A fatal case of non-Hodgkin lymphoma - a case report

Въведение: Неходжкиновият лимфом (НХЛ) е хетерогенно заболяване, произхождащо от малигнената трансформация на лимфоцити и включващо множество подтипове, всеки със специфични молекулярни и клинични характеристики. Клиничната манифестация варира от вяло протичане до агресивен ход. Решенията за лечение за предпочитане се правят в мултидисциплинарна среда. През последните десетилетия се наблюдава нарастване на случаите, дължащо се на увеличаване на средната възраст на населението, подобряване на диагностиката, пандемията от ХИВ и имуносупресивната терапия. Материали и методи: Дискутира се клиничен случай на 65-годишна починала пациентка с Неходжкинов лимфом. Тя е диагностицирана през април 2017 г. с клинично проявени двустранно увеличени цервикални лимфни възли, астено-адинамичен синдром, замайване и абдоминална болка. Първоначално тя е приета в МБАЛ-Силистра, където е открита бицитопения (анемия и тромбоцитопения), поради което е препратена към УМБАЛ „Св. Марина` във Варна. Флоуцитометричното изследване на периферна кръв де-монстрира патологична B-клетъчна популация. Резултати и обсъждане: CT сканирането на гръдния кош, корема и таза показа генерализирана лимфаденопатия, синдром на горна празна вена и значително уголемена слезка с лацерация, пенетрация на хематома през капсулата и малка колекция кръв в малкия таз. Поради ниския брой на тромбоцитите тя е оценена като високорисков хирургичен пациент. По тази причина успешно е извършена емболизация на лиеналната артерия. През август 2017 г. се появява загуба на апетит, коремна болка, повръщане, запек и анурия, което довежда до приемане по спешност в болница. Пациентката остава анурична въпреки проведената хидратация и лечение, изпада в кома и настъпва екзитус леталис. Заключение: Прогнозата на пациентите с НХЛ зависи главно от хистологичния клас, разпространението, възрастта на пациента и от това дали болестта е разпространена в екстранодални органи. Наличието на тези прогностични фактори в дискутирания случай може да обясни изхода на пациента.Introduction: Non-Hodgkin lymphoma (NHL) is a heterogeneous disease resulting from the malignant transformation of lymphocytes and includes multiple subtypes, each with specific molecular and clinical characteristics. The clinical presentation ranges from an indolent to an aggressive nature. Treatment decisions are preferably made in a multidisciplinary setting. The last decades have seen a growth due to increased mean age of the population, improvements in diagnosis, the HIV pandemic, and immunosuppressive therapy. Materials and Methods: A case report of a 65-year-old deceased female patient with a non-Hodgkin lymphoma is discussed. She was diagnosed in April 2017 with clinical manifestation of bilaterally enlarged cervical lymph nodes, astheno-adynamic syndrome, dizziness and abdominal pain. She was initially admitted at MHAT-Silistra, where they discovered bicytopenia (anemia and thrombocytopenia) due to which she was referred to Sv. Marina University Hospital in Varna. The flow cytometric analysis of peripheral blood demonstrated a pathological B-cell population. Results and Discussion: CT scan of chest, abdomen and pelvis showed generalized lymphadenopathy, superior vena cava syndrome and significantly enlarged spleen with laceration, penetration of the haematoma through the capsule and a small collection of blood in the lower abdomen. Due to low platelet count she was evaluated as a high-risk surgical patient. A splenic arterial embolization was successfully per formed. In August 2017, she developed loss of appetite, abdominal pain, vomiting, constipation and anuria, which required urgent hospital admission. The patient remained anuric despite proper hydration and treatment, became comatose and died. Conclusion: The prognosis of NHL patients depends mainly on the histological grade, dissemination, patient`s age and whether the disease spreads in extranodal organs. The presence of these prognostic factors in the discussed case may explain the outcome for this patient

The role of CT and MRI in the diagnosis and management of pilomyxoid astrocytoma with spontaneous intratumoral hemorrhage - a case report

