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

Lymphoplasmacytic lymphoma relapse presenting as isolated multifocal subcutaneous adipose tissue infiltrates on 18F-FDG PET/CT

Lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM) is an uncommon mature B cell lymphoma usually involving the bone marrow and, less commonly, the spleen and/or lymph nodes. This case presents a pathology-confirmed isolated extramedullary relapse of LPL, located in subcutaneous adipose tissue, 5 years after successful treatment of WM

Severe COVID-19 Infection Management in a Patient with Mild Haemophilia—A Case Report

Patients with haemophilia present a significant challenge when admitted into the intensive care unit. To prevent haemorrhagic complications related to the infection or due to invasive procedures factor (F) VIII/IX must be substituted. As thromboembolic complications are frequent among critically ill COVID-19 patients, thromboprophylaxis is also applied to patients with haemophilia. This requires careful monitoring of FVIII/IX activity as well as other haemostatic parameters, such as D-dimer and antiXa. We describe a 44-year old patient with mild haemophilia A (FVIII activity of 6%), who required a prolonged intensive care unit stay due to a severe SARS-CoV-2 infection. FVIII was substituted via boluses, and dalteparin was given according to recommendations. The patient successfully recovered from the disease

Severe COVID-19 Infection Management in a Patient with Mild Haemophilia—A Case Report

Patients with haemophilia present a significant challenge when admitted into the intensive care unit. To prevent haemorrhagic complications related to the infection or due to invasive procedures factor (F) VIII/IX must be substituted. As thromboembolic complications are frequent among critically ill COVID-19 patients, thromboprophylaxis is also applied to patients with haemophilia. This requires careful monitoring of FVIII/IX activity as well as other haemostatic parameters, such as D-dimer and antiXa. We describe a 44-year old patient with mild haemophilia A (FVIII activity of 6%), who required a prolonged intensive care unit stay due to a severe SARS-CoV-2 infection. FVIII was substituted via boluses, and dalteparin was given according to recommendations. The patient successfully recovered from the 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

National reccomendations for the management of patients with haemophilia

The document presents recommendations for the comprehensive treatment of patients with haemophilia in Slovenia. It enables health workers at all three levels of health care to become well-acquainted with all the possible aspects of treatment based on the best practices of major centres worldwide, and on the studies and experience of health professionals at the National Haemophilia Centre, University Medical Center Ljubljana. The document contains definitions, treatment algorithms and lists of medications with their characteristics and appropriate dosages. It specifically defines indications for the exclusive competence of the tertiary level in Ljubljana due to the actual availability of teams and laboratory options. It also contains an extensive list of the literature on haemophilia