Introduction
To the best of our knowledge, the present study is the first in the literature to assess distal femoral cartilage thickness and its relationship with ferritin levels in adult patients ...with beta thalassaemia major (BTM).
Materials and methods
45 patients with BTM and 45 healthy controls were included in the study. Ferritin and haemoglobin levels of the patient and healthy groups were determined by blood analysis and distal femoral cartilage thicknesses were measured via ultrasound. Then, the patient group was divided into subgroups according to whether their ferritin levels were below or above 2500 µg/L. They were then compared among themselves and with the healthy control group using the available data.
Results
Distal femoral cartilage thickness values were statistically significantly lower in the BTM group compared to the healthy control group (
p
values < 0.001). Patients with a ferritin level below 2500 µg/L had statistically significantly higher right and left average distal femoral cartilage thickness values than the patients with a ferritin level above 2500 µg/L (
p
= 0.029 and
p
= 0.019, respectively). The right and left average distal femoral cartilage thickness values of the patient subgroup with low ferritin levels were statistically similar to the control group (
p
= 0.146 and
p
= 0.164, respectively).
Conclusion
Our study showed that thalassaemia patients are more likely to develop osteoarthritis (OA) than the normal population and possible OA development can be prevented by keeping the ferritin levels of these patients in the optimum range.
Hemolytic anemia defines a group of anemias occurring due to the shortening of normal red blood cell (RBC) lifespan due to factors extrinsic to RBCs or structural changes in RBCs. As a result of the ...increase in RBC hemolysis, anemia and associated clinical symptoms become manifest. Hemolytic anemias can be categorized under two broad titles: hereditary and acquired. Here, we present a case diagnosed with pemphigus vulgaris who was determined to have Glucose-6-phosphate dehydrogenase (G6PD) deficiency based on the tests performed subsequent to hemolytic anemia that occurred during dapsone therapy.
66 year-old female patient presented to the dermatology polyclinic with raised erythema and bullous lesions in a butterfly distribution on the face involving the eyelids. The patient was diagnosed with pemphigus vulgaris based on punch biopsy and, as treatment, was started on 2 × 50 mg dapsone (PO), 1 × 16 mg methylprednisolone (PO) and corticosteroid pomades. Blood parameters at diagnosis were as follows: leukocyte, 8.1 × 109/L (4.4-11); hemoglobin (Hgb), 12.3 gr/dl (12-16); thrombocyte, 270 × 109/L (142-424); MCV, 86 fl (80-100); LDH, 210 U/L (135-214); ALT, 22 U/L (0-33); AST, 16 U/L (0-32); direct bilirubin, 0.5 mg/dl (0-0.3); indirect bilirubin, 0.8 mg/dl (0.1-0.9); creatinine, 0.59 mg/dl (0.5-0.9); folate, 10 ng/ml (5.4-24); vitamin B12, 310 ng/ml (210-910). The patient presented to the dermatology polyclinic 6 days after the onset of treatment due to fatigue, pallor, icterus of the sclerae. The patient was referred to the hematology polyclinic based on the following test results: Hgb, 3.8 gr/dl; leukocyte, 11 × 109/L; thrombocyte, 222 × 109/L; MCV, 108 fl; creatinine, 0.8 gr/dl; LDH, 810 U/L; indirect bilirubin, 6.4 mg/dl; direct bilirubin, 0.8 mg/dl.
The patient's history and anamnesis did not include a similar condition that followed medication use or an operation. On physical examination; sclerae were icteric, skin was pale, and there was no organomegaly or peripheral lymphadenopathy. In addition, urine was dark in color. On peripheral blood smear; macrocytosis, anisocytosis-poikilocytosis, polychromasia and Heinz bodies were observed. Corrected reticulocyte was determined as 5.2% (0.5-2%); ANA, anti-dsDNA, direct Coombs (IgG) and indirect Coombs' tests were negative. The haptoglobulin level was determined as 8 mg/dl (30-200) and was below the reference range. As the present hemolytic anemia picture was reasoned to be associated with dapsone, the medication was stopped and 16 mg methylprednisolone was started. No pathological findings were determined on abdominal ultrasonography and lung radiography. Based on the perception that anemia was associated with dapsone, G6PD enzyme levels were examined. The patients' G6PD level was found as 3.52 IU/gHb (7.48-10.20 IU/gHb), and was below the reference. During follow-up, fatigue, subicterus and pallor improved. Hgb levels increased, LDH and indirect bilirubin levels showed a gradual decrease. Blood parameters after 10 days were as follows: Hgb 11,8, gr/dl; leukocyte, 7.6 × 109/L; thrombocyte, 234 × 109/L; MCV, 98 fl; creatinine, 0,6 gr/dl; LDH, 260 U/L; direct bilirubin, 0.42 mg/dl; indirect bilirubin, 0.44 mg/dl.
