Prehranska obravnava bolnikov s hematološko maligno boleznijo

Pri hematoloških bolnikih obstaja veliko tveganje za razvoj podhranjenosti in s prehranjenostjo povezanih motenj (sarkopenija, krhkost). Prevalenca podhranjenosti se giblje med 30 % in 50 %. Zmanjšani vnos hrane, malabsorpcija hranil ter presnovne spremembe vodijo v slabše prehransko stanje oz. v podhranjenost bolnika, kar poveča tveganje za zaplete, okužbe, podaljša čas hospitalizacije, poveča stroške zdravljenja ter vpliva na slabše delovanje vseh organskih sistemov v telesu, slabšo kakovost življenja in slabši izid zdravljenja. Tega smo se vse bolj začeli zavedati na Kliničnem oddelku za hematologijo (KOH), UKC Ljubljana, kjer smo leta 2020 pričeli izvajati sofinancirani triletni program »Implementacija klinične poti prehranske podpore na terciarni ravni«, ki smo ga odobrili na javnem razpisu Ministrstva za zdravje (MZ). Z zaposlitvijo kliničnega dietetika na oddelku smo uvedli redno prehransko obravnavo bolnikov in oblikovati dokument klinične poti prehranske podpore. Klinični dietetik v obdobju od maja 2020 do junija 2022 opravil 1.593 individualnih prehranskih obravnav, od tega 377 prvih pregledov in 1.216 kontrolnih pregledov. Začeli smo tudi ambulantno obravnavo in izvedli 16 pregledov. Ob vsakem bolnišničnem zdravljenju bi bolnik moral imeti možnost zgodnjega prepoznavanja prehranske ogroženosti in dostopnost do zgodnjih prehranskih ukrepov, saj gre za ključne postopke preprečevanja velikih izgub puste mišične mase, za ohranjanje sposobnosti bolnika za izvajanje vsakodnevnih aktivnosti in za ohranjanje kakovosti življenja. Pomembno je, da je klinična pot prehranske obravnave dobro vpeljana in se izvaja na vseh oddelkih, saj le tako bolnikom lahko zagotovimo standardizirano, pravočasno, individualno prilagojeno ter multidisciplinarno obravnavo

Concurrent acquired inhibitors to factor VIII and IX, a laboratory artifact: a case report.

Acquired inhibitors to coagulation factors other than factor VIII are extremely rare. We describe a case of a 59-year-old woman with abnormal bleeding, diagnosed with concurrent inhibitor antibodies to factor VIII and IX by Bethesda testing. We demonstrate that anti-FVIII antibodies of a very high titre are capable of disturbing the aPTT-based Bethesda assay, resulting in falsely-positive antibodies to factor IX. The case also illustrates the usefulness of the immunological assay (ELISA) in complementing the inhibitor diagnosis

Obravnava bolnikov z neklonsko eritrocitozo

Eritrocitoza je stanje povečane skupne mase eritrocitov, ki se pojavi zaradi zelo heterogenih vzrokov. Bolniki so lahko brez simptomov ali pa imajo simptome in znake povečane viskoznosti krvi. Pri obravnavi bolnika z eritrocitozo uporabljamo diagnostični algoritem, ki omogoča opredelitev vzroka eritrocitoze in ustreznega zdravljenja. V prvem koraku potrjujemo absolutno eritrocitozo s koncentracijo hemoglobina (Hb) > 185 g/L in/ali hematokritom (Ht) > 0,52 za moške ter s Hb > 165 g/L in/ali Ht > 0,48 za ženske. V drugem koraku hkrati izključujemo pravo policitemijo in iščemo sekundarne pridobljene vzroke eritrocitoze, kot so bolezni pljuč, srca, ledvic, tumorji z neustreznim izločanjem eritropoetina. Omeniti velja, da je po smernicah Svetovne zdravstvene organizacije (SZO) diagnosticiranje prave policitemije ob ustrezni klinični sliki določeno že pri nižjih vrednostih, natančneje ob Hb > 165 g/L ali Ht > 0,49 za moške ter Hb > 160 g/L ali Ht > 0,48 za ženske. V tretjem koraku bolnike, ki nam jih ni uspelo opredeliti kljub natančnim diagnostičnim preiskavam, napotimo na genetsko testiranje za opredelitev prirojene eritrocitoze. Ko izključimo pravo policitemijo, sekundarno pridobljeno in prirojeno eritrocitozo, ostane skupina oseb s t. i. idiopatsko eritrocitozo. Priporočeno zdravljenje je odvisno od vzroka eritrocitoze, najpogosteje pa vključuje jemanje acetilsalicilne kisline in ustrezno zniževanje hematokrita z venepunkcijami ob rednih kontrolah krvne slike

Slovenian recommendations for the management of chronic myeloid leukaemia

The paper presents recommendations for the management of patients with chronic myeloid leukaemia. Clinical manifestations, diagnostics, treatment and monitoring of treatment are presented

