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Annals of African Medicine 2023Aplastic anemia (AA) is an uncommon condition characterized by pancytopenia and hypocellular bone marrow. Interleukin (IL)-6 and IL-8 have been shown to inhibit...
BACKGROUND
Aplastic anemia (AA) is an uncommon condition characterized by pancytopenia and hypocellular bone marrow. Interleukin (IL)-6 and IL-8 have been shown to inhibit myelopoiesis and are major mediators of tissue damage. The primary goal of this study was to determine the IL-6 and IL-8 levels in children with AA, as well as their relationship to illness severity and immunosuppressive medication response.
MATERIALS AND METHODS
The IL-6 and IL-8 levels were tested in 50 children aged 3-18 years who had AA. As controls, 50 healthy age and sex matched individuals were used. A sandwich enzyme-linked immunosorbent assay kit (solid-phase) was used to measure IL-6 and IL-8 levels quantitatively. The concentrations of IL-6 and IL-8 in pg/mL were used to represent the results. Immunosuppressive medication was given to the patients in accordance with the British Committee for Standards in Haematology Guidelines 2009.
RESULTS
The patients' average age was 11.3 ± 3.7 years. Patients with AA had significantly higher IL-6 and IL-8 levels than controls (278.88 ± 216.03 vs. 4.51 ± 3.26; P < 0.001) and (120.28 ± 94.98 vs. 1.79 ± 0.78; P < 0.001), respectively. The IL-6 and IL-8 levels were also investigated with respect to AA severity, with statistically significant differences (P < 0.01) between different grading strata. Patients with very severe AA (VSAA) had the highest IL-6 levels (499.52 ± 66.19), followed by severe AA (SAA) (201.28 ± 157.77) and non-SAA (NSAA) (22.62 ± 14.63). For IL-8 levels, a similar trend (P < 0.01) was detected, with values of 209.81 ± 38.85, 92.12 ± 78.0, and 9.29 ± 10.68 for VSAA, SAA, and NSAA, respectively. After 6 months of immunosuppressive treatment (IST), mean levels of IL-6 and IL-8 in responders and nonresponders were again assessed. The mean IL-6 level in the responders' group (46.50 ± 45.41) was significantly lower, when compared to the nonresponders' group (145.76 ± 116.32) (P < 0.001). Similarly, the mean IL-8 level in the responder's group (33.57 ± 27.14) was significantly lower, compared to the nonresponder's group (97.49 ± 69.00) (P < 0.001).
CONCLUSIONS
Children with AA had higher IL-6 and IL-8 levels than normal age- and sex-matched controls. Increased levels were linked to the severity of the condition, suggesting that IL may have a role in AA. IL levels can be monitored in AA patients during IST, which can assist in predicting response to IST.
Topics: Child; Humans; Adolescent; Anemia, Aplastic; Interleukin-6; Interleukin-8; Treatment Outcome; Immunosuppressive Agents; Immunosuppression Therapy; Patient Acuity
PubMed: 38358144
DOI: 10.4103/aam.aam_106_22 -
Mikrobiyoloji Bulteni Oct 2023Malaria is a parasitic disease transmitted by infected female Anopheles mosquitoes. There are five species of Plasmodium species that can infect humans. Of these...
Malaria is a parasitic disease transmitted by infected female Anopheles mosquitoes. There are five species of Plasmodium species that can infect humans. Of these species, especially P.falciparum and P.vivax pose the greatest threat to human health. In the 2014 report of the World Health Organization, it was reported that there were no locally acquired cases of malaria in 16 countries including Türkiye. Malaria cases originating from outside the country and imported due to migration, travel and working abroad are reported as import cases. In this report, a case of non-imported malaria followed with a preliminary diagnosis of leukemia was presented. A 14-year-old female patient who was admitted to a health institution with complaints of high fever, headache, chills, nausea-vomiting, and diarrhea that had been going on for two weeks, was pre-diagnosed as leukemia and was referred to Manisa Celal Bayar University Faculty of Medicine, Hafsa Sultan Hospital, Department of Pediatric Hematology and after pancytopenia was detected in the complete blood count. The anamnesis of the patient revealed that she had no history of international travel and that she had been prescribed medications such as paracetamol, amoxicillin, and metoclopramide for flu-like complaints while working in the Southeastern Anatolia, Aegean, and Mediterranean Regions of Türkiye. Bone marrow aspiration was performed for the etiological examination of pancytopenia. Giemsa-stained blood smears, rapid diagnostics, and real-time quantative polymerase chain reaction (qRt-PCR) analyses were performed in the medical parasitology laboratory and malaria was suspected in both bone marrow and peripheral blood smears. P.vivax erythrocytic forms and gametocytes were present in abundance in smear preparations stained with Giemsa, and rapid diagnosis kit was positive for P.vivax. The strain was genotyped as P.vivax by qRt-PCR analysis. For the treatment of the patient, airalam (artemether + lumefantrine) tablets were provided with 2 x 4 daily posology for three days after the diagnosis, and primaquine was provided after one week of the diagnosis as 1 x 2 tablets (1 x 15 mg) for 14 days, and the patient was discharged without complications following the treatment regimen. The fight against malaria continues uninterruptedly since the establishment of the Republic of Türkiye. Tropical diseases, especially malaria, is of great importance for Türkiye due to numerous reasons such as its location in the subtropical region where Anopheles mosquitoes are capable of malaria transmission, it is situated at the crossroads on the migration routes between continents where human traffic is busy, there are many people who go abroad for work and most importantly rising temperatures due to climate change. For this reason, this case report is important to emphasize the importance of malaria for the country and to increase the awareness of clinicians and laboratories about malaria and the possibility of autochthonous malaria transmission in Türkiye.
