Mediterranean Journal of Hematology and Infectious Diseases最新文献

Background: Rickettsioses are considered emerging or re-emerging hematophagous arthropod-borne zoonosis. In addition, Meningeal syndromes are among the most common reasons for consultation in infectious disease emergencies.

Objectives: Our study aimed to identify Rickettsia spp using IFA and qPCR for serological and molecular tests, respectively, in patients presenting with Meningeal Syndrome at the National Centre of Infectious Diseases El-HADI FLICI Hospital, Nicolle-Laveran department in Algiers.

Methods: We collected 55 whole blood and 55 sera from patients with meningeal syndrome of various ages and genders, with a mean age of 24.03 (ranging from 2 to 50 years old).

Results: The indirect immunofluorescence assay (IFA) for Rickettsia spp returned positive results in seven sera (7/55, 12.72%). We diagnosed four cases of Spotted Mediterranean Fever (MSF) caused by R. conorii, two cases of Murine Typhus caused by R. typhi, and one case of Flea-borne Spotted Fever caused by R. felis. Concerning the spinal tap, we reported that three patients were positive for R. conorii, where cerebrospinal fluid was opalescent, and there was a mixed cellular profile on cytology. These findings suggest a possible co-infection with R. conorii in patients with meningeal syndrome. Thus, the serological findings coupled with the Meningeal syndrome and the diverse clinical manifestations of both pathogens observed among these patients suggest a possible overlap in clinical expression.

Conclusion: These results highlight the importance of heightened awareness among infectious disease specialists, particularly when faced with confirmed cases of Meningeal Syndrome that may exhibit clinical similarities with Rickettsial diseases, especially in regions where hematophagous arthropod-borne zoonoses are prevalent.

Background: Hematopoietic stem transplantation (HSCT) from matched related donors (MRD) is offered as a curative therapeutic option in children with Sickle cell disease (SCD).

Objective: We wanted to assess the outcome and long-term complications observed in children undergoing HSCT at a single transplant center in Saudi Arabia.

Patients and methods: One hundred and twenty-nine children were transplanted for severe Sickle cell disease (SCD) consecutively from 2006 to 2020 at our center. The main transplant indication was cerebral vasculopathy in 57 (43%), followed by the recurrent vaso-occlusive crisis (VOC) in 47 (36%). Median age at transplant was 9.1 years (range, 1.5-13.9 years). All patients received myeloablative conditioning with Busulfan, Cyclophosphamide, and Anti T-Lymphocyte Globulin (Grafalon®): BU/CY/ATG in 114 (88.4%), BU/CY in 13 (10%) and other in 2 (2%). Bone marrow was the main stem cell source in 123 (95%).

Results: All patients showed granulocyte engraftment. Acute graft-versus-host-disease (aGVHD) and chronic GVHD were observed in 26 (20%) and 12 (9%) patients, respectively. At a median follow-up of 4.36 years (range, 0.13-15.5 years), 10-year overall survival (OS) and event-free survival (EFS) of 94% and 91% was observed. The OS and EFS were significantly better in patients receiving BU/CY/ATG when compared to BU/CY (OS: 97.4%±1.5%, vs. 76.2%±12.1 P=0.003 and EFS: 94.7%±2.1% vs. 76.2%±12.1%, P=0.019).

Conclusion: HSCT for children with sickle cell disease from fully matched siblings offers the best outcome using myeloablative conditioning. However, significant toxicities were observed secondary to myeloablative regimens, in particular long-term complications, which demands exploring the use of less toxic regimens.

Heparin-binding protein (HBP) is a granule protein derived from neutrophils, located in secretory vesicles and neutrophilic granules, also known as cationic antimicrobial protein of 37 kDa (CAP37) or azurocidin. This study evaluates the diagnostic and prognostic value of HBP levels in relation to infection, organ dysfunction, and mortality in adult patients. A systematic review and meta-analysis were conducted by searching PubMed, Web of Science, EMBASE, and the Cochrane Database from their inception through June 2024. Original studies assessing HBP levels' diagnostic and prognostic utility in predicting infection and disease severity in critically ill adult patients were included. The primary outcome was the diagnostic and predictive role of HBP in infection and severity. The Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) tool was used to evaluate bias risk. A total of 56 studies involving 11,486 patients were included. Pooled analysis showed HBP had a sensitivity of 0.87 (95% CI, 0.82-0.91), specificity of 0.87 (95% CI, 0.79-0.92), and an AUC of 0.93 (95% CI, 0.91-0.95) for infection diagnosis. For prognostic assessment, sensitivity was 0.77 (95% CI, 0.74-0.80), specificity was 0.72 (95% CI, 0.68-0.76), and AUC was 0.81 (95% CI, 0.78-0.85). HBP outperformed procalcitonin (PCT), C-reactive protein (CRP), and white blood cell count (WBC) in diagnosing and predicting critical illness. No publication bias was detected. HBP demonstrates high sensitivity and specificity for diagnosing infections in critically ill adult patients. Additionally, it effectively predicts disease progression, including organ dysfunction and mortality, surpassing traditional biomarkers such as PCT, CRP, and WBC. All that cannot be true for subjects with severe neutropenia.