Concurrent congenital hemophilia B and acquired hemophilia A: a unique case report.

Congenital hemophilia B is a rare X-linked recessive bleeding disorder caused by factor IX deficiency. Acquired hemophilia A is a rare, acquired bleeding disorder that presents with new-onset bleeding, especially in older adults, due to the development of auto-antibodies against factor VIII (FVIII). This case report presents the medical management of a patient with congenital hemophilia B and acquired hemophilia A. We highlight the limitations of maintaining factor levels with factor replacement therapy alone, particularly in hemophilia patients who have developed factor inhibitors. In addition, we draw attention to the need for dose escalation, the cost, and the need for immune-tolerance induction therapy. This case illustrates that when the current diagnosis does not explain the full clinical picture and laboratory data are inadequate, it is important to continue to seek alternative diagnoses and cost-effective treatment.

The effect of thrombocytapheresis on hemogram and biochemistry parameters in patients with essential thrombocytemia.

BACKGROUND: Essential thrombocythemia is one of the chronic myeloproliferative neoplasms characterized by clonal proliferation of myeloid cells with variable morphological maturation and hematopoietic activity.It is characterized by excessive clonal platelet production with a tendency to thrombosis and bleeding. Thrombocytapheresis is the removal of platelets by apheresis techniques. Thrombocytapheresis is generally recommended in patients with essential thrombocythemia with acute, severe thrombotic or hemorrhagic events. METHODS: The study included 39 patients who were diagnosed with essential thrombocythemia, started cytoreductive and aspirin therapy, and underwent thrombocytapheresis due to the development of acute severe thrombotic or hemorrhagic events, diagnosed in the adult hematology clinic of Inönü University Turgut Ozal Medical Center. Hemogram and biochemistry values of the patients were scanned retrospectively. RESULTS: After thrombocytapheresis, a statistically significant difference was found between the first and last measurements of hemoglobin, mean platelet volume, White blood cell, neutrophil, platelet, platelet distribution width, creatine, lactate dehydrogenase, fibronogen and calcium levels of the patients. CONCLUSION: The use of thrombocytapheresis in patients with essential thrombocytosis causes a rapid decrease in platelet values as well as an effect on hemogram and biochemistry parameters. Other hemogram and biochemistry parameters such as platelet value should be monitored in patients.

Effect of extracorporeal photopheresis on survival in acute graft versus host disease.

BACKGROUND: Extracorporeal photopheresis (ECP) is the main non-pharmacological approach accompanying systemic medical treatments in steroid-resistant acute or chronic graft versus host disease. The study aimed to examine the effect of ECP on survival in acute graft versus host disease (aGVHD). METHODS: A total of 35 patients who were followed up in the adult hematology clinic of Inönü University Turgut Özal Medical Center for aGVHD were included in the study. Stem cell transplantation and ECP application parameters that may affect the survival of the patients were examined. RESULTS: In aGVHD using ECP, the degree of involvement affects survival. Involvements with a clinical and laboratory score (Glucksberg system) of 2 and above significantly reduced survival. The duration of ECP use is associated with survival. Especially, 45 days and longer use increases survival (hazard ratio, P-value <.05). The duration of steroid use was found to be effective in survival in aGVHD (P < .001). ECP administration day (P = .003), duration of steroid use (P < .001), duration of ECP use (P = .001), and grade of aGVHD (P < .001) affect survival. CONCLUSION: ECP use is effective in survival in patients with aGVHD score ≥2. In patients with aGVHD, especially the use of 45 days and longer has a positive effect on survival. The duration of steroid use is associated with survival in aGVHD.

Does ferritin level affect the outcomes of autologous stem cell transplantation equally in all diseases?

BACKGROUND: In this retrospective study, we evaluated the effect of ferritin levels on the outcomes of autologous stem cell transplantation in patients with MM or lymphoma. METHODS: In this study, 170 patients with measured ferritin levels within one month before transplantation who underwent ASCT with the diagnosis of MM or lymphoma were evaluated. The cut-off value of ferritin was determined as 500 ng/mL to evaluate the transplant outcomes in both groups. The hematological recovery status/duration, febrile neutropenia rate, hospitalization time, transplant-related mortality (TRM) in the first 100 days, and OS were evaluated according to the ferritin level RESULTS: Of all patients, 105 (61,8%) were diagnosed with MM and 65 (38.2%) with lymphoma. Ferritin levels had no statistically significant effect on the engraftment status/times, the febrile neutropenia rates, and hospitalization times of both lymphoma and myeloma patients (p > .05). Ferritin level was not significantly associated with TRM in MM (p = .224). However, in lymphoma, ferritin level was significantly associated with TRM (33.3% for ferritin level ≥500 ng/L vs. 5.3% for ferritin level ng/mL, p = .005). There was no statistically significant correlation between ferritin value and OS in MM group [ferritin level ≥ 500 ng/L: 39.9 months (95% CI: 33.7-46.1) and ferritin level 500 ng/mL: 39.4 months (95% CI: 36.5-42.2), p = .446]. Ferritin level was significantly associated with OS in patients with lymphoma [ferritin level ≥ 500 ng/L: 22.1 months 95% CI: 14.7-29.5), ferritin level 500 ng/mL: 27.3 months (95% CI: 22.4-32.2), p = .038] CONCLUSION: High ferritin level is important prognostic factor on survival after ASCT in patients with lymphoma.

Evaluation of pulmonary artery stiffness and right ventricle functions in polycythemia vera patients by transthoracic echocardiography.

