Amina H Hassab, Dalia A Nafea, Rania S Swelem and Basma M Ghazal
Acute myeloid leukemia (AML) is a heterogeneous hematologic malignancy characterized by a clonal expansion of myeloid blasts. Treatment strategies of patients with AML are based on various prognostic factors, including age and performance status of the patient, as well as cytogenetic and molecular characteristics of the leukemic clone.
Our aim was to study the expression of cluster of differentiation (CD)25 in adult Egyptian patients with newly diagnosed AML and to assess its prognostic relevance.
This study was conducted on 50 newly diagnosed AML patients at the Hematology Unit, Internal Medicine Department, Alexandria Main University Hospital. All patients were subjected to full history taking, thorough clinical examination, and laboratory investigations, including detection of CD25 expression on blast cells by flow cytometry. Conventional karyotyping was done on 11 patients at the time of diagnosis.
In our study group, 12 patients were positive for CD25 expression, and this positivity was associated with worse overall survival and shorter leukemia-free survival. On evaluating the response to treatment among CD25-positive AML patients with normal karyotype, they had lower complete remission rates and higher relapse and death rates.
Expression of CD25 in AML patients at presentation can be considered a poor independent prognostic factor.
Yelda Dere, Volkan Karakuş, Özcan Dere and Nazan Özsan
Synchronous malignancies are rare conditions in oncology practices, generally seen as solid tumors with hematological neoplasms. However, occurrence of two different hematological malignancies in the same patient is extremely rare. Two primary malignancies should be considered especially in patients with extraordinary presentations and treatment resistance.
Mean platelet volume (MPV) is an indicator of platelet (Plt) function and activation. The purpose of this study is to demonstrate whether MPV and MPV/Plt ratio, indicators of Plt activation, are increased in migraine patients, compared to tension-type headache (TTH) and healthy control groups, in our large-scale study, and whether these two primary headache types with unknown pathophysiology may be differentiated by using MPV and MPV/Plt ratio.
Materials and methods
Eighty patients diagnosed with migraine as per the 2004 diagnosis criteria of the International Headache Society (IHS), 80 patients with TTH who have applied to the Neurology Clinic of our hospital, and 80 healthy subjects who have applied to the Family Practice Clinic and Blood Bank were enrolled in this study. MPV and MPV/Plt ratio in both patients of similar age and gender who were diagnosed with migraine as well as in the TTH group and healthy control group were compared.
The MPV/Plt ratio was 0.046±0.008 in migraine patients, 0.037±0.009 in patients with TTH, and 0.036±0.009 in the healthy control group. This difference was statistically significant (p<0.05). The cutoff value of MPV/Plt ratio for differentiating migraine and TTH was 0.037. The sensitivity of this value was 92.5%, while specificity was 55%.
We have demonstrated that the most commonly confused primary headache types may be differentiated by using MPV and MPV/Plt ratio, and therefore, the disability caused by migraine and unsuitable, unnecessary, and long-term drug therapies may be avoided in patients.
Katarzyna Zych-Krekora, Michał Krekora, Marek Kopala and Maria Respondek-Libserska
Introduction: APVS (Absent Pulmonary Valve Syndrome) is a rare congenital heart disease. Its incidence according to The Polish National Registry of Fetal Cardiac Pathology in years 2004 - 2016 was 0.6%.This disease is caused by the absence or the residual pulmonary artery valve resulting in significant dilation of the pulmonary trunk and its branches. In utero deaths are reported. After birth the major problem is respiratory failure and high preoperative and postoperative mortality. Material and methods: In 1995 to 2016, 11 fetuses with APVS were diagnosed in our unit, at the average 27,5 weeks of gestation (min. 18.5- max 37.1 weeks of gestation). Two groups were analysed in this series of cases: “Old one” by 2011 (n = 6) and “New one” since 2011 (n = 5) and perinatal care as well as survival were compared. We analysed the fetal echo results, perinatal care including transplacental digoxin and steroids treatment in NEW group, the longevity of the pregnancy and neonatal/infants outcome. Results: In Old group the average day of cardiac surgery was day 91st after birth (max. 161) and the survival was 50% . In the New group the average surgery day was 41st day and the postoperative survival was 60%, however there was no statistical significance ( p > 0,05). Conclusions: There is no single parameter from prenatal life in foetuses with APVS which may allow to predict the positive outcome meaning neonatal survival. However optimal perinatal care (early detection of defect, transplacental digoxin at least 3-4 weeks, steroids, no preterm delivery, on-time delivery, postnatal care in tertiary center) and relatively early cardiac surgery may have combined impact on the improvement of survival after prenatal diagnosis of APVS, however more data are necessary to prove this hypothesis.
Heart defects which includes narrowing of aortic isthmus - aortic coarctation (CoA) are one of the most prevalent birth defects. Making a correct prenatal diagnosis of CoA is very difficult and problematic. We are still observing many false (+) and false (-) diagnoses. In presenting 3 cases with prenatal suspicion of CoA only one patient confirmed this defect in the postnatal life. In the fetal echocardiography inappropriate dimensions of great vessels and PA/Ao ratio are very relevant in the CoA diagnostics. Based on such suspicion before delivery we can select a group in which birth in the tertiary center, prostin infusion, control ECHO examinations and planned cardiac surgery will be needed. But wide differential diagnosis including pulmonary dilatation (due to pulmonary hypertension or fetal blood redistribution due to possible infection) is required.