Progression of coronary calcium burden and carotid stiffness in patients with essential thrombocythemia associated with JAK2 V617F mutation

Background and aims: Patients with myeloproliferative neoplasms often succumb to cardiovascular events, but little is known on the early stages of their vascular disease. We studied how patients with JAK2 V617F positive essential thrombocythemia (ET) without overt atherosclerotic disease differed from control subjects in the progression of carotid artery stiffness and preclinical atherosclerosis. Methods: Thirty-six patients with JAK2 V617F positive ET and 38 age-, gender- and Framingham coronary heart disease (CHD) -matched control subjects were examined twice within 4 years. Clinical and laboratory testing, echo-tracking ultrasound of carotid arteries, coronary calcium measurement and digital plethysmography were performed (ClinTrials.gov NCT03828422). Results: Coronary calcium correlated with the Framingham CHD risk score at the first examination in the control group (r$_s$ = 0.410), but not among the ET patients (r$_s$ = 0.116). Both groups progressed in coronary calcium, but the outliers were more prominent among ET patients. Carotid artery stiffness increased with time in the ET patients much more than in the control group: the increase in β-index 1.95 (SD 2.18) vs. 0.22 (SD 1.99), p < 0.001, and the increase in carotid pulse wave velocity 0.72 (SD 0.92) vs. 0.08 (SD 0.72) m/s, p = 0.001. There was no correlation between carotid stiffness and Framingham CHD risk in either group. Digital endothelial function did not change. Conclusion: Carotid artery stiffness progressed faster in patients with JAK2 V617F positive ET than in control subjects. Coronary calcium correlated with the Framingham CHD risk only in control subjects. This indicates that JAK2 V617F positive ET acted as a non-classical risk factor for vascular disease

Is it possible to predict clonal thrombocytosis in triple-negative patients with isolated thrombocytosis based only on clinical or blood findings?

JAK2, MPL, and CALR mutations define clonal thrombocytosis in about 90% of patients with sustained isolated thrombocytosis. In the remainder of patients (triple-negative patients) diagnosing clonal thrombocytosis is especially difficult due to the different underlying conditions and possible inconclusive bone marrow biopsy results. The ability to predict patients with sustained isolated thrombocytosis with a potential clonal origin has a prognostic value and warrants further examination. The aim of our study was to define a non-invasive clinical or blood parameter that could help predict clonal thrombocytosis in triple-negative patients. We studied 237 JAK2 V617-negative patients who were diagnosed with isolated thrombocytosis and referred to the haematology service. Sixteen routine clinical and blood parameters were included in the logistic regression model which was used to predict the type of thrombocytosis (reactive/clonal). Platelet count and lactate dehydrogenase (LDH) were the only statistically significant predictors of clonal thrombocytosis. The platelet count threshold for the most accurate prediction of clonal or reactive thrombocytosis was 449 × 10$^9$/L. Other tested clinical and blood parameters were not statistically significant predictors of clonal thrombocytosis. The level of LDH was significantly higher in CALR-positive patients compared to CALR-negative patients. We did not identify any new clinical or blood parameters that could distinguish clonal from reactive thrombocytosis. When diagnosing clonal thrombocytosis triple-negative patients are most likely to be misdiagnosed. Treatment in patients with suspected triple-negative clonal thrombocytosis should not be delayed if cardiovascular risk factors or pregnancy coexist, even in the absence of firm diagnostic criteria. In those cases the approach “better treat more than less” should be followed

Expert opinion on current and future prophylaxis therapies aimed at improving protection for people with hemophilia A

The next frontier in hemophilia A management has arrived. However, questions remain regarding the broader applicability of new and emerging hemophilia A therapies, such as the long-term safety and efficacy of non-factor therapies and optimal regimens for individual patients. With an ever-evolving clinical landscape, it is imperative for physicians to understand how available and future hemophilia A therapies could potentially be integrated into real-life clinical practice to improve patient outcomes. Against this background, nine hemophilia experts from Central European countries participated in a pre-advisory board meeting survey. The survey comprised 11 multiple-choice questions about current treatment practices and future factor and non-factor replacement therapies. The survey questions were developed to reflect current unmet needs in hemophilia management reflected in the literature. The experts also took part in a follow-up advisory board meeting to discuss the most important unmet needs for hemophilia management as well as the pre-meeting survey results. All experts highlighted the challenge of maintaining optimal trough levels with prophylaxis as their most pressing concern. Targeting trough levels of ≥30–50 IU/L or even higher to achieve less bleeding was highlighted as their preferred strategy. However, the experts had an equal opinion on how this could be achieved (i.e., more efficacious non-factor therapies or factor therapy offering broader personalization possibilities such as targeting trough levels to individual pharmacokinetic data). In summary, our study favors personalized prophylaxis to individual pharmacokinetic data rather than a "one-size-fits-all" approach to hemophilia A management to maintain optimal trough levels for individual patients