INTRODUCTION: Polycythemia vera (PV) is known to be a subgroup of chronic myeloproliferative neoplasms and is recognized as a cause of pulmonary hypertension (PH). Pulmonary artery stiffness (PAS) is a relatively new noninvasive echocardiographic index developed to evaluate the structural features and functions of the pulmonary vascular bed. In this study, we aimed to evaluate right ventricular (RV) functions and PAS in PV patients and healthy controls. METHODS: A group of 65 consecutive PV patients and 40 healthy controls were included in the study. RV global longitudinal strain (RVGLS) and RV free wall longitudinal strain were (RVFwLS) evaluated using two-dimensional (2D) strain echocardiography. RV volume, systolic and diastolic functions were evaluated with three-dimensional (3D) echocardiography. PAS was calculated using the maximum frequency shift (MFS) and acceleration time of the pulmonary artery flow trace. RESULTS: PAS values were significantly higher in the PV group than in the control group (25.2 ± 5.2 vs. 18.2 ± 4.2, p < .001). We found that tricuspid annular plane systolic excursion (TAPSE) (p < .001), RV fractional area change (p < .001) and RV ejection fraction (p < .001) measurements evaluated by 3D echocardiography were significantly lower in the PV group. CONCLUSION: In our study, PAS values were higher in PV patients than in the healthy control group. Patients with PV may have subclinical RV dysfunction, and PAS value can be used in the early diagnosis of PH and RV dysfunction in this patient group.

The effect of G-CSF used after allogeneic hematopoietic stem cell transplantation on engraftment times and platelet suspension replacement numbers.

BACKGROUND: With the use of granulocyte colony stimulating factor (G-CSF) after allogeneic hematopoietic stem cell transplantation (HSCT), the duration of neutrophil engraftment and hospitalization were shortened. However, there is no consensus on the effect of G-CSF on platelet engraftment time. The primary aim of our study is to determine the effect of G-CSF use on platelet engraftment time after HSCT. Secondary purposes are to determine the number of platelet suspension, number of erythrocyte suspension and incidence of acute graft versus disease after HSCT. MATERIAL AND METHODS: Patients who had allogeneic stem cell transplantation at our center between 01.01.2011 and 01.01.2022 were retrospectively analyzed. Patients were divided into 2 groups as those who received and did not receive G-CSF after transplantation. RESULTS: A total of 64 patients were included. While 32 patients were given post-HSCT G-CSF support, the other 32 patients were not given. Neutrophil engraftment time and length of hospital stay were shorter in the group receiving G-CSF (p < 0.05). Platelet engraftment time was shorter in the group that did not receive G-CSF (p < 0.05). The incidence of acute GVHD of the patients in group 1 tended to be higher than the patients in group 2 (40.6 % vs 15.6 %, p = 0.052). Post-HSCT platelet suspension was less in the group that did not receive G-CSF, but this difference was not statistically significant (p = 0.173). CONCLUSION: While the positive effect of post HSCT G-CSF use on duration of neutrophil engraftment and hospitalization is evident, its effects on platelet engraftment need to be investigated.

The relationship between bioelectrical impedance parameters and pulmonary artery stiffness in obese subjects.

OBJECTIVES: Obesity is a public health problem that needs to be treated and it occurs as a result of excessive fat accumulation in the body. The relationship between obesity and pulmonary hypertension is well known. The aim of this study is to evaluate the relationship between pulmonary artery stiffness, right ventricular functions and bioelectrical impedance parameters in obese, overweight, and healthy individuals. METHODS: In this study, 41 obese (17 female and 24 male, mean age 43.5±10.3), 39 overweight (20 female and 19 male, mean age 38.6±10.4), 34 healthy control group (19 female and 15 male, mean age 40.5±8.6) were included. Anthropometric measurements and bioelectrical impedance parameters of all participants were performed. Right ventricular functions and pulmonary artery stiffness were evaluated by using conventional echocardiography. RESULTS: Right ventricle myocardial performance index, pulmonary artery stiffness values were statistically different between groups. Positive correlation was observed between pulmonary artery stiffness and Body Mass Index, Waist and Hip circumferences. Significant negative correlation was observed between muscle to fat ratio and pulmonary artery stiffness. In the linear regression analysis, it was observed that the muscle to fat ratio was independent predictor of pulmonary artery stiffness (ß = -1.835; 95%CI(-2.434 - - .784); p < 0.001). CONCLUSIONS: This study showed that right ventricular function was impaired and pulmonary artery stiffness increased in obese individuals. These findings could be considered as early markers of pulmonary hypertension in obese patients who do not yet have clinical evidence of cardiovascular disease.

Assessment of left atrial volume and function in patients with Sjögren's syndrome using three-dimensional echocardiography.

OBJECTIVE: We used real time, three-dimensional transthoracic echocardiography (3DTTE) to evaluate left atrial (LA) volume and mechanical function in patients with primary Sjögren's syndrome (SS). METHODS: We prospectively included 42 consecutive patients with primary SS and 42 controls who were similar in terms of basal characteristics. 3DTTE was used to assess LA function. RESULTS: Maximum LA volume, minimum LA volume, pre-atrial contraction LA volume, LA Active Stroke Volume (ASV), LA Total Stroke Volume (TSV), maximal left atrial volume index (LAVImax), Left atrial pre-contraction volume index, and Left atrial minimum volume index, ASV index, and TSV index were significantly higher in the SS group, and the LA Total Emptying Fraction, LA Expansion Index, and LA Passive Emptying Fraction were significantly lower. Although the active emptying fraction was higher in the SS group, the difference was not statistically significant. LAVImax was positive correlated with disease duration (r = .753). CONCLUSION: Left atrial function is impaired in SS patients and serves as an early marker of subclinical cardiac involvement.