Topics: Adolescent; Animals; Female; Humans; Leukemia; Malaria; Malaria, Vivax; Pancytopenia; Plasmodium; Travel
PubMed: 37885398
DOI: 10.5578/mb.20239958 -
Revista Espanola de Enfermedades... Nov 2023This case reports illustrates a 44-year-old Caucasian male with ileal Crohn´s disease under combined immunosuppression that first presented with unspecific...
This case reports illustrates a 44-year-old Caucasian male with ileal Crohn´s disease under combined immunosuppression that first presented with unspecific constitutional symptoms, newly pancytopenia and elevated inflammatory markers. The infectious screening was negative except for an ileal abscess that resolved with conservative antibiotic therapy. Due to concerns for lymphoproliferative disease in a patient under anti-TNF and azathioprine, a myelogram was performed that ruled out dysplastic changes. After abscess resolution the symptoms relapsed with evening fever, nocturnal sudoresis and worsen pancytopenia. A more thorough work-up was performed with bone marrow and ileal biopsies that demonstrated numerous intra-and-extracellular leishmania amastigote forms, which confirmed the diagnosis of visceral leishmaniasis infiltrating the small bowel. The patient recovered after adequate treatment and withheld of immunosuppression during follow-up.
PubMed: 38031908
DOI: 10.17235/reed.2023.10097/2023 -
Frontiers in Immunology 2023Cryoglobulinemic vasculitis is a type of small vessel vasculitis diseases that can cause dysfunction in multiple organs. It is characterized by general symptoms, often...
INTRODUCTION
Cryoglobulinemic vasculitis is a type of small vessel vasculitis diseases that can cause dysfunction in multiple organs. It is characterized by general symptoms, often accompanied by nonspecific cutaneous, articular, neurological, and renal manifestations. Diagnosing cryoglobulinemia through biological testing can be time-consuming and sometimes yields negative results, making diagnosis challenging. There are also other potentially life-threatening complications that can significantly impact prognosis and delay urgent treatment, including digestive manifestations and heart failure.
CASE PRESENTATION
We report the case of a 60-year-old male patient with a medical history of rheumatoid arthritis. He was admitted to the Nephrology Department for investigation of necrotic vascular purpura, acute kidney injury, and pancytopenia. Laboratory tests revealed consumption of the C3 and C4 complement fractions and the presence of mixed-type III cryoglobulinemia. Despite the initiation of the treatment, the patient rapidly developed multiple severe organ failures, including renal, cardiac, respiratory, and finally digestive complications. Acute colic ischemia led to emergency surgery and the patient was transferred to the Intensive Care Unit. Despite surgical intervention and hemodynamic support, the patient experienced multi-visceral organ failure and died two hours after admission.
DISCUSSION
Mixed cryoglobulinemia vasculitis may result in rare cases of acute and life-threatening organ damage, such as cardiac or respiratory failure with pulmonary hemorrhage, gastrointestinal ischemia, and neurological disorders. These severe manifestations are associated with a poor prognosis and it is crucial to promptly initiate an aggressive therapeutic strategy.
Topics: Male; Humans; Middle Aged; Cryoglobulinemia; Vasculitis; Complement C4; Prognosis; Multiple Organ Failure; Ischemia
PubMed: 37901234
DOI: 10.3389/fimmu.2023.1271584 -
BMC Pediatrics Aug 2023Lysinuric protein intolerance is a rare inherited metabolic disease due to autosomal recessive mutations of the SLC7A7 gene. The affected patients commonly present with...