Въведение: Около половината от мозъчните тумори в детска възраст са астроцитоми. Пиломиксоидният астроцитом е агресивен вариант на пилоцитния астроцитом. Най-често тези тумори са добре отграничени, неинфилтриращи околната тъкан. Материали и методи: Представяме случай на пиломиксоиден астроцитом в детска възраст, придружен със спонтанно интратуморно кървене. Дете на 3-годишна възраст беше прието в спешното отделение на УМБАЛ „Св. Марина` в гр. Варна по повод нестабилна хемодинамика, повишена телесна температура, повръщане и загуба на съзнание. При неврологичния преглед се установи, че зениците са двустранно нереаги-ращи на светлина. По Глазгоу кома скалата пациентът получи 6 точки. От извършеното предоперативното компютърно томографско (КТ) изследване се установи наличие на интрацеребрален хематом, причиняващ вътрешна хидроцефалия и дилатация на латералните вентрикули. Бе проведено спешно оперативно лечение с поставяне на интравентрикуларен дрен, последвано от провеждане на магнитнорезонансна томография (МРТ) на глава. МРТ доказа наличието на голяма ту-морна формация с размери 49х55 мм, разположена интра- и супраселарно. Бе извършена кранио томия и парциална екстирпация на тумора чрез използване на микрохирургична техника. Резултатът от хистологичното изследване доказа морфологичната картина на пиломиксоиден астроцитом WHO grade II. Резултати и обсъждане: В ранния постоперативен период пациентът беше хемодинамично стабилен. От проведеното постоперативно КТ изследване се установи персистираща интравентрикуларна хеморагия, въпреки наличието на интравентрикуларни дренове. Във фронталните дялове бе забелязана хиподенсна лезия с вид на исхемична. Поради липсата на подобрение в неврологичния статус, както и наличието на резидуална туморна маса, бе предложено продължаване на лечението. Заключение: Тъй като липсват специфична за този вид тумор клинична изява, най-често първите симптоми са свързани с повишаване на интракраниалното налягане и компресията на паренхима, които се предизвикват от мас-ефекта и локализацията на тумора.Introduction: About half of childhood brain tumors are astrocytomas. Pilomyxoid astrocytomas are an aggressive variant of pilocytic astrocytoma. Generally, these tumors are well circumscribed without infiltration. Materials and Methods: We report a case of pilomyxoid astrocytoma associated with tumoral bleeding in a pediatric patient. A 3-year-old girl presented in the Emergency Department of St.Marina University Hospital in Varna with unstable haemodinamics, fever, vomiting and loss of consciousness. The neurological examination revealed non-reactive to light pupils. The patient was assessed to have a Glasgow Coma Scale (GCS) score of 6. The preoperative CT scan showed an intracerebral haematoma, causing hydrocephalus and dilatation of the lateral ventricles. After an insertion of an external ventricular drain, a MRI scan was performed. It revealed a giant intra- and suprasellar tumor with dimentions of 49x55 mm. The patient underwent craniotomy and microsurgical partial extirpation of the tumor. After a histological examination was performed, the tumor was considered pilomyxoid astrocytoma WHO grade II. Results and Discussion: In the early postoperative period, the patient was haemodinamically stable. The postoperative CT scan demonstrated a persisting intraventricular haemorrhage, despite the inserted external ventricular drains. A hypodense lesion in the right frontal lobe consistent with subacute infarction was noticed. Due to the lack of neurological improvement and the presence of a residual tumor (confirmed by the CT scan), a subsequent surgery was necessary. Conclusion: There is no characteristic clinical presentation and the symptoms will be related to the mass effect and the tumor location, usually symptoms of raised intracranial pressure or parenchymal compression

The capabilities of ultrasound - from standard echocardiography to Transcranial Doppler in the diagnostics of atrial septal defects

