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Acta Neurologica Belgica Apr 2023Nowadays the problem of comorbidity is still relevant. In this review, we describe clinical cases of the disease of the neuromuscular junction (myasthenia gravis (MG)... (Review)
Review
Nowadays the problem of comorbidity is still relevant. In this review, we describe clinical cases of the disease of the neuromuscular junction (myasthenia gravis (MG) generalized form) and the demyelinating disease of the central nervous system (DD CNS) (multiple sclerosis, neuromyelitis optica spectrum disorder (NMOSD), etc.) combinations registered in our practice with precise pathogenetic analysis. Although the number of the described associations is growing every year, the exact development mechanisms of this cross syndrome as well as the nature of the association between the discussed autoimmune diseases remain unknown. At the beginning of both disorders there is a considerable loss of auto tolerance of the immune system and, as a result, an increased response from autoreactive T-lymphocytes to the structures of the nervous system: brain cells and neuromuscular synapses. There are three main theories for comorbidity: initial predisposition, direct case relationship with disease-modifying therapy (DMT) application, and coincidence. It is known that early diagnostics of MG and timely administration of necessary adequate treatment reduce the risk of process generalization and lead to a decline in mortality. Therefore, the offer to examine MS patients with atypical symptoms for possible MG identification seems very rational. Similarly, MG patients having uncharacteristic symptoms that can be indicative of other autoimmune nervous system diseases also demand special diagnostics. Considering the presence of similar pathogenetic links, several authors propose a possibility of a new nosological unit establishment, including described comorbidity.
Topics: Humans; Myasthenia Gravis; Neuromyelitis Optica; Pigmentation Disorders; Multiple Sclerosis; Central Nervous System
PubMed: 35699899
DOI: 10.1007/s13760-022-01926-z -
Pathology, Research and Practice Apr 2020Granular cell tumor (GCT) remains a diagnostic clinicopathologic problem because the exact frequency of its detailed morphological and clinical characteristics is...
BACKGROUND
Granular cell tumor (GCT) remains a diagnostic clinicopathologic problem because the exact frequency of its detailed morphological and clinical characteristics is unknown as most observations are collected from small series or isolated cases. Herein, our aim is to highlight the frequency of all clinicopathological characteristics of this rare tumor based in our series and the available medical (PubMed) literature.
MATERIAL AND METHODS
42 cases were evaluated for: tissue layers involved by the tumor (in skin and mucosae), growth pattern, nuclear pleomorphism, mitotic index, necrosis, spindling, calcification, hyalinization, and pustule-ovoid bodies of Milian, as well as perineural and vascular invasion, and the presence of adjacent epithelium changes, and lymphocytes and eosinophils infiltration., Follow-up was analyzed. The tumors were subclassified into benign, atypical and malignant according to Fanburg-Smith criteria and into benign or GCT of uncertain malignant potential according to Nasser criteria. The same characteristics were analyzed for 1499 cases reviewed according to PRISMA guidelines.
RESULTS
In the current series, the mean age at diagnosis was 45.8 years (range 6-69 years). Most patients were females (60 %) and the involved organs were by descending frequency: skin and subcutaneous tissue, bronchus, esophagus, breast, tongue, larynx, pharynx, gingiva, trachea, right colon, vulva, and hypopharynx. No recurrence or progression was seen, despite 32 cases were incompletely excised, with the exception of one malignant tumor. The growth pattern was either infiltrative (85.71 %) or well limited (7.14 %). Sixteen tumors had vesicular nuclei. Mitotic activity was found in two tumors. Lymphocytic infiltration was found in 14 tumors. Eosinophils were present in 6 cases. One GCT of the right colon showed extensive calcification and hyalinization. Perineural invasion was noted in 6 lesions. No vascular invasion was found. One tumor was clinically malignant and the patient died 2 years after diagnosis. Medical literature review showed similar results in terms of frequency of the reported clinical and morphological features. Among cases with available follow up, almost 20 % showed positive margins and of those 20 % developed local recurrence. According to the Fanburg-Smith criteria, 72 % would be benign, 17 % atypical and 11 % malignant tumors, while according to those of Nasser, 93 % would be benign and 7% of uncertain malignant potential. However, true malignancy, as affirmed by metastasis of GCT is found in almost 2.5 % of the cases.
CONCLUSION
GCT is a usually benign tumor, affecting any anatomic location. Necrosis and mitotic activity seem to be the most effective histologic criteria for detecting aggressive tumors, but the presence of metastasis (2.5 % of the cases) remains the most accepted definitive criterion for diagnosis of malignant GCT.
