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Volumen 14, Asunto 3 (2023)

Series de casos

Clinical Features and Management of Langerhans Cell Histiocytosis: A Case Series

Tayssir Ben Achour1*, Maysam Jridi, Ines Naceur, Monia Smiti, Fatma Said, Mohamed Habib Houman and Eya Azouz

Background: Langerhans histiocytosis (LCH) is a rare hematopoietic disease characterized by clonal expansion of myeloid precursors differentiating into CD1a+/CD207+ histiocytes infiltrating different organs. The study aimed to report the clinical features such as the onset presentation and systemic lesions of LCH among Tunisian adult patients and the therapeutic management of the disease with onboard medications.

Methods: A descriptive, retrospective and monocentric study taking place in the internal medicine department of Rabta University Centre.

Results: The study included eight patients. The median age at the diagnosis was 35 years. The mean duration between the first symptoms and the diagnosis was 17 months. The first most consulted doctors were neurologists. The revealing symptoms were neurological in five cases including headaches, paraplegia or walking trouble. Bone pain was the first symptom in three cases and in one case cervical adenopathy was the first manifestation. Bone involvement was the most frequently found (N=6). Four patients had central nervous system lesions. PLCH was observed in four cases and only one patient was a smoker. Two patients had lymphadenopathy. Retroperitoneal fibrosis and abdominal aortitis were found in one case. Skin involvement was found in one case and was associated with a multisystem LCH form. We observed one case of orbital involvement with optical canal infiltration. In all cases, the diagnosis of LCH was established based on histological features. Seven patients received a treatment with prednisone. Vinblastine was used in six cases.

Conclusion: Langerhans cell histiocytosis is a rare disease with different facets and heterogenous initial presentation. Patients with LCH would usually see different doctors and would have misleading signs with a variety of differential diagnoses before recognizing LCH.

Mini reseña

Contrast-Enhanced Imaging Characteristics of Steatohepatitic Hepatocellular Carcinoma and Clinicopathological Investigation

Xuhao Song*

With 906,000 newly diagnosed cases worldwide in 2020, hepatocellular carcinoma is the sixth most common malignant tumour and the third leading cause of cancer-related deaths. The prevalence of HCC varies by ethnicity, with the majority of patients suffering from chronic infections of the hepatitis B and C viruses, metabolic-dysfunction-associated fatty liver disease, or alcoholic steatohepatitis. MAFLD, formerly known as nonalcoholic fatty liver disease, is characterised histologically by hepatocyte steatosis, steatohepatitis, Mallory's body, and fibrosis. MAFLD currently affects 30% of the global population, despite the fact that only 5% of patients will progress to MAFLD-related fibrosis or HCC. Although chronic HBV infection remains a major cause of HCC in China, MAFLD-related primary liver cancer has increased.

Mini reseña

Subsets of Regulatory T Cells in Sarcoidosis Patients

Piotr Yablonskiy*

Many researchers have recently backed the autoimmune theory of sarcoidosis. The presence of an uncontrolled inflammatory response at the local and systemic levels in sarcoidosis patients did not rule out the possibility that immunoregulatory mechanisms were compromised. The purpose of this study was to assess the distribution and disturbance of circulating Treg cell subsets in the peripheral blood of sarcoidosis patients. Materials and procedures: In 2016-2018, a prospective comparative study was conducted. The diagnosis of pulmonary sarcoidosis was made using standard criteria. For Treg immunophenotyping, we used two ten-color antibody combinations.

Artículo de investigación

Morphological Study of Teeth and Periodontal Structures after Extraction in Syrian Hamster

Ahmad Mohammad Khattab* and Hikmat Yakub

Background: Syrian Hamsters are considered a prime animal model to conduct histological examination in the oral cavity, having an exceptional ability to constantly regenerate their incisors. Syrian hamsters were also commonly used in experimentally induced cancerous growth studies.