Въведение: Ултразвукът е част от арсенала на лекари и радиолози повече от 50 години, но неговите възможности и днес продължават да нарастват чрез въвеждането на все по-нови методики в диагностиката на редица патологии. Една от тях са междупредсърдните септални дефекти (ASD) и макар стандартната трансторакална ехокардиография (ТТЕ) да е доказала ефективността и ползата си във времето, днес на разположение на специалистите съществува нов и също толкова надежден диагностичен метод - транскраниалната доплер ехография (cTCD). Материали и методи: Докладът разглежда нарастващите възможности на ехографията в диагностиката на АSD. Установено е, че около 25% от световното население има персистиращ форамен овале (ПФО). Мнозинството от тези хора нямат симптоми, свързани с това състояние. Въпреки това в последните години се предлага становището, че пациентите, преживели криптогенни инсулти или транзиторни исхемични атаки (TИА), имат по-висока честота на ПФО. cTCD е много чувствителна методика, която може да бъде използвана за оценката на дясно-леви шънтове (RLS) през ПФО. За диагностиката на ASD се използва транстемпорален изглед, при което се изобразява средната мозъчна артерия (СМА). Методиката се състои в интравенозна апликация на предварително приготвен физиологичен разтвор, като при поява на микромехурчета в зоната на СМА поставя с голяма сигурност диагнозата ASD. Резултати и обсъждане: Редица проучвания доказаха, че при голям процент от пациентите, преживели инсулти или ТИА, е налице такъв дефект, a специфичността и точността на метода са съизмерими с тези на ТТЕ и трансезофагеалната ехокардиография (ТЕЕ), която се приема за златен стандарт. Заключение: cTCD има сходна чувствителност и специфичност с тази на cTEE при откриването на ASD, но има предимството да е неинвазивен и доказано безопасен при пациенти с емболичен инсулт от неизяснен произход. В обобщение, достъпността до високосенситивни диагностични техники, каквато е TCD, осигурява възможност за ефективен и надежден скрининг на пациенти със съответните показания.Introduction: Ultrasound has been a part of the arsenal of medical specialists for more than 50 years but its uses keep growing with the invention of newer methods in diagnostics of different pathologies. One of them are the atrial septal defects (ASD) and even though the standard transthoracic echocardiography (TTE) has proven its efficacy, there is nowadays, available to professionals, a new and equally reliable diagnostic method - transcranial Doppler ultrasonog-raphy (cTCD). Materials and Methods: This report reviews the growing capabilities of ultrasound in ASD diagnostics. It is estimated that approximately 25% of the world`s population have patent foramen ovale (PFO). The majority of these people have no symptoms associated with this condition. However, in recent years, it has been suggested that patients with cryptogenic strokes or transient ischaemic attacks (TIA) have higher prevalence of PFO. cTCD is a very sensitive technique which can be used for evaluation of right-to-left shunts (RLS) via PFO. In the diagnostics of ASD transtemporal view is used and the middle cerebral artery (MCA) is visualized. The method is performed by intravenous application of saline and the appearance of microbubbles in the MCA determines with high certainty the diagnosis of ASD. Results and Discussion: Numerous studies have shown that in large percentage of the patients with strokes or TIA such defect is present and the specificity and accuracy of the method are comparable to those of TTE and transesophageal echocardiography (TEE), which is considered a standard method for detecting RLS. Conclusion: cTCD has similar sensitivity and specificity to cTEE in detecting ASD but has the advantage of being noninvasive and reportedly safe in patients with embolic stroke of undetermined source. In summary, the availability of high sensitive diagnostic techniques such as TCD provides the opportunity of effective and reliable screening of patients with appropriate indications

Oncolytic virotherapy - can a poliovirus cure cancer?