BACKGROUND
Lysinuric protein intolerance is a rare inherited metabolic disease due to autosomal recessive mutations of the SLC7A7 gene. The affected patients commonly present with protein-rich food intolerance, failure to thrive, hepatosplenomegaly, muscle hypotonia and lung involvement due to impaired intestinal absorption and excessive urinary excretion of dibasic amino acids. Presentation with splenomegaly and cytopenia without other features has not been reported. Here we report a Sri Lankan girl with lysinuric protein intolerance presenting with pancytopenia and splenomegaly mimicking acute leukaemia.
CASE PRESENTATION
Two years and six months old Sri Lankan girl presented with persistent pancytopenia following a viral illness. She was asymptomatic without vomiting, diarrhoea, abdominal pain or irritability. Physical examination revealed pallor and isolated firm splenomegaly of 2 cm. Growth parameters and other system examinations were normal. Full blood count revealed anaemia, leukopenia and thrombocytopenia. The blood picture showed a mixture of hypochromic microcytic and normochromic normocytic red cells with occasional pencil cells and macrocytes. Bone marrow examination was normal except for occasional megaloblasts; however, serum vitamin B12 and red blood cell folate were normal. The metabolic screen showed a high anion gap compensated metabolic acidosis, high lactate and ketosis. Genetic mutation analysis using whole exome sequencing revealed compound heterozygous variants of the SLC7A7 gene, confirming the diagnosis of lysinuric protein intolerance.
CONCLUSION
We report a child with lysinuric protein intolerance presenting with pancytopenia and splenomegaly without other disease features. This case report adds to the heterogenic presentations of lysinuric protein intolerance, which is considered a multifaceted disease.
Topics: Child; Female; Humans; Infant; Pancytopenia; Splenomegaly; Amino Acid Metabolism, Inborn Errors; Leukemia; Leukopenia; Thrombocytopenia; Amino Acid Transport System y+L
PubMed: 37528333
DOI: 10.1186/s12887-023-04207-7 -
Molecular Genetics and Metabolism Dec 2023Many classical inherited metabolic diseases (IMDs) are associated with significant hematological complications such as anemia or thrombosis. While these may not be the... (Review)
Review
Many classical inherited metabolic diseases (IMDs) are associated with significant hematological complications such as anemia or thrombosis. While these may not be the prominent presenting feature of these conditions, management of these issues is important for optimal outcomes in people with IMDs. Some disorders that are included in the nosology of inherited metabolic disorders, such as inherited disorders of red cell energy metabolism, have purely hematological features, and have typically been cared for by a hematologist. In the 16th issue of the Footprints series, we identified 265 IMDs associated with hematological abnormalities. We review the major hematological manifestations of IMDs, suggest further investigation of hematological findings, and discuss treatment options available for specific hematological complications of IMDs.
Topics: Humans; Metabolic Diseases; Anemia
PubMed: 37989003
DOI: 10.1016/j.ymgme.2023.107735 -
Saudi Medical Journal May 2024To investigate the prevalence of hematologic findings and the relationship between hemogram parameters and brucellosis stages in patients.
OBJECTIVES
To investigate the prevalence of hematologic findings and the relationship between hemogram parameters and brucellosis stages in patients.
METHODS
This multi-center study included patients older than 16 years of age who were followed up with a diagnosis of brucellosis. Patients' results, including white blood cell, hemoglobin, neutrophil, lymphocyte, monocyte, mean platelet volume, platelet and eosinophil counts were analyzed at the initial diagnosis.
RESULTS
In this study 51.3% of the patients diagnosed with brucellosis were male. The age median was 45 years for female and 41 years for male. A total of 55.1% of the patients had acute brucellosis, 28.2% had subacute, 7.4% had chronic and 9% had relapse. The most common hematologic findings in brucellosis patients were anemia (25.9%), monocytosis (15.9%), eosinopenia (10.3%), and leukocytosis (7.1%). Pancytopenia occurred in 0.8% of patients and was more prominent in the acute phase. The acute brucellosis group had lower white blood cell, hemoglobin, neutrophil, eosinophil, and platelet counts and mean platelet volume, and higher monocyte counts compared to subacute and chronic subgroups.
CONCLUSION
It was noteworthy that in addition to anemia and monocytosis, eosinopenia was third most prominent laboratory findings in the study. Pancytopenia and thrombocytopenia rates were low.
Topics: Humans; Brucellosis; Male; Female; Adult; Middle Aged; Turkey; Young Adult; Thrombocytopenia; Adolescent; Aged; Anemia; Blood Cell Count
PubMed: 38734423
DOI: 10.15537/smj.2024.45.5.20230847 -
Medicine Oct 2023Hemophagocytic lymphohistiocytosis (HLH) is a progressive and potentially life-threatening disorder. It is classified into primary and secondary HLH. The objective of...
