Intraventricular treatment of secondary central nervous system lymphoma – Case study and literature overview

Secondary nervous system lymphoma (SCNSL) is a rare extranodal form of non-Hodgkin lymphoma (NHL). This applies to a particular form of lymphoma that does not originally derive from the central nervous system (CNS); it can be both an isolated form of relapse or a systemic part of disease progression. Due to poor prognosis and a lack of established algorithms of therapeutic procedures, it is a big challenge for physicians from many specializations. In our study, we present an interesting case of a patient with a relapsed form of SCNSL for whom a unique form of treatment was used – intraventricular administration of rituximab and methotrexate

Haemophagocytic lymphohistiocytosis: case series. Serum ferritin level as an indicator of treatment effectiveness

Introduction: Haemophagocytic lymphohistiocytosis (HLH) is a medical condition associated with over-activation of the immune system. HLH results from the inactivity of NK cells and uncontrolled activity of cytotoxic T lymphocytes and macrophages with the massive cytokine response.[1] Cytohistological examinations might show hemophagocytes in different tissues (e.g., bone marrow). Exceptionally high level of ferritin is often observed in the laboratory findings. Due to aggressive course of the disease mortality rate is extremely high. Material and methods: We presented three patients with acquired form of HLH treated successfully in Heaematooncology Department of Medical University of Lublin from September 2018 to April 2021. Results: In the first patient, HLH developed during pregnancy. Patient 2 was first hospitalized in the Intensive Care Unit (ICU) and 10 therapeutic plasma exchanges (TPE) were carried out. In both patients, stabilization -of ferritin levels and remission of the disease were achieved soon after the application of the treatment according to the HLH-2004 protocol. Case 3 presents a patient in whom HLH was induced by EBV and SARS CoV-2 infections. In the first stage of the treatment, the patient received drugs according to the HLH-2004 protocol, but the serum ferritin did not normalize. The reinfection with SARS CoV-2 virus occurred again in the course of the treatment. Due to the disease resistance and inability to continue the HLH-2004 protocol, it was decided to start ruxolitinib therapy, which resulted in stabilization of the serum ferritin and improvement of the general condition. The patient was qualified for allogeneic bone marrow transplantation. Conclusions: HLH is a difficult and interdisciplinary diagnostic and therapeutic problem. It is essential to popularize knowledge about fast and targeted diagnostics. Among laboratory finding, the ferritin concentration seemed to be particularly helpful as a predictor of treatment effectiveness. Proper diagnosis and treatment implemented as early as possible could save patients’ lives.Introduction: Haemophagocytic lymphohistiocytosis (HLH) is a medical condition associated with the over-activation of the immune system. HLH results from the inactivity of natural killer cells and uncontrolled activity of cytotoxic T lymphocytes and macrophages, with a massive cytokine response. Cytohistological examinations might show haemophagocytes in different tissues (e.g., bone marrow). Among laboratory findings especially(particularly) high ferritin level is often seen. Due to the aggressive course of the disease mortality rate is extremely high. Case reports: The study presented three patients with an acquired form of HLH treated successfully in the Haemato-Oncology Department Medical University of Lublin from September 2018 to April 2021. In case 1 HLH developed during pregnancy. Patient 2 was first hospitalized in the Intensive Care Unit and 10 therapeutic plasma exchanges were carried out. In both patients, stabilization of ferritin levels and remission of the disease were achieved soon after the application of treatment according to the HLH-2004 protocol. Case 3 presents a patient in whom HLH was induced by Epstein-Bárr virus and severe acute respiratory syndrome coronavirus type SARS-CoV-2 infections. In the first stage of treatment, the patient received drugs according to the HLH-2004 protocol, but the serum ferritin did not normalize. The reinfection with (SARS-CoV-2) virus occurred again during the treatment. Due to the disease resistance and the inability to continue the HLH-2004 protocol, it was decided to start ruxolitinib therapy, which resulted in the stabilization of the serum ferritin and improvement of the general condition. The patient was qualified for allogeneic bone marrow transplantation. Conclusions: HLH is a difficult and interdisciplinary diagnostic and treatment problem. It is necessary to popularize knowledge about fast and targeted diagnostics. Among laboratory finding the ferritin concentration seemed to be especially helpful as a predictor of treatment effectiveness. Proper diagnosis and treatment introduced as early as possible could save patients’ life

JAK2 mutation status, hemostatic risk factors and thrombophilic factors in essential thrombocythemia (ET) patients

The recently discovered JAK2 V617F point mutation, found in 50–60% of ET patients, has been reported to be associated with a higher risk of thrombotic events. In this study, we explored if JAK2 V617F mutation, or coexisting thrombophilic and hemostatic risk factors, contributed to these complications. We examined 32 patients with ET, and looked for pathogenetic JAK2 V617F mutation and prothrombotic genes mutations: factor V Leiden, prothrombin and MTHFR. We also evaluated plasma levels of fibrinogen, factors VIII and XII, AT, protein C, protein S and serum level of homocysteine. Urokinase concentration was assessed in patients’ plasma as well as platelet lysates. There was no difference in the number of thrombotic complications between ET patients with and without JAK2 mutation. However, we found a number of thrombophilic and hemostatic risk factors that could contribute to thrombotic complications in ET patients. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 2, pp. 267–271

