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Open access

Suleimman Al-Sweedan, Mona Alassiri, Ghaida Aljamal, Rafat Jafri, Amal Alseraihy and Khawar Siddiqui

Abstract

In MDS, the bone marrow produces abnormal, immature blood cells called blast cells. Imprecise, in half of pediatric MDS, blast count is normal. A retrospective observational study was conducted to review the outcome of our HCT in pediatric patients with MDS. Record of 35 MDS patients after BMT, 1993-2016, were reviewed. Median age at transplant was 4 yrs (0.8-14.8) and median time to transplant from diagnosis 8.1 (2.3-102.5) months. TRM was 17.1% (6); [low risk (LR) = 5 (19.2%) and high risk (HR) = 1 (11.1%)] MDS group succumbed within first 100 days. The rest were fully engrafted; [low risk = 21 (72.4%) and high risk = 8 (27.6%)]. Primary and secondary graft failure was observed in one patient each (2.9%). VOD was seen in 2 patients (5.7%) and 5 (14.3%) had hemorrhagic cystitis. With a median follow-up of 112.4 months and 12 events of mortality, 3-years OS was 68.1% ± 8.0%. No significant risk factor including age, time to transplant, disease risk group, gender, conditioning regimen, source of stem cells, or a GvHD through uni- or multi-variable analyses were found to be associated with OS. Bu/Cy/±ATG conditioning regimen showed a trend of superiority for OS and EFS in our small series. The relapse incidence in our cohort was 11.5% in LR MDS.

Open access

Karolina Kaźmierczak-Siedlecka, Agnieszka Piekarska, Ewelina Lubieniecka-Archutowska, Marcin Bicz, Marcin Folwarski, Wojciech Makarewicz and Jan Maciej Zaucha

Streszczenie

Niedożywienie jest istotnym problemem dotykającym chorych poddanych transplantacji komórek krwiotwórczych (HCT). W okresie wczesnym po HCT, powikłania ze strony przewodu pokarmowego po postępowaniu przygotowawczym prowadzą do zmniejszonej podaży pokarmu i zaburzeń wchłaniania. Nasileniu niedożywienia sprzyja znacznie zwiększony katabolizm oraz rozwój choroby przeszczep przeciw gospodarzowi. Prowadzenie optymalnego leczenia żywieniowego pozostaje istotnym elementem opieki potransplantacyjnej. W pracy przedstawiono znaczenia regularnej oceny stanu odżywienia biorców HCT oraz omówiono dostępne metody leczenia żywieniowego. Żywienie doustne, które należy kontynuować tak długo jak jest to możliwe, można uzupełnić o preparaty wysokobiałkowe i wysokokaloryczne. Jeżeli żywienie doustne nie jest możliwe lub nie pokrywa zapotrzebowanie białkowo-kalorycznego, można rozważyć żywienie dojelitowe, jednak w praktyce klinicznej najczęściej stosowane jest żywienie pozajelitowe. W leczeniu żywieniowym chorych po HCT zastosowanie mogą znaleźć składniki immunomodulujące, w tym glutamina i kwasy tłuszczowe omega-3, oraz prebiotyki i probiotyki, które mogą korzystnie wpłynąć na skład mikrobiomu jelit.

Open access

Marcelina Kaleta, Joanna Zawitkowska, Jerzy R. Kowalczyk and Tomasz Olcha

Abstract

Wielolekowa chemioterapia stanowi podstawę współczesnych metod leczenia nowotworów układu krwiotwórczego u dzieci. Stosując poszczególne grupy leków, należy mieć na uwadze ewentualne ich skutki uboczne, m.in. zaburzenia gastroenterologiczne. Prezentujemy przypadki kliniczne dwóch pacjentów z ostrą białaczką limfoblastyczną, leczonych w Klinice Hematologii, Onkologii i Transplantologii Dziecięcej w Lublinie, u których powikłaniem chemioterapii była perforacja jelit. W obydwu przypadkach w trakcie leczenia indukcyjnego wystąpiły silne bóle brzucha. W badaniu fizykalnym stwierdzono wzdęcie brzucha, brak perystaltyki, wypuk bębenkowy, a w wykonanym zdjęciu radiologicznym jamy brzusznej cechy niedrożności oraz perforacji jelit. W trybie pilnym wykonano zabieg chirurgiczny. Zarówno operacja, jak i czas rekonwalescencji były przyczyną długich przerw w chemioterapii, co mogło wpływać na rokowanie. Dotychczas nie zostały opracowane algorytmy dotyczące postępowania w przypadku perforacji u pediatrycznych pacjentów onkologicznych. Szeroko rozumiana profilaktyka stanowi niekwestionowaną rolę w zapobieganiu działaniom niepożądanym leków.