Maria Respondek-Liberska, Jerzy Węgrzynowski, Przemysław Oszukowski, Ewa Gulczyńska, Elżbieta Nykiel, Lucjusz Jakubowski, Mariusz Grzesiak, Ewa Czichos and Hanna Romanowicz
This is a case report about very rare findings in 2nd half of pregnancy (after normal 1 trimester scan ) at 18th week of gestation fetal macrosomia was detected unrelated to maternal diabetes, and acceleration fetal growth later on with unusual cardiac abnormalities (fetal cardiomegaly, cardiomyopathy, partial abnormal venous connection ). Progressive features of congestive heart failure with polyhydramnios in a fetus with estimated 5500 g predicted a poor outcome and severe neonatal condition, which was presented and discussed with the parents to be. Casearean section was performed at 33rd weeks of gestation due to maternal dyscomfort, severe legs edema and her tachypnoe. Baby boy was delivered with birth weight of 5050g, Apgar 4 with mutiple tumors. Conservative care was introduced and neonated died on the 3rd day. Differential diagnosis was discussed with special attention to Costello syndrome however without proved by genetic make-up from neonatal blood.
Iwona Strzelecka, Eliza Michalska, Katarzyna Zych-Krekora and Maria Respondek-Liberska
Introduction: Echocardiography of the fetal heart is an ultrasound examination that allows the evaluation of the anatomical structure and cardiovascular system usually performed in first half of pregnancy. Material and methods: This work presents the data of 107 fetuses with normal heart anatomy (NHA) and normal heart study (NHS) and their neonatal follow-up. In this group (in an addition to routine prenatal work-up) has been performed also a echocardiography examination in the third trimester of pregnancy, after 37th week of gestation: 61% of pregnant women were referred to the prenatal cardiology center due to the presence of high-risk pregnancies and 39% were low-risk pregnancies. Results: In two cases episodes of fetal arrhythmias were present during obstetrical examinations. After birth in the study group of 107 fetuses, 72% of newborns left the hospital during the 4 days and 28% newborns stayed in the hospital for longer time. In 16 cases their stay was extended due to maternal reasons and in 14 for newborns reasons. Conclusions: 1) In the neonate group, after prenatal cardiac evaluation > the 37th week of gestation such as “ normal fetal heart anatomy & normal heart study”, all newborns in our center were born in good general condition. 2) Late prenatal echocardiography in 3rd trimester of pregnancy maybe considered as additional tool to prove fetal well being, specially in high risk pregnancies.
Maria Respondek-Liberska, Maciej Łukaszewski, Adam Oleś, Michał Podgórski, Piotr Grzelak and Maciej Słodki
Introduction: Fetal echocardiography is a method of choice for diagnosing cardiovascular anomalies prenatally. However, in the majority of cases, the complexity of a defect creates a diagnostic challenge. Moreover, postnatal validation of sonographic findings rarely can be obtained. Nevertheless, the feedback is vital for improving diagnostic capabilities. Thus, the aim of this research was to compare results of prenatal echocardiography with postnatal angio-CT in patients with anomalies of great vessels. Material and methods: We retrospectively compared results of prenatal echocardiography and postnatal angio-CT in 10 patients with selected anomalies of the aortic arch. This was a qualitative analysis, thus discrepancies in recognized anomalies were compared between these two modalities. Results: In 8/10 patient diagnoses were fully consistent. Nevertheless, the tiny caliber of vessels created a diagnostic challenge (e.x. to differentiate the hypoplastic aortic arch from the aortic arch interruption). In the remaining case, the discrepancy was due to a problem with complete visualization of all branches of the aortic arch in prenatal ultrasound. Conclusions: Fetal echocardiography in tertiary center was a reliable method for assessment of great vessels anomalies. However, critically narrow vessels remain a diagnostic challenge and neonatal angio-CT seems to be the method of choice in cases of diagnostic doubts.
Vein of Galen Malformation (VGM) it is the most common cerebral arteriovenus malformation in fetuses and children. Usually VGM causes volume overload of the heart and can induce mass effect in the brain, causing progressive neurological impairment. Modern treatment allow on earlier therapy (before the 5th monthof life). This gives the newborn with isolated VGM and heart failure a chance of survival. This work presents case of isolated vein of Galen malformation with diagnosed cardiomegaly, monophasic flow through the tricuspid valve, large right heart, pericardiac effusion, dilatation of SVC and abnormal doppler exam. Postnatal management included aggressive medical treatment of cardiac failure and early transarterial embolization of the selected vessels feeding the aneurysm at 6 days of age. The newborn is 6 month old, growing normally without neurological deficits or developmental retardation and waiting of next stage therapy. New techniques of treatment give fetuses with VGM and prenatal signs of cardiac insufficiency a chance of survival and healthy life, in a group of selected cases. The analysis of the cardiovascular system in fetuses with VGM according to uniform criteria is essential in order to assess the influence of these malformations on the survivability of newborns.