IntroductionOncolytic virotherapy is a promising form of gene therapy for cancer, using already existing “natural” agents - viruses. Tumor resistance to a lot of drugs after intensive chemotherapy is considered to be the main obstacle in curing cancer patients. Recent studies have shown that oncolytic viruses demonstrate a potential for effective treatment in a variety of cancer patients.AimThe aim of this work is to investigate the possible curative effect of the oncolytic poliovirus (PVS-RIPO) in patients with glioblastoma multiforme. Material and methodsPVS-RIPO is a genetically engineered polio virus which is infused directly into the patient`s tumor. Once inside the tumor, PVS-RIPO infects and kills tumor cells. PVS-RIPO consists of a genetically modified non-pathogenic version of the oral poliovirus Sabin type 1. Although this tumor cell killing alone may have tumor-fighting results, the likely key to therapy with PVS-RIPO is its ability to recruit the patients’ immune response against the cancer.ResultsThe researchers have already achieved remarkable results in trials on animals, and may be on the verge of a completely new treatment for people. Currently, Phase I clinical trials of PVS-RIPO against glioblastoma are ongoing. For now this treatment is showing promising results by extending the life expectancy of patients with recurrent glioblastoma multiforme.ConclusionEverything in the world of cancer treatment develops extremely fast these days. The key to better cancer treatment is a better understanding of the disease and the mechanisms that may work to fight it

Congenital anomalies of the kidney and the urinary tract - leading cause for chronic kidney disease in children - a case report

Introduction: The spectrum of congenital anomalies of the kidneys and the urinary tract is extremely broad and ranges from mild, asymptomatic malformations to severe, life-threatening ones. In young children congenital anomalies are the leading cause for kidney failure and for kidney transplantation or dialysis. Prenatal sonography has facilitated the detection of urological abnormalities presenting with hydronephrosis. The goal of management is preservation of renal function through mitigation of the risk for recurrent urinary tract infections (UTI) and/or obstruction.Material and methods: A case report of a 14-year-old male patient is discussed. He is diagnosed at the age of two months with congenital bilateral grade 2 hydronephrosis and megaureters, caused by ob­structive uropathy - Marion`s disease (congenital bladder neck sclerosis).Results: The diagnosis is confirmed via abdominal ultrasound, intravenous urography and comput­ed tomography (CT). At four months the patient is consulted with a pediatric urologist in Universi­ty Hospital for Emergency Medicine `N.I. Pirogov`, where corrective surgery is performed. He is ad­mitted multiple times at the Pediatric urology department in Sofia and at the Pediatric department in University Hospital `St. Marina` in Varna due to UTIs and an exacerbation of his kidney disease. The patient underwent five surgeries after the diagnosis in accordance with the severity of his anom­aly. The imaging studies demonstrated grade 1 hydronephrosis with improvement of urinary tract drainage. During the periodical follow-ups his renal laboratory test results indicated chronic kidney disease stage 2.Conclusion: Congenital anomalies of the kidney and urinary tract are the main cause for chronic kid­ney disease in pediatric patients. Advances in prenatal diagnostics are essential for early detection and diagnosis, requiring a multidisciplinary approach in management and prevention of this condi­tion

The place of Ivabradine in management of chronic heart failure patient with implanted cardiac resynchronization therapy - case report

Introduction: Heart failure is a syndrome characterized by typical symptoms (e.g. dyspnoea, pretibial oedema, fatigue) caused by structural and/or functional cardiac abnormalities, resulting in reduced cardiac output and/or elevated intracardiac pressures at rest or during stress. It is end-stage of all car­diovascular diseases and due to its wide spread may easily be described as an epidemic of 21st century.Materials and methods: We present a case of 52-year-old male with a history of coronary artery dis­ease who underwent percutaneous coronary intervention due to chest pain with implantation of 2 drug-eluting stents. In 2016 after suffering anterior myocardial infarction he was hospitalized again and another PCI of stent thrombosis of the proximal LAD was performed. During the patient`s hos­pital stay an ejection fraction of 30% and left bundle branch block were established and he devel­oped signs of total HF. The combination of low EF and LBBB lead to the implantation of CRT-D. On 03.2017 he was urgently admitted to II. Cardiology Clinic in UMHAT `St. Marina` with progressive symptoms of CHF. After a correction of therapy and optimization of the CRT-D under ultrasound control the patient had subjective improvement and reduction of swelling. Due to lack of adequate heart rate control after correction of CRT parameters and optimal dose of beta-blocker, Ivabradine was added to patients therapy.Results: The following check-ups showed significant improvement, no symptoms of exacerbated HF, no readmissions due to the condition and NYHA class II-III.Conclusion: Management of HF patients with CRT should follow the same algorithm as in all other HF patients. In cases of sinus rhythm and HR>70/min Ivabradine is a drug of choice for HR control which also improves physical capacity and quality of life. However, despite the availability of numer­ous therapy methods that increase the life expectancy of these patients, the prognosis remains poor