Open access

Łukasz Klasa, Alicja Sadowska-Klasa, Agnieszka Piekarska, Magdalena Dutka, Maria Bieniaszewska, Dariusz Wydra and Jan Maciej Zaucha

Streszczenie

Transplantacja komórek krwiotwórczych (hematopoietic cell transplantation – HCT ) jest ugruntowaną metodą leczenia zarówno nienowotworowych, jak i rozrostowych chorób układu krwiotwórczego. U kobiet wiąże się jednak z występowaniem powikłań wczesnych i późnych dotyczących układu moczowo-płciowego. Konsekwencją gonadotoksycznego postępowania przygotowawczego (chemioterapii i radioterapii) jest przedwczesne wygaśnięcie funkcji jajników. U biorczyń allogenicznych przeszczepów (allogeneic HCT, allo-HCT) dodatkowo występują powikłania związane z występowaniem przewlekłej choroby przeczep przeciw gospodarzowi (chronic graft-versus-host disease – cGvHD) w istotny sposób upośledzające jakość życia kobiet w przypadku zajęcia strefy anogenitalnej (GvHDgyn). Dodatkowo przewlekła immunosupresja sprzyja występowaniu wtórnych nowotworów układu moczowo-płciowego. Pacjentki po HCT, szczególnie obciążone cGvHD, wymagają długoletniej interdyscyplinarnej opieki, włączając opiekę ginekologiczną. W pracy przedstawiono problemy ginekologiczne pacjentek po HCT oraz zaproponowano schemat standardu opieki ginekologicznej po transplantacji na podstawie piśmiennictwa i własnych doświadczeń.

Open access

Agnieszka Szymczyk, Aleksandra Nowaczyńska, Maciej Korpysz, Helena Donica, Agnieszka Bojarska-Junak, Monika Długosz-Danecka, Waldemar Tomczak, Ewa Wąsik-Szczepanek and Iwona Hus

Abstract

Introduction

κ and λ serum free light chains (sFLCs) are produced during physiological lymphopoesis by plasmocytes and B lymphocytes in a constant ratio related to heavy chains. The measurement of sFLC plays an important role in the diagnosis and monitoring of patients with multiple myeloma (MM). The first reports suggested that sFLC disturbances might have prognostic value also in patients with chronic lymphocytic leukemia (CLL). Aim of the study: The aim of the study was to evaluate the relationship between sFLC concentration and recognized prognostic factors and clinical course of CLL. Materials and methods: The sFLC concentration was measured using a latex-enhanced immunoassay in 59 patients with newly diagnosed CLL. The relationship between sFLC concentration and time to start of the treatment (TFT), the response rate to therapy (ORR) and overall survival (OS) was assessed.

Results

A significant correlation was found between sFLC κ concentration and the clinical stage of leukemia according to Rai classification, β-2 microglobulin concentration, LDH activity, CD38 expression, as well as between sFLC λlevel and β-2 microglobulin concentration and platelet count (PLT ). There was also a correlation between the values of summated κ and λ and the clinical stage of disease according to Rai classification, β-2 microglobulin concentration, CD38 expression, white blood cells count (WBC), lymphocyte count (ALC) and hemoglobin (Hgb) concentration. The κ/λ ratio (FCLR) values were significantly different in the CD38+ and CD38- population.

Summary

Simple and reproducible clonality index, which constitutes the sFLC concentration assessment, can be an attractive, potential prognostic marker in patients with CLL, however further studies are needed on a larger group of patients especially in relation to the predictive value of sFLC.

Open access

Edyta Klimczak-Jajor, Joanna Skulimowska, Anna Ejduk, Katarzyna Guz, Małgorzata Uhrynowska and Ewa Brojer

Abstract

Background

This report presents a case of an adult Polish women of Caucasian origin who was heterozygous for the nondeletional mutation: Hb Handsworth (HBA2 or HBA1: c.55G > C, p.Gly19Arg) and deletional (-α3.7) α-thalassemia mutation.

Methods

The HbA 2 and HbF levels were measured by microcolumn chromatography and alkaline denaturation procedure, respectively, while electrophoresis was used to detect pathological hemoglobin fraction. The β- and α-globin genotypes were determined by DNA sequencing, gap-polymerase chain reaction, α gene triplication and MLPA.

Results

The HbA2 and HbF levels were normal, but hemoglobin electrophoresis on agarose gel alkaline pH showed a strong band migration in a position of hemoglobin S and faint bands in the neighborhood of band A on acid electrophoresis. Molecular analysis of the alpha globin cluster detected a point mutation at codon 19 in HBA2 (c.55G > C, p.Gl- y19Arg) and deletion -α3.7.