Dapsone is used widely in the treatment of various disorders, most notably, dermatological disorders. In G6PD deficiency, using dapsone is risky and is associated with a high probability of hemolytic anemia occurrence. In this case presentation, we aimed to stress that hemolytic anemia encountered in a patient on dapsone would be linked to G6PD enzyme deficiency.
This study aimed to evaluate patients with relapsed/refractory multiple myeloma (RRMM) who underwent daratumumab (DARA) therapy.
This multicenter retrospective study included 134 patients who ...underwent at least two courses of DARA from February 1, 2018, to April 15, 2022. Epidemiological, disease, and treatment characteristics of patients and treatment-related side effects were evaluated. Survival analysis was performed.
The median age at the start of DARA was 60 (range: 35-88), with 56 patients (41.8%) being female and 48 (58.2%) being male. The median time to initiation of DARA and the median follow-up time were 41.2 (5.1-223) and 5.7 (2.1-24.1) months, respectively. The overall response rate after DARA therapy was 75 (55.9%), and very good partial response or better was observed in 48 (35.8%) patients. Overall survival (OS) and progression-free survival (PFS) for all patients were 11.6 (7.8-15.5) and 8.0 (5.1-10.9) months, respectively. OS was higher for patients undergoing treatment with DARA and bortezomib-dexamethasone (DARA-Vd) compared to those undergoing treatment with DARA and lenalidomide-dexamethasone (DARA-Rd) (16.9 vs. 8.3 months; p=0.014). Among patients undergoing DARA-Rd, PFS was higher in those without extramedullary disease compared to those with extramedullary disease (not achieved vs. 3.7 months; odds ratio: 3.4; p<0.001). The median number of prior therapies was 3 (1-8). Initiation of DARA therapy in the early period provided an advantage for OS and PFS, although it was statistically insignificant. Infusion-related reactions were observed in 18 (13.4%) patients. All reactions occurred during the first infusion and most reactions were of grade 1 or 2 (94.5%). The frequency of neutropenia and thrombocytopenia was higher in the DARA-Rd group (61.9% vs. 24.7%, p<0.001 and 42.9% vs. 15.7%, p<0.001).
Our study provides real-life data in terms of DARA therapy for patients with RRMM and supports the early initiation of DARA therapy.
Hemolytic anemia is characterized by a decrease in the number of circulating erythrocytes due to an increase in their hemolysis. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common ...erythrocyte enzyme defects related to hemolysis. The G6PD enzyme abrogates the hemolysis of erythrocytes by protecting them against oxidative stress due to its involvement in the glutathione metabolism. G6PD enzyme deficiency-related hemolytic anemia may present as neonatal jaundice or become manifest due to exposure to infections, favism and medications later in life. Dapsone is a medication that is preferred by doctors in the treatment of many dermatological disorders such as pemphigus vulgaris, and leads to hemolysis in the presence of G6PD enzyme deficiency. In this type of non-immune hemolysis, haptoglobulin is low and Coombs' tests are negative. Hemolytic anemia, a serious complication that may appear subsequent to dapsone use, can be prevented by testing G6PD enzyme levels prior to dapsone therapy. In this case, we emphasized that the hemolytic anemia in the patient using dapsone may be due to G6PD enzyme deficiency.
Aims: The aim of this study was to evaluate the rate of hepatitis B virus reactivation (HBVr) in hematology patients receiving immunosuppressive therapy in our center and the clinical characteristics ...of patients with HBVr. We will also investigate the importance of effective prevention of this potentially life-threatening event and management of hepatitis B virus (HBV) prophylaxis.
Methods: In this study, hepatitis B prophylaxis and its effects on patients over 18 years of age receiving immunosuppressive therapy in the hematology clinic were analyzed. 122 patients were included in the study. The HBV markers of the patients were determined by the chemiluminescence method. In the study, HbsAg(+) and isolated antiHbc IgG-positive patients received prophylactic antiviral treatment. The differential diagnosis of HBV reactivation and the criteria determined to define HBV reactivation were performed. Clinical characteristics and descriptive information of patients receiving HBV prophylaxis were analyzed using SPSS 25.0.