Hemophagocytic lymphohistiocytosis (HLH) is a progressive and potentially life-threatening disorder. It is classified into primary and secondary HLH. The objective of our study was to determine the outcome of primary and secondary HLH in pediatric and adult patients based on HScore and treatment modality. We conducted a retrospective analysis done from July 2010 to June 2020. Variables analyzed included age, gender and history of death in siblings. HScore was used for disease classification while clinical and laboratory findings which were required to fulfill the HScore diagnostic criteria were also recorded. Continuous variables were summarized as median and categorical variables as frequencies and percentages. Categorical variables were compared using chi-square test and Fisher Exact test. Significance of different variables between primary and secondary HLH was calculated using independent-samples t test. A P value of < .05 was taken as significant. A total of 51 patients were included in the analysis (41 in primary and 10 in secondary HLH group). In primary HLH, 36 patients were in the pediatric age group and 12.2% had a history of death in sibling. All 41 patients had increased ferritin and decreased fibrinogen levels. The overall survival in primary HLH was 44%. In the secondary HLH group, viral infections were the most common etiology and ferritin was increased as well. The overall survival in secondary HLH was 60%. The median survival was 15 ± 4.8 months. The overall survival of both groups combined was 53%. Primary HLH should be considered in pediatric patients who present with pancytopenia and hepatosplenomegaly. In centers where genetic testing is not available, HScore along with serum ferritin and fibrinogen is a good substitute for disease classification.
Topics: Adult; Humans; Child; Retrospective Studies; Lymphohistiocytosis, Hemophagocytic; Tertiary Care Centers; Fibrinogen; Ferritins
PubMed: 37904401
DOI: 10.1097/MD.0000000000034898 -
Cureus Jun 2023Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease characterized by excessive immune response activation. Numerous conditions, including...
Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease characterized by excessive immune response activation. Numerous conditions, including infectious etiologies, are implicated in its development. We report the case of a 16-year-old girl with HLH associated with polyserositis and infection. A 16-year-old girl presented with a high-grade fever and abdominal pain that had been ongoing for 20 days. She had been treated for malaria at a local hospital but was referred to our hospital due to the worsening of her condition. On examination, she was found to have an enlarged liver and spleen, pale skin, and hypotension, with bilateral basal crackles on chest examination. Her blood profile revealed pancytopenia, elevated C-reactive protein, and a deranged coagulation profile. Peripheral smears showed anisocytosis, microcytes, hypochromia in RBCs, and a few platelet clumps. A bone marrow biopsy revealed increased megakaryocytes and hemophagocytes. Ultrasound and computed tomography of the abdomen and pelvis showed hepatosplenomegaly, pericholecystic edema, mild ascites, and long-segment diffuse colonic wall thickening, suggesting pancolitis. Blood culture revealed , which is rarely associated with HLH. The patient was started on the HLH-2004 protocol and showed improvement on the fourth day of initiating therapy, but due to a delayed diagnosis, the patient collapsed on the sixth day of admission. HLH is a rare but life-threatening disease with various underlying causes. The diagnosis of HLH is challenging, and early diagnosis and prompt treatment are crucial for a better prognosis. The association between HLH and infection is rare, and this case highlights the importance of considering unusual etiologies in HLH. Clinicians should be vigilant about this association, especially in endemic regions, to ensure early diagnosis and prompt treatment.
PubMed: 37525775
DOI: 10.7759/cureus.41182 -
Journal of Medical Case Reports Aug 2023Nitrous oxide is a medical and household gas that has seen its use drift to recreational purpose among the young population in recent years. Significant neurological,...
BACKGROUND
Nitrous oxide is a medical and household gas that has seen its use drift to recreational purpose among the young population in recent years. Significant neurological, hematological and psychiatric side effects, generally related to an induced functional vitamin B12 deficiency, have been described separately in the literature.
CASE REPORT
A 22-year-old woman of North African origin experienced an exceptional combination of polyneuropathy, bilateral pulmonary embolism and severe pancytopenia related to vitamin B12 deficiency and hyperhomocysteinemia induced by recreational nitrous oxide use. After treatment with vitamin B12 supplementation and intensive rehabilitative management, the patient progressively regained the ability to walk and her biological parameters gradually returned to normal. The pathophysiological mechanisms related to a decrease in vitamin B12 activity are the reduction of products needed for synthesis of deoxyribonucleic acid, carbohydrate or fatty acids, and the increase of hyperhomocysteinemia. Other mechanisms involving a direct action of N2O are also suspected.
CONCLUSION
This case report brings elements to support our knowledge about pathological pathway, recovery and prognosis of recreational N2O abuse complications. The general and medical population should be aware to the serious consequences of this type of consumption.
Topics: Female; Humans; Young Adult; Adult; Pancytopenia; Hyperhomocysteinemia; Nitrous Oxide; Polyneuropathies; Pulmonary Embolism
PubMed: 37587485
DOI: 10.1186/s13256-023-04075-w