Hemolytic-uremic syndrome - case report

Introduction: Hemolytic-uremic syndrome (HUS) is a common cause of community-acquired acute kidney injury in young children. It is characterized by the triad of microangiopathic hemolytic ane­mia, thrombocytopenia, and renal insufficiency. The syndrome predominantly occurs in infants and children after prodromal diarrhea. HUS is classified into two main categories, depending on whether it is associated with Shiga-like toxin (Stx) or not and has clinical features in common with thrombot­ic thrombocytopenic purpura (TTP), rendering the differential diagnosis difficult.Materials and Methods: A case report of a 2-year-old child is discussed. Two days prior to hospital­ization the patient had diarrhea, vomiting and a sudden onset of weakness and lethargy. The physical examination revealed generalized edema, lack of rashes and no neurological deficit.Results: The laboratory tests showed normocytic normochromic anemia (hemoglobin 59 g/l), throm­bocytopenia (27Ñ…10^9), elevations in serum blood, urea, nitrogen and creatinine. The results from the peripheral blood smear came back with anisocytosis, poikilocytosis and schistocytosis. Urinal­ysis showed proteinuria, leukocyturia and hematuria. Abdominal ultrasonography detected bilater­al hyperechoic renal parenchyma, as well as the presence of free fluid in the pelvis,. Immediate an­tibiotic treatment, infusion therapy and hemotransfusion were initiated. An infusion of furosemide was administered.Conclusion: With early recognition and intensive supportive care, the mortality for diarrhea-asso­ciated HUS is <5%. Although most of the patients recover renal function completely, there are risks of chronification of the disease. HUS is the most common cause of acute kidney injury in children. Knowing clinical symptomatology and course of action allows for a timely diagnosis and accurate treatment

Assessment of Right-to-Left Shunts Using Contrast Transcranial Doppler Ultrasound - Advantages and Reliability of the Method

Introduction: It is estimated that 25-30% of the world`s population has patent foramen ovale (PFO). The majority of these people have no problems associated with this condition. However, in recent years, it has been suggested that patients with cryptogenic strokes or transient ischemic attacks (TIA) have a higher prevalence of PFO. Contrast transcranial Doppler (cTCD) ultrasonography is a very sensitive, noninvasive, portable technique which can be used for evaluation of right-to-left shunt (RLS) via a PFO.Materials and Methods: In this study, a group of 19 patients, 9 males and 10 females, at a mean age 45±2 with cryptogenic ischаemic stroke were investigated by cTCD of both middle cerebral arteries (MCA). The technique is performed with intravenous application of 10 mL agitated saline without and subsequently with the Valsalva maneuver (VM).  The appearance of bubbles in the MCA is detected, thereby suggesting the diagnosis of RLS. Magnetic resonance imaging (MRI) of the brain was performed.Results: In approximately 58% of the evaluated patients microembolic signals (MES) were detected in MCA suggesting existence of RLS, which was confirmed by transesophageal echocardiography (TEE). In 7 patients, MRI revealed multiple ischaemic lesions.Conclusion: PFO appears more often in patients with cryptogenic stroke than in the general population. The cTCD technique has a similar sensitivity and specificity as cTEE, which is considered a standard method for detecting RLS. But while routine TEE application is often limited in patients with acute stroke because of acute illness, coagulopathy, and lack of patient cooperation, TCD is noninvasive and reportedly safe in patients with embolic stroke of undetermined source (ESUS). In summary, the availability of simple highly sensitive diagnostic techniques such as TEE and TCD for RLS assessment seems to supply a large amount of data on the relevance in the treatment of this condition and both methods must be considered complementary