Topics: Adolescent; Adult; Aged; Child; Female; Granular Cell Tumor; Humans; Male; Middle Aged; Young Adult
PubMed: 32089415
DOI: 10.1016/j.prp.2020.152865 -
International Journal of Infectious... Jun 2011To compare modes and sources of infection and clinical and biosafety aspects of accidental viral infections in hospital workers and research laboratory staff reported in... (Comparative Study)
Comparative Study Review
OBJECTIVES
To compare modes and sources of infection and clinical and biosafety aspects of accidental viral infections in hospital workers and research laboratory staff reported in scientific articles.
METHODS
PubMed, Google Scholar, ISI Web of Knowledge, Scirus, and Scielo were searched (to December 2008) for reports of accidental viral infections, written in English, Portuguese, Spanish, or German; the authors' personal file of scientific articles and references from the articles retrieved in the initial search were also used. Systematic review was carried out with inclusion criteria of presence of accidental viral infection's cases information, and exclusion criteria of absence of information about the viral etiology, and at least probable mode of infection.
RESULTS
One hundred and forty-one scientific articles were obtained, 66 of which were included in the analysis. For arboviruses, 84% of the laboratory infections had aerosol as the source; for alphaviruses alone, aerosol exposure accounted for 94% of accidental infections. Of laboratory arboviral infections, 15.7% were acquired percutaneously, whereas 41.6% of hospital infections were percutaneous. For airborne viruses, 81% of the infections occurred in laboratories, with hantavirus the leading causative agent. Aerosol inhalation was implicated in 96% of lymphocytic choriomeningitis virus infections, 99% of hantavirus infections, and 50% of coxsackievirus infections, but infective droplet inhalation was the leading mode of infection for severe acute respiratory syndrome coronavirus and the mucocutaneous mode of infection was involved in the case of infection with influenza B. For blood-borne viruses, 92% of infections occurred in hospitals and 93% of these had percutaneous mode of infection, while among laboratory infections 77% were due to infective aerosol inhalation. Among blood-borne virus infections there were six cases of particular note: three cases of acute hepatitis following hepatitis C virus infection with a short period of incubation, one laboratory case of human immunodeficiency virus infection through aerosol inhalation, one case of hepatitis following hepatitis G virus infection, and one case of fulminant hepatitis with hepatitis B virus infection following exposure of the worker's conjunctiva to hepatitis B virus e antigen-negative patient saliva. Of the 12 infections with viruses with preferential mucocutaneous transmission, seven occurred percutaneously, aerosol was implicated as a possible source of infection in two cases, and one atypical infection with Macacine herpesvirus 1 with fatal encephalitis as the outcome occurred through a louse bite. One outbreak of norovirus infection among hospital staff had as its probable mode of infection the ingestion of inocula spread in the environment by fomites.
CONCLUSIONS
The currently accepted and practiced risk analysis of accidental viral infections based on the conventional dynamics of infection of the etiological agents is insufficient to cope with accidental viral infections in laboratories and to a lesser extent in hospitals, where unconventional modes of infection are less frequently present but still have relevant clinical and potential epidemiological consequences. Unconventional modes of infection, atypical clinical development, or extremely severe cases are frequently present together with high viral loads and high virulence of the agents manipulated in laboratories. In hospitals by contrast, the only possible association of atypical cases is with the individual resistance of the worker. Current standard precaution practices are insufficient to prevent most of the unconventional infections in hospitals analyzed in this study; it is recommended that special attention be given to flaviviruses in these settings.
Topics: Accidents, Occupational; Containment of Biohazards; Cross Infection; Health Personnel; Humans; Laboratory Infection; Medical Laboratory Personnel; Occupational Exposure; Research; Risk Assessment; Virus Diseases
PubMed: 21497126
DOI: 10.1016/j.ijid.2011.03.005 -
Infectious Diseases (London, England) Feb 2019Mycobacterium avium complex (MAC) infection is often disseminated and mainly involves lymph nodes, spleen, liver or bone marrow. Peritonitis due to MAC infection (PMAC)...
BACKGROUND
Mycobacterium avium complex (MAC) infection is often disseminated and mainly involves lymph nodes, spleen, liver or bone marrow. Peritonitis due to MAC infection (PMAC) is a very uncommon manifestation.
METHODS
In this report, after describing the case of the only PMAC infection in our 10-year retrospective study, which occurred in an AIDS patient who was non-adherent to highly active antiretroviral therapy (HAART), we performed a systematic literature review of documented bacteriological PMAC.
