Differential Diagnoses of Systemic Mastocytosis in Routinely Processed Bone Marrow Biopsy Specimens: A Review

Diagnosis of systemic mastocytosis (SM) is mainly based on the morphological demonstration of compact mast cell infiltrates in various tissue sites. In almost all patients such infiltrates are detected in the bone marrow. Reliable immunohistochemical markers for the diagnosis and grading of SM have been established, but various differential diagnoses including myeloproliferative neoplasms, basophilic and eosinophilic leukemias may be very difficult to delineate. Even more challenging is the recognition of hematological neoplasms with signs of mast cell differentiation but not fulfilling diagnostic criteria for SM, especially the rare myelomastocytic leukemia. It is also important to separate the reactive state of mast cell hyperplasia from indolent variants of SM, especially those with a very low degree of bone marrow infiltration and absence of compact mast cell infiltrates. When the lymphocytic component of the SM infiltrate is very prominent, SM may be confused with an indolent lymphoma, especially lymphoplasmacytic lymphoma which almost always shows a marked reactive increase in mast cells. In aggressive and leukemic variants of SM, mast cells may be very atypical and devoid of metachromatic granules. This hypogranulation can be regarded as cellular atypia and may lead to the misdiagnosis aspect of monocytic leukemia or histiocytic neoplasm. Regarding immunohistochemical anomalies, mast cells in aggressive and leukemic SM have been found to express CD30 (Ki1-antigen). Thus, anaplastic large cell lymphoma or Hodgkin's disease may first be considered rather than SM. There is increasing evidence that most patients with long-standing adult-type urticaria pigmentosalike skin lesions have in fact indolent SM. Therefore, such skin lesions are an important clue to the correct diagnosis in these patients. However, in aggressive or leukemic SM skin lesions are usually absent and then the correct diagnosis relies on an appropriate investigation of bone marrow biopsy specimens using both SM-related immunohistochemical markers (tryptase, KIT, CD25, CD30) but also markers excluding potential differential diagnoses. Investigation for presence of the activating KIT point mutation D816V is very helpful to establish a correct diagnosis of SM in all the difficult cases exhibiting a low degree of bone marrow infiltration or puzzling morphological findings. Copyright (C) 2010 S. Karger AG, Base

Elijah - Raised in the Fire

Felix Christopher McKean Memorial Award (undergrad

Elijah - Raised in the Fire

Felix Christopher McKean Memorial Award (undergrad

Kolebanje nekih sastojaka mlijeka krava sivosmeđe i jersey pasmine u toku godine

Poznato je, da je mlijeko po svom sastavu vrlo različito kako u pogledu biološki visokovrijednih supstanca koje posjeduje, tako i s obzirom na količinu pojedinih sastavnih elemenata, koja nije konstantna, već se men ja pod utjecaj em različitih faktora. U literaturi mogu se naći podaci o sastavu mlijeka svih poznatih pasmina krava

Public Participation As An Instrument Of Performance Of Eu-Funded Cross-Border Projects

The objective of the European territorial cooperation programme INTERREG has been financing joint projects of cross-border areas ever since 1990. Since many projects, financed in the past, did not provide longterm effects, the European Union decided to introduce the “result-driven approach” to these programmes. The realization of results will be consequently monitored during the project financing and after completion of selected projects. If one wishes to ensure sustainability also after the end of financing, the public and target groups must be actively involved in preparation and implementation of projects from the very beginning. The purpose of this paper is to present possibilities of public participation in different phases of the project. A comprehensive model of public participation was developed on the basis of research, involving the public into the sustainable life-cycle of a cross-border project

KITD816V+ systemic mastocytosis associated with KITD816V+ acute erythroid leukaemia: first case report with molecular evidence for same progenitor cell derivation

Toll-like receptor (TLR)-9 recognizes CpG motifs in microbial DNA. TLR9 signalling stimulates innate antimicrobial immunity and modulates adaptive immune responses including autoimmunity against chromatin, e.g., in systemic lupus erythematosus (SLE). This review summarizes the available data for a role of TLR9 signalling in lupus and discusses the following questions that arise from these observations: 1) Is CpG-DNA/TLR9 interaction involved in infection-induced disease activity of lupus? 2) What are the risks of CpG motifs in vaccine adjuvants for lupus patients? 3) Is TLR9 signalling involved in the pathogenesis of lupus by recognizing self DNA