Combined Autogenous and Alloplastic Cranioplasty in a 15-Year-Old Trauma Patient - a Case Report

Introduction:The skull integrity is of utmost importance for the permanent brain protection. Posttraumatic skull defects are disfiguring and potentially hazardous for the underlying brain especially when they are extensive and when they are in children. Cranioplasty represents a classical surgical procedure to repair large skull defects. Nowadays, cranioplasty remains a challenging surgical intervention even for experienced neurosurgeons, particularly when concerning the reconstruction of spacious skull defects in the cosmetically important frontotemporal regions of the child skull.MaterialsandMethods:We present a 15-year-old female patient with a bone fracture оf the skull caused by direct trauma due to a traffic accident. CT examination of the head showed polyfragmented fracture in the right frontotemporal region with underlying brain contusion. Craniotomy and extirpation of the impressed bone fragments were performed. Autogenous and alloplastic cranioplasty materials were used for total reconstruction of the skull defect. The patient was followed-up clinically and radiologically. Glasgow Outcome Scale (GOS) scores were assessed for 3 months postoperatively.Results:The duration of the cranial reconstruction procedure was 4 hours. Clinically in the postoperative period the patient didn`t develop any neurological deficits and the postoperative CT examination demonstrated excellent cosmetic effect. The patient received antibiotic prophylaxis up to 5 days after surgery. GOS result was 5 at the 3-month follow-up.Conclusion:The data suggests that the combined аutogenous and аlloplastic сranioplasty is a safe and effective treatment option for polyfragmented skull fractures in children. The total cranial reconstruction in children must always consider the eventual forthcoming growth of the skull

Case of a Stage IIIB Hodgkin`s Lymphoma Patient Treated with Brentuximab - Modern Concepts for Immunotherapy

Introduction: Hodgkin`s Lymphoma (HL) is a malignant lymphoma with distinct histology, biologic behavior, and clinical characteristics. The neoplastic Reed-Sternberg cell is mostly of B-cell origin, derived from lymph node germinal centers but incapable of producing antibodies. Although it is potentially curable, some patients remain primary resistant to standard chemotherapy and appear to be a serious therapeutic challenge. The emerging new agents, however, give new perspectives in the treatment of HL.Materials and Methods: A case report of a 57-year-old man with nodular sclerosis HL is discussed. He was diagnosed in 2012. Seven cycles of chemotherapy by BEACOPP were prescribed as first line treatment with subsequent assessment of the tumor response. Because of the resistant disease, he received four cycles of ICE, four cycles of DHAP, four cycles of IGEV as salvage therapy, followed by mobilization of peripheral stem cells, high-dose chemotherapy BEAM and autologous stem cell transplantation (ASCT). His PET/CT still remained positive after ASCT.Brentuximab vedotin (BV) is an antibody-drug conjugate, consisting of chimeric IgG1 antibody cAC10, specifically targeting human CD30, expressed on Reed-Sternberg cells, and microtubule disrupting agent, monomethyl auristatin E. It is indicated as consolidation therapy following ASCT in patients at high risk of relapse.The human IgG4 anti-PD-1 monoclonal antibody Nivolumab, a checkpoint inhibitor approved in May 2016, is used for resistant HL, relapsed or progressed after ASCT and post-transplantation BV.A survey of the literature was conducted regarding the currently available information about the above-mentioned novel agents and their mechanism of action.Results: After ASCT, the patient was prescribed Brentuximab vedotin as a consolidation therapy and the result of the evaluation by PET/CT after four cycles shows for the first time a complete remission.Conclusion: The concept for cell biology and antibody-based immunotherapy is a modern heuristic approach for discovering innovative therapeutics against resistant HL