Methods: 28 Syrian Hamsters have been distributed into six main groups in addition to control group. The animals of the control group have been sacrificed to get the normal morphological structure of the teeth and the periodontal structures. While the control group of animals have been sacrificed gradually, in order to study the stages of the restorative process, after the days that followed the extraction process.

Results: This research illustrates the morphology of the teeth in Syrian hamsters, mainly describing the depression area in the medial part of the root. It confirmed the presence of periodontal ligament tissue around these teeth. This Study confirmed in detail and with microscopic images the phases of the restorative process from the beginning of forming and organizing the thrombus till its Differentiation into the different dental and periodontal tissues.

Conclusions: The most significant achievement of this research its ability to detect focal regions of active cells which are able to differentiate into animal, dentin, and other different tissues.

Artículo de investigación

Histoanalysis for Epithelial and Mezanchyme Stem Cells Responsible for Continuous Regeneration of Incisors in Syrian Hamsters, an Experimental Study to Monitor Reproductive and Differentiation Properties after Extraction

Ahmad Mohammad Khattab* and Hikmat Yakub

Background: Syrian Hamsters are considered prime animal models used in numerous experimental research and applications especially in the field of dentistry. Studies on Syrian hamsters have shown their exceptional regenerative abilities of incisors after extraction. Isolating and identifying cells responsible for the constant regeneration of the incisors, determining their unique properties, and confirming their ability to differentiate into ectoderm and mesoderm derived cells. Exploring their capabilities to construct cellular cultures in sufficient numbers and identifying the proper cellular markers is an extremely important issue.

Methods: 15 Syrian Hamsters were distributed evenly into three Main groups plus a control group. Control group was sacrificed in order to obtain a sound Morphological structure in the incisor region. Specimens in Main groups were gradually sacrificed to monitor the progress of the regeneration process within the next days following extraction on the first, fifth, and tenth day respectively.

Results: Results confirmed the presence of lymphoblastic lengthening encased inside the bone cavity consisting of active homogenous cell cultures. These cells can proliferate and differentiate forming the pulp, dentin, cementum, and periodontal ligaments. This was confirmed with immunohistochemical markers proving these cells are in fact adult stem cells in the periodontal tissues.

Conclusion: Regeneration process within the bone cavity happened fairly quickly in hamsters, incisor regeneration after extraction took less than one week, which provides adequate conditions to monitor the regenerative process within the cavity after extracting incisors in hamsters.

Reporte de un caso

Diagnosis of Scar Endometriosis on FNAC: A Case Report of Two Cases

Rani Sahu

The presence of functioning endometrial glands and stroma outside the uterus is called endometriosis. Scar endometriosis is a rare occurrence and seen in 0.03%-1.08% of women following gynaecologic or obstetric surgeries. Many of abdominal scar endometriosis is associated with previous caesarean sections. We report two cases of abdominal scar endometriosis in reproductive age female patients with a previous history of obstetric and gynaecologic procedures. Both the cases were diagnosed on FNAC and followed with excision biopsy and histopathology correlation.

Reporte de un caso

Metastatic Carcinoma ex Pleomorphic Adenoma in Pleural Effusion

Hsiao-Ching Chen1*

Carcinoma ex pleomorphic adenoma (CXPA) arising from primary or recurrent pleomorphic adenoma is characterized by the presence of longstanding palpable mass with growth spurt mainly in the parotid region. Overall, patients with CXPA have a poor prognosis. Pleural effusion involved by CXPA is precedingly rare, compared to that common dissemination from pulmonary or mammary origin. Here we reported a metastatic CXPA in pleural effusion from an 82-year-old male. The clinical presentation, cytological morphology, and immunohistochemistry confirmed the diagnosis. When dealing patients with prolonged history of pleomorphic adenoma, underwent recent rapid growth of malignant transformation and malignant cells presented in the pleural effusion, metastatic CXPA should be seriously considered.

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