suspected to initiate the start of lymphoma. The main clinical features associated with the development of lymphoma in SS include persistent major salivary gland enlargement (> 2 months), persistent lymphadenopathy or splenomegaly, monoclonal gammapathy and type II mixed cryoglobulinemia. The treatment and prognosis of lymphoma associated with SS depend on the type and stage of lymphoma. CONCLUSION: Patients with SS develop a variety B lymphoproliferative disorders. The nature of these must be determined by multiparameter analysis including clinical, histopathological, immunohistochemical and genotypic studies.
Antigens, CD20↗
Mucosa-associated lymphoid tissue lymphoma (MALT) of salivary glands and scleroderma: a case report.
The connection between scleroderma and lymphoma is uncommon and its pathogenic relationship is a much debated subject. We describe the case of a patient with mucosa-associated lymphoid tissue lymphoma (MALT) of both parotid glands without clinical signs of Sjögren's syndrome who simultaneously developed scleroderma. Independently of the pathogenic mechanism of these two diseases, it seems very important to emphasize that scleroderma may be the first manifestation of lymphoma.
Adult↗
Histopathological and immunohistochemical analysis of lymphoid follicles in labial salivary glands in primary and secondary Sjögren's syndrome.
BACKGROUND: Ectopic lymphoid follicles are considered morphological indices of local chronic antigen stimulation. The objective of this study was to present a detailed histopathological and immunohistochemical analysis of ectopic lymphoid follicles in salivary glands in Sjögren's syndrome (SS), compared to lymphoid follicles in lymph nodes. MATERIAL/METHODS: Samples of labial salivary glands from 37 patients with primary and 57 with secondary SS were examined by histopathology and immunohistochemical methods with monoclonal antibodies (DAKO) to identify follicular dendritic cells, T and B lymphocytes, immunoglobulins and class II MHC HIA DPQR. Lymph nodes from 2 patients with primary SS were also examined for the same markers. RESULTS: Focal inflammatory infiltrations in labial salivary glands in 64.9% of primary and 23.1-49.6% secondary SS were found to morphologically mimic lymphoid follicles in various stages of development. Secondary ectopic lymphoid follicles resemble the histological structure and immunohistochemistry of germinal centers in lymphoid follicles in reactive lymph nodes due to the large number of centrally located B cells (CD20+), reticular pattern of dendritic cells (CD21+) and T helper/inducer cells (CD4+). The centers of secondary ectopic lymphoid follicles contain deposits of IgG and IgM. CONCLUSIONS: Ectopic lymphoid follicles quite often present in labial salivary gland in SS show structural and immunohistochemical features typical for lymphoid tissue. These ectopic follicles are the place of immune complex trapping, antigen presentation on dendritic cells and initiation of autoimmunologic response. This finding seems be very important for better understanding of the pathogenesis and diagnosis of SS as an autoimmunlogical disorder.
Adult↗
[Cardiac amyloidosis--an update].
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Amyloidosis↗
Angiodysplasia as a cause of recurrent bleeding from the small bowel in patients with von Willebrand disease. Report of 4 patients.
Angiodysplasia, characterized by the presence of malformed vessels in the submucosa of the gastrointestinal tract, may be a cause of recurrent bleeding. Bleeding angiodysplasia can be associated with von Willebrand disease (vWD) and this coincidence is probably the consequence of the lack of high molecular weight molecules of von Willebrand factor in the plasma. We report four patients with unexplained repeated massive intestinal bleeding, recurrent melena and iron deficiency anemia, which required numerous blood transfusions. All patients were adults (average age 68 years). Three patients have congenital von Willebrand disease (type 1, 2A and 3) and one idiopathic acquired von Willenbrand syndrome. Correct diagnosis was made 2-5 years after the onset of the symptoms and was confirmed by histopathological examination of surgically resected small bowel, where vascular lesions were located. Elderly patients with recurrent gastrointestinal bleeding and unexplained iron deficiency anemia should be diagnosed for angiodysplasia and vWD.
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