Privatizacija obveščevalne dejavnosti v sodobnem varnostnem okolju

Cilj prispevka je opozoriti na pojav privatizacije obveščevalne dejavnosti na varnostnem področju. Kateri so tisti ključni dejavniki, ki so pripeljali do privatizacije obveščevalne dejavnosti, kdo so tisti, ki izvajajo zasebno obveščevalno dejavnost in kako se obveščevalna dejavnost države razlikuje od obveščevalne dejavnosti zasebnih subjektov, so ključna vprašanja, s katerimi se bomo ukvarjali v tem prispevku. Analizirali bomo tudi pravno podlago za razvoj obveščevalne dejavnosti zasebnih subjektov v Republiki Sloveniji

Systemic mastocytosis with associated myeloproliferative disease and precursor B lymphoblastic leukaemia with t(13;13)(q12;q22) involving FLT3.

Systemic mastocytoses represent neoplastic proliferations of mast cells. In about 20% of cases systemic mastocytoses are accompanied by clonal haematopoietic non-mast cell-lineage disorders, most commonly myeloid neoplasms. A case of systemic mastocytosis carrying the characteristic mutation at codon 816 (D816V) in the KIT gene of mast cells, with two concurrent accompanying clonal haematopoietic non-mast cell-lineage disorders, chronic myeloproliferative disease, unclassifiable and precursor B lymphoblastic leukaemia is documented. Both accompanying clonal haematopoietic non-mast cell-lineage disorders carried the wild-type KIT gene, but had a novel t(13;13)(q12;q22) involving the FLT3 locus at 13q12. The chronic myeloproliferative disease, unclassifiable and the precursor B lymphoblastic leukaemia were cured by syngenous stem cell transplantation, but the systemic mastocytosis persisted for more than 10 years. The additional impact of molecular techniques on the correct diagnosis in haematological malignancies is highlighted, and evidence is provided that, apart from internal tandem duplications and mutations, FLT3 can be activated by translocations

Neke psihološke i pravne karakteristike korištenja neubrojivosti u obrani na suđenjima za ratne zločine u Europi

In trials against war criminals, the defendants often plead not guilty by reason of insanity in an effort to avoid assuming responsibility for the charged acts. The paper discusses the history of the insanity defence and some factors that might explain why war crimes are committed. The authors concentrate primarily on the psychological elements of insanity and the reasons for extreme violence appearing at the individual level. Persons charged with war crimes often use post-traumatic stress disorder as the basis for an insanity defence. The authors also consider insanity from the perspective of international criminal law. By explicitly and precisely defining insanity, the Rome Statute moved away from the general provisions employed by the Nuremberg Tribunal, while at the same time making a clear distinction between insanity and incapacity for trial. Insanity may be a complete defence resulting in exclusion of the criminal act and exclusion of the offender’s culpability.Na suđenjima ratnim zločincima optuženici su se često izjašnjavali da nisu krivi zbog neubrojivosti u nastojanju da izbjegnu preuzimanje odgovornosti za kaznena djela za koja su optuženi. Rad razmatra povijest korištenja neubrojivosti za obranu i neke čimbenike koji bi mogli objasniti zašto su neki ratni zločini počinjeni. Autori se usredotočuju prvenstveno na psihološke elemente neubrojivosti i razloge za ekstremno nasilje, koje se pojavljuje na individualnoj razini. Osobe koje se terete za ratne zločine često koriste posttraumatski stresni poremećaj kao osnovu za korištenje neubrojivosti u obrani. Autori također razmatraju neubrojivost iz perspektive međunarodnog kaznenog prava. Rimski statut se sa izričitim i preciznim definiranjem neubrojivosti odmaknuo od općih odredaba, upotrjebljenih na Nürnberškom sudu, u isto vrijeme čineći jasnu razliku između neubrojivosti i nesposobnosti za suđenje. Neubrojivost može činiti cjelovitu obranu, koja rezultira isključenjem kaznenog djela i isključenjem krivnje počinitelja

Određivanje količine bjelančevina u mlijeku primjenom Amidoschwarz metode

Bjelančevine mlijeka sve su važniji predmet naučnih istraživanja i diskusija. Zbog svoje visoke biološke i prehrambeno-fiziološke vrijednosti kao i zbog privrednog značaja u sve većoj mjeri privlače pažnju inostranih i domaćih stručnih institucija