RESULTS
Including our patient, 51 cases of PMAC have been reported. Patients were most often male (sex ratio 2.14), with a median age of 41 years (2.8-72) and an immunodeficiency in all cases, most often AIDS (57%), cirrhosis (20%) and continuous ambulatory peritoneal dialysis (CAPD) (18%). Ascites was more often chylous (54%) than exudative (46%) and, in this case, lymphocytic (60%), with an inconstantly positive acid-fast bacilli smear (54%). Non-disseminated PMAC patients were more likely to have peritoneal dialysis (39% versus 6.5%, p < .01) or cancer with immunosuppressive therapy (39% versus 0%, p < .0001), while AIDS was the leading underlying disease in disseminated-PMAC patients (83% versus 11%, p < .001). Mortality was high (50%), with no difference between disseminated and non-disseminated PMAC.
CONCLUSIONS
This report highlights the need to be aware of an atypical presentation of PMAC infection, which is associated with a high rate of mortality even for non-disseminated infection.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Humans; Infant; Male; Middle Aged; Mycobacterium avium Complex; Mycobacterium avium-intracellulare Infection; Peritonitis; Retrospective Studies; Young Adult
PubMed: 30318980
DOI: 10.1080/23744235.2018.1519639 -
Acta Haematologica 2014Sweet's syndrome (SS) is an acute febrile neutrophilic dermatosis. It can occur as an idiopathic, drug-induced or malignancy-associated entity. SS is also seen in... (Review)
Review
Initially lymphocytic Sweet's syndrome in male patients with myelodysplasia: a distinguished clinicopathological entity? Case report and systematic review of the literature.
BACKGROUND
Sweet's syndrome (SS) is an acute febrile neutrophilic dermatosis. It can occur as an idiopathic, drug-induced or malignancy-associated entity. SS is also seen in patients with myelodysplastic syndrome (MDS) where it may present atypically, both clinically and histologically. In a few rare cases of MDS, lymphocytic infiltrates are the presenting feature of SS.
METHODS
MEDLINE and Scopus were the data sources for our review.
RESULTS
A clinicopathological subsetemerged of 12 male SS patients with MDS and a mean age of 67.3 years in which the initial SS lesions were lymphocytic infiltrates. However, from 0.5 to 8 years later, sequential biopsies revealed neutrophilic dermal infiltration typical of SS.
CONCLUSION
Initially lymphocytic infiltrates in this subset could be attributed either to an early timing of the biopsy concerning the age of the lesion or to the dysgranulopoiesis syndrome. A possible relationship between the dysfunction of the receptor of the granulocyte-macrophage colony stimulating factor, the gene of which is located on the pseudoautosomal X-Y region, may exist in MDS patients with initially lymphocytic SS. This could explain the male gender of this subset and might establish initially lymphocytic SS as a distinguished clinicopathological entity for predicting the occurrence and even the prognosis of MDS.
Topics: Aged; Anemia, Refractory, with Excess of Blasts; Biopsy; Chromosomes, Human, X; Chromosomes, Human, Y; Disease Progression; Humans; Lymphocytes; Male; Middle Aged; Models, Immunological; Myelodysplastic Syndromes; Neutrophil Infiltration; Receptors, Granulocyte-Macrophage Colony-Stimulating Factor; Sex Factors; Skin; Sweet Syndrome
PubMed: 24714374
DOI: 10.1159/000357933 -
Journal Der Deutschen Dermatologischen... Feb 2019Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (PCSMP-TLPD) is a provisional entity with uncertain malignant potential according to...
Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (PCSMP-TLPD) is a provisional entity with uncertain malignant potential according to the latest revision of the WHO classification for lymphoid neoplasms. We conducted a systematic literature review of all previously reported cases of PCSMP-TLPD to highlight their typical and atypical features. The main features of PCSMP-TLPD and its possible clinicopathologic overlap with similar disorders are also discussed. It is hoped that this review will provide a useful outline of this condition and the most important differential diagnoses. Finally, we recommend a rigorous consensus among cutaneous lymphoma experts in drafting diagnostic criteria and the best case definition.
Topics: Adult; Aged; CD4-Positive T-Lymphocytes; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Lymphoma, T-Cell, Cutaneous; Lymphoproliferative Disorders; Male; Meta-Analysis as Topic; Middle Aged; Prognosis; Skin Diseases; Skin Neoplasms; Ultrasonography, Doppler, Color
PubMed: 30506921
DOI: 10.1111/ddg.13691