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INTRODUCTION: Hairy-cell leukemia (HCL) is a rare B-cell chronic lymphoproliferative disorder (B-CLPD), whose favorable prognosis has changed with the use of purine nucleoside analogs (PNAs), such as cladribine (CDA) or pentostatin (P). However, some patients eventually relapse and over time HCL becomes resistant to chemotherapy. Many discoveries have been made in the pathophysiology of HCL during the last decade, especially in genomics, with the identification of the BRAFV600E mutation and cellular biology, including the importance of signaling pathways as well as tumor microenvironment. All of these new developments led to targeted treatments, especially BRAF inhibitors (BRAFis), MEK inhibitors (MEKis), Bruton's tyrosine kinase (BTK) inhibitors (BTKis) and recombinant anti-CD22 immunoconjugates. RESULTS: The following major changes or additions were introduced in these updated guidelines: the clinical relevance of the changes in the classification of splenic B-cell lymphomas and leukemias; the increasingly important diagnostic role of BRAFV600E mutation; and the prognostic role of the immunoglobulin (IG) variable (V) heavy chain (H) (IGHV) mutational status and repertory. We also wish to insist on the specific involvement of bones, skin, brain and/or cerebrospinal fluid (CSF) of the disease at diagnosis or during the follow-up, the novel targeted drugs (BRAFi and MEKi) used for HCL treatment, and the increasing role of minimal residual disease (MRD) assessment. CONCLUSION: Here we present recommendations for the diagnosis of HCL, treatment in first line and in relapsed/refractory patients as well as for HCL-like disorders including HCL variant (HCL-V)/splenic B-cell lymphomas/leukemias with prominent nucleoli (SBLPN) and splenic diffuse red pulp lymphoma (SDRPL).
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Zinner syndrome is a rare congenital malformation of the mesonephric duct comprising of seminal vesicle cyst, ipsilateral renal agenesis, and ejaculatory duct obstruction. Clinical presentation varies with perineal pain, painful ejaculation, hematospermia and infertility common presenting complaints. Here, we present a case of Zinner syndrome in a 35-year-old male with a rare clinical presentation of only abdominal discomfort. The purpose of this case report is to highlight the challenging clinical presentation of Zinner syndrome and the use of imaging modalities in diagnosing the condition.
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Gastrointestinal tract perforation (GITP) due to metastatic lung cancer is an exceptionally rare occurrence. Symptoms can range from mild abdominal discomfort to severe and life-threatening bowel perforation. In this case presentation, we describe an unusual instance involving squamous non-small cell lung cancer (NSCLC), where microscopic metastases in the small bowel led to bowel perforation. Our patient, a 71-year-old male with a history of stage IIIa squamous cell carcinoma in the right lung and smoking history, completed chemoradiation therapy and is currently undergoing treatment with durvalumab. He presented to the ED with complaints of abdominal pain, nausea, and abdominal distention. His review of systems revealed no other significant issues, and his vital signs were stable. However, the abdominal examination revealed noticeable distention with tenderness upon palpation and guarding. Laboratory results were significant for leukocytosis with a left shift of neutrophils and mildly elevated kidney function. A CT scan of the abdomen and pelvis revealed widespread pneumoperitoneum, indicating a bowel perforation. Consequently, the patient underwent an urgent exploratory laparotomy, during which a small bowel perforation measuring 0.6 cm x 0.3 cm in the jejunum was identified, necessitating the resection of the affected bowel segment. Intraoperative esophagogastroduodenoscopy (EGD) showed normal findings. The histopathological examination of the resected bowel revealed clusters of squamous cell carcinoma with a desmoplastic reaction, affecting the submucosal and muscular layers at the site of the defect, with surgical margins free of tumor or inflammation. This finding indicated metastatic disease originating from the known lung squamous cell carcinoma. After the operation, the patient was admitted to the ICU due to septic shock caused by E. coli and Klebsiella peritonitis, requiring intubation and circulatory support with pressors. Ultimately, he was discharged following treatment. This case underscores the rarity of symptomatic bowel perforation from micro-metastasis in squamous NSCLC and emphasizes the need for rigorous assessment and timely surgical intervention. However, it is important to recognize the significant risk of complications and a high mortality rate, leading to a challenging prognosis. As such, individuals with a known history of lung carcinoma who present with abdominal symptoms should undergo comprehensive evaluation to prevent life-threatening complications through early intervention.
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BACKGROUND: Scabies is one of the prevalent dermatological conditions, accounting for a substantial proportion of skin diseases in developing countries. It represents a significant health challenge when an outbreak appears in homecare and refugee camps as it may lead to enormous morbidity and high treatment costs. Because Scabies can be easily prevented through education, the purpose of this study was to assess the impact of socio-demographic factors on the level of knowledge, attitude, and practices among Syrian refugees in Jordan's northern region. METHODS: A prospective cross-sectional study was conducted among Syrian refugees attending primary health centers in northern Jordan, Ramtha, Mafraq, and Irbid, from February 2021 to May 2021. The targeted population was adult Syrian refugees above the age of 18. Two thousand participants were included in this study using simple random selection. The study questionnaire included socio-demographic characteristics and knowledge questions such as hearing about Scabies, causes of Scabies, signs and symptoms of Scabies, and its way of transmission. The data was analyzed using (SPSS) version 25. RESULTS: Females with a mean age of 37.9 years old comprised the majority of the participants. The majority of the participants were married and had intermediate levels of education. The knowledge about Scabies lacked among the majority of the respondents (N = 1259); 321 respondents had moderate knowledge, while only 650 had good knowledge. A statistically significant association was found between knowledge scores and all demographic characteristics, including gender, marital status, income, and educational level. CONCLUSION: The general knowledge about Scabies among Syrian refugees is relatively low, with 1259 out of 2000 participants having a bad knowledge score. Moreover, the level of knowledge, attitude, and practices toward Scabies is highly affected by the demographic factors of the Syrian refugees' Health education for refugees is needed to improve their knowledge and help implement prevention programs.
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OBJECTIVE: HAI cause considerable morbidity and mortality and are associated with prolonged hospital stay and increased health care costs. To describe the incidence of HAI in paediatric cancer patients as the first step towards improving infection control policies. METHODS: A prospective surveillance study was performed in the Casablanca university hospital paediatric haematology/oncology unit over an 8-month period from January to August 2011. Data including extrinsic risk factors associated with HAI were recorded. RESULTS: The incidence of HAI was 28 per 1000 patient-days. The median age was 9.6 years and the most frequent diagnosis was acute myeloid leukaemia (32%). Neutropenia at diagnosis was significantly correlated with the risk of HAI. 55.7% of HAls were nosocomial fever of unknown origin. Gram-negative bacteria were the main pathogens (60%), gram-positive cocci were responsible for 26% of HAI and Candida for 14% of HAI. The length of hospital stay for patients with and without infection were 16.5 and 5 days, respectively (P < 0.001). Six of the 11 deaths were related to HAI. CONCLUSION: These findings suggest the need to evaluate infection control measures in order to reduce morbidity and mortality in paediatric haematology/oncology units.