Results: The median age of 122 patients (59.8% male) was 58 years. It was determined that five HbsAg-positive patients had no prior follow-up and did not receive antiviral treatment. 117 patients had isolated anti-HBc IgG positivity. The median duration of prophylaxis was 15 (9- 21.25) months, and the total follow-up period was 19.5 (11.75- 30.25) months. 81.1% of the patients received regular HBV prophylaxis treatment; 59% of them received entecavir, and the rest received tenofovir disoproxil. Bone marrow transplantation was performed in 25 patients. HBV reactivation was detected in only 4 patients (3.3%); one of these patients had received allogeneic and one autologous bone marrow transplantation; and three patients had received chemoimmunotherapy including Rituximab. The diagnoses of the patients with HBVr were acute myeloid leukemia, lymphoma, and chronic lymphocytic leukemia. During the follow-up period, 29 patients (23.8%) died due to their primary disease, but there were no deaths due to HBV reactivation.
Conclusion: The data obtained in this study show that effective hepatitis B prophylaxis treatment is successful in preventing HBV reactivation in hematology patients. HBVr was observed in four patients who did not take HBV prophylaxis medication regularly.
Cancer was a common noncommunicable disease in Syria before the present conflict and is now a major disease burden among 3.6 million Syrian refugees in Turkey. Data to inform health care practice are ...needed.
To explore sociodemographic characteristics, clinical characteristics, and treatment outcomes of Syrian patients with cancer residing in the southern border provinces of Turkey hosting more than 50% of refugees.
This was a retrospective hospital-based cross-sectional study. The study sample consisted of all adult and children Syrian refugees diagnosed and/or treated for cancer between January 1, 2011, and December 31, 2020, in hematology-oncology departments of 8 university hospitals in the Southern province of Turkey. Data were analyzed from May 1, 2022, to September 30, 2022.
Demographic characteristics (date of birth, sex, and residence), date of first cancer-related symptom, date and place of diagnosis, disease status at first presentation, treatment modalities, date and status at last hospital visit, and date of death. The International Statistical Classification of Diseases and Related Health Problems, Tenth Revision and International Classification of Childhood Cancers, Third Edition, were used for the classification of cancer. The Surveillance, Epidemiology, and End Results system was applied for staging. The diagnostic interval was defined as the number of days from first symptoms until the diagnosis. Treatment abandonment was documented if the patient did not attend the clinic within 4 weeks of a prescribed appointment throughout the treatment.
A total of 1114 Syrian adult and 421 Syrian children with cancer were included. The median age at diagnosis was 48.2 (IQR, 34.2-59.4) years for adults and 5.7 (IQR, 3.1-10.7) years for children. The median diagnostic interval was 66 (IQR, 26.5-114.3) days for adults and 28 (IQR, 14.0-69.0) days for children. Breast cancer (154 13.8%), leukemia and multiple myeloma (147 13.2%), and lymphoma (141 12.7%) were common among adults, and leukemias (180 42.8%), lymphomas (66 15.7%), and central nervous system neoplasms (40 9.5%) were common among children. The median follow-up time was 37.5 (IQR, 32.6-42.3) months for adults and 25.4 (IQR, 20.9-29.9) months for children. The 5-year survival rate was 17.5% in adults and 29.7% in children.
Despite universal health coverage and investment in the health care system, low survival rates were reported in this study for both adults and children with cancer. These findings suggest that cancer care in refugees requires novel planning within national cancer control programs with global cooperation.
Objectives. Hypertension and hypervolemia relationship was proven among renal disease, although it is not known in normal population. Present study determines the fluid distribution defects in ...relation to blood pressure. Material and Methods. In a population-based survey in Turkey demographics, height, weight, blood pressure, urine analysis, and serum creatinine measurements were recorded. Bioimpedance measured with the Body Composition Monitor. Results. Total 2034 population of 71.6% male, mean age 47 ± 12.6 (18–89) years, systolic blood pressure (SBP) 134.7 ± 20, diastolic blood pressure 77.9 ± 11.6 mmHg. Body mass index (BMI) was 28.5 ± 4.5 (15.8–50.6) kg/m2; overhydration was 0.05 ± 1.05 L. There was a correlation between extracellular water (ECW)/height and SBP (r = 0.21, P < 0.001). Receiver operating characteristic (ROC) curve with the performance of 0.60 (P < 0.001) that showed cut-off value of ECW/height was 10.06 L/m, with the 69% sensitivity and 45% specificity for SBP: 140 mmHg values. Risk factors for high SBP were increase of ECW/Height, age, BMI and presence of diabetes. ECW/height, SBP, and fat tissue index (FTI) increased in BMI categories (low, normal, and obese) and in diabetics. SBP and FTI were lower in smokers. Conclusions. High blood pressure may be accompanied by increased extracellular volume indices. In the future volume status assessment could be of use in evaluating the effectiveness of pharmacological intervention in the treatment of hypertension.