Conclusions

Our compound heterozygosity does not produce severe clinical or hematological symptoms but it is important to say that in our part of Europe such cases do appear. Molecular analysis of the alpha globin cluster is required for correct diagnosis in patients with normal HbA2 levels. Compound heterozygosity was unmasked by molecular diagnosis only.

Open access

Teresa Iwaniec, Joanna Zdziarska and Artur Jurczyszyn

Abstract

Multiple myeloma (MM) is a rare malignancy, characterized by clonal proliferation of plasma cells, secreting monoclonal immunoglobulin. It is usually diagnosed based on histopathologic and immunophenotypic bone marrow examination. Abnormal results of screening coagulation tests, including prothrombin time, activated partial thromboplastin time and thrombin time, are commonly encountered in patients with plasma cell neoplasms. They do not, however, reflect bleeding tendency. We describe a 71-year-old patient who was accidentally diagnosed with multiple myeloma during coagulation diagnostics.

Open access

Savaş Yılmaz, Özgür Emiroğlu, Sadi Aksu, Sercan Başkurt and Nazmi Polat

Abstract

The relationships between otolith size and body length of North African catfish Clarias gariepinus (Burchell, 1822), collected from Sakaryabaşı (the main spring area of the Sakarya River) between July 2014 and November 2014, were examined. Length-weight relationship was also calculated for overall specimens. The wild fish were captured by electrofishing using SAMUS 725MP. A total of 132 individuals ranging from 13.6 to 59.0 cm in total length and from 15.9 to 1755.0 g in weight were sampled. The lagenar otolith (asteriscus) length and height, and the utricular otolith (lapillus) length and width were recorded for each fish. The relationships between otolith measurements and fish somatic growth were described by a non-linear function (power model). The t test revealed no considerable differences in these relationships between left and right otoliths. The ANCOVA test on the relationships between total length and otolith variables did not show significant differences between females and males. All relationships were highly significant and the mean percent prediction errors were less than 7%. The results showed that otolith growth reflected body growth.

Open access

Antonia Kurtela and Nenad Antolović

Abstract

A global problem of today is the large amount of waste in the seas and oceans, primarily plastic waste. It is estimated that every year 1.25 to 2.41 million tons of plastic material is being carried by rivers into the seas and oceans. Waste is a major problem for marine organisms, causing entanglement, choking, strangulation, malnutrition and death. In 1972 the problems caused by microplastics, particles smaller than 5 mm, were first observed. Such particles bind pathogenic microorganisms on to their surface. Increasing quantities of microplastics have been found in the stomachs of fish, and also in shellfish that feed by filtering sea water. Ingested by marine organisms, such plastics may eventually pass through the food web and can end up ingested by humans. In addition, plastic releases chemical compounds whose effect on marine organisms and humans has still not been studied. Many international and state organizations offer solutions through recycling plastic waste, as well as reducing the production of plastic materials and informing the public about the problem.

Open access

Azubuike Victor Chukwuka, Adedeji Hameed Adebowale and Oloyede Adeyemi Adekolurejo

Abstract

The relationship between morphological traits and feeding ecology of the only two tilapiine cichlids (Sarotherodon galilaeus and Oreochromis niloticus) in Lake Geriyo, northeastern Nigeria, was examined. Stomach contents of 504 individuals were examined and analyzed to relate morphological differences of each species to its dietary preferences. Eleven ecological relevant morphological variables, including total length, standard length, head length, body depth, eye diameter, pectoral spine length, pelvic spine length, dorsal fin ray count, dorsal spine count and anal fin ray count, were also measured and subjected to Principal coordinate analysis (PCoA) to relate morphological differences of each species to its dietary preferences. The PCoA (using Euclidean distance) revealed a large morphological distance between species indicating adaptation for different spatial and vertical distributions within the lake. Furthermore, the correlation of these morphological differences with particular diet categories suggests a potential for specialized trophic tendencies, however, the high occurrence of two major food types (macrophytes and plankton) in stomach contents of both species indicate generalist trophic tendencies. While a high trophic overlap index (0.98) was recorded for both species, the trophic niche breadth was higher for S. galilaeus (4.18±0.32), compared to O. niloticus (3.33±0.24). Despite the large morphological differences of both tilapias, the high trophic overlaps indicate limited food options within Lake Geriyo. In addition, the successful coexistence of the tilapias in the face of limited food resources indicates suitable partitioning of food resources in the lake.