Colistin methanesulfonate (CMS), a clinical form of colistin, is widely used as a last-line treatment for multidrug-resistant (MDR) gram-negative bacterial infections in critically ill patients ...presenting a considerably high mortality rate. However, nephrotoxicity is considered to be a critical adverse effect that limits CMS’s clinical use. Alpha-lipoic acid (ALA) is a strong antioxidant that is effective in preventing nephrotoxicity in many models. The aim of this study was to investigate ALA’s ability to protect against nephrotoxicity induced by colistin in rats. Male Wistar albino rats were randomly divided into four groups. Group 1 was the control group (Control; n = 6), in which isotonic saline was administered to the rats. Group 2 was the ALA group (ALA; n = 6) in which rats received 100 mg/kg ALA. Groups 3 was the CMS (CMS; n = 7) in which 450.000 IU/kg/day of CMS was administered to the rats. Groups 4 was the CMS + ALA group (n = 6), in which rats were injected with 100 mg/kg of ALA 30 min before administration of CMS. All injections were performed intraperitoneally at 1, 4, 7, and 10 days. Urine was collected by using a metabolic cage for 24 h after each administration. The rats were euthanized under ether anesthesia after 24 h of the last administration. Blood and kidney samples then were collected for histological and biochemical analysis. ALA pretreatment could reverse the effects of colistin-induced nephrotoxicity, partly through its suppressing effect on Nox4 and caspase-3, which in turn results in its antioxidant and antiapoptotic effect. Therefore, ALA may be an effective strategy for the management of colistin nephrotoxicity.
Objective
Pulmonary complications are common in patients with liver cirrhosis. Devolopment of pulmonary hypertension (PH) is associated with a poor prognosis in these patients. Pulmonary arterial ...stiffness (PAS) is considered an early sign of pulmonary vascular remodeling. The aim of this study is to investigate PAS and compare it with right ventricular (RV) functions in patients with cirrhosis who are scheduled for liver transplantation.
Methods
The study included 52 cirrhosis patients (mean age 51.01 ± 12.18 years, male gender 76.9%) who were prepared for liver transplantation and 59 age and sex matched (mean age 51.28 ± 13.63 years, male gender 62.7%) healthy individuals. Patients with left ventricular ejection fraction (LVEF) less than 55%, ischemic heart disease, more than mild valvular heart disease, chronic pulmonary disease, congenital heart disease, rheumatic disease, moderate to high echocardiographic PH probability, rhythm or conduction disorders on electrocardiography were excluded from the study. In addition to conventional echocardiographic parameters, PAS value, pulmonary vascular resistance (PVR) and RV ejection efficiency was calculated by the related formulas with transthoracic echocardiography (TTE).
Results
Demographic characteristics and cardiovascular risk factors of the groups were similar. PAS, PVR, and sPAP values were found to be significantly higher in the patient group (20.52 ± 6.52 and 13.73 ± 2.05; 1.43 ± 0.15 and 1.27 ± 0.14; 27.69 ± 3.91 and 23.37 ± 3.81 p < 0.001, respectively). RV FAC and RV Ee were significantly lower and RV MPI was significantly higher in the patient group (45.31 ± 3.85 and 49.66 ± 3.62, p < 0.001; 1.69 ± 0.35 and 1.85 ± 0.23, p = 0.005; 0.39 ± 0.07 and 0.33 ± 0.09, p = 0.001, respectively). PAS was significantly correlated with RV FAC and MPI (r = −0.423, p < 0.001; r = 0.301, p = 0.001, respectively).
Conclusions
Increased PAS in cirrhosis patients may be associated with early pulmonary vascular involvement. Evaluation of RV functions is important to determine the prognosis in these patients. FAC, MPI, and RV Ee measurements instead of TAPSE or RV S′ may be more useful in demonstrating subclinical dysfunction. The correlation of PAS with RV FAC and MPI may indicate that RV subclinical dysfunction is associated with early pulmonary vascular remodeling in patients with liver cirrhosis.
The correlation between PAS and right ventricular FAC values in the study group.
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