Secondary malignancy in patients with essential thrombocythemia – case reports

Nadpłytkowość samoistna (ET; essential thrombocythemia) należy do przewlekłych chorób mieloproliferacyjnych i ma na ogół mało agresywny przebieg, jednak czas przeżycia chorych jest krótszy w porównaniu z ogólną populacją. Do niedawna za główne przyczyny skrócenia czasu życia chorych uznawano zaawansowany wiek i przebyte epizody zakrzepowe. Obecnie coraz więcej uwagi zwraca się na inny powód, jakim jest rozwój u tych chorych wtórnych nowotworów niehematologicznych.ET is a relatively indolent disease, but a shorter survival of patients has been observed. Previously the main risk factors for reduced survival time were advanced age and the history of thrombotic complications. Recently the other cause of reduced survival is put on – secondary nonhematological neoplasms

Mild hyperhomocysteinemia in patients with essential thrombocythemia (ET) and its relation with MTHFR gene mutation and folic acid concentration

In this study we assessed homocysteine level in 106 patients with ET – 80 females and 26 males, mean age 54 (23–82) and in 20 healthy persons – 6 males and 14 females, mean age 41 (31–54). We also searched for a relation between homocysteine level and MTHFR gene mutation as well as vitamin B12 and folic acid concentration. Median homocysteine serum level was higher in ET patients than in control group. Elevated homocysteine level primarily stems from folic acid deficiency rather than from the presence of MTHFR gene mutation. Median folic acid level was lower in ET patients presenting thrombotic and bleeding complications than in ET patient without vascular episodes. We concluded that folic acid substitution may not only prevent hyperhomocysteinemia but also the development of vascular complications in ET patients

Limfohistiocytoza hemofagocytarna — opis serii przypadków. Stężenie ferrytyny w surowicy jako wskaźnik skuteczności leczenia

Introduction: Haemophagocytic lymphohistiocytosis (HLH) is a medical condition associated with the over-activation of the immune system. HLH results from the inactivity of natural killer cells and uncontrolled activity of cytotoxic T lymphocytes and macrophages, with a massive cytokine response. Cytohistological examinations might show haemophagocytes in different tissues (e.g., bone marrow). Among laboratory findings especially(particularly) high ferritin level is often seen. Due to the aggressive course of the disease mortality rate is extremely high. Case report: The study presented three patients with an acquired form of HLH treated successfully in the Haemato-Oncology Department from September 2018 to April 2021. In case 1 HLH developed during pregnancy. Patient 2 was first hospitalized in the Intensive Care Unit and 10 therapeutic plasma exchanges were carried out. In both patients, stabilization of ferritin levels and remission of the disease were achieved soon after the application of treatment according to the HLH-2004 protocol. Case 3 presents a patient in whom HLH was induced by Epstein-Bárr virus and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections. In the first stage of treatment, the patient received drugs according to the HLH-2004 protocol, but the serum ferritin did not normalize. The reinfection with SARS CoV-2 occurred again during the treatment. Due to the disease resistance and the inability to continue the HLH-2004 protocol, it was decided to start ruxolitinib therapy, which resulted in the stabilization of the serum ferritin and improvement of the general condition. The patient was qualified for allogeneic bone marrow transplantation. Conclusions: HLH is a difficult and interdisciplinary diagnostic and treatment problem. It is necessary to popularize knowledge about fast and targeted diagnostics. Among laboratory finding the ferritin concentration seemed to be especially helpful as a predictor of treatment effectiveness. Proper diagnosis and treatment introduced as early as possible could save patients’ life.Wprowadzenie: Limfohistiocytoza hemofagocytarna (HLH) jest chorobą związaną z nadmierną aktywacją układu odpornościowego. Charakteryzuje się brakiem aktywności komórek naturalnej toksyczności i niekontrolowaną aktywnością cytotoksycznych limfocytów T i makrofagów, z masywną odpowiedzią cytokinową. Badanie cytologiczne może wykazać obecność hemofagocytów w różnych tkankach (np. szpiku). W badaniach laboratoryjnych często obserwuje się wyjątkowo duże stężenie ferrytyny. Ze względu na agresywny przebieg choroby śmiertelność jest niezwykle wysoka. Opis przypadku: Przedstawiono opis 3 pacjentów z nabytą postacią HLH leczonych z powodzeniem w klinice hematoonkologii i transplantacji szpiku od września 2018 do kwietnia 2021 roku. W 1 przypadku HLH rozwinęła się w czasie ciąży. Pacjenta 2. początkowo hospitalizowano na oddziale intensywnej terapii, gdzie przeprowadzono 10 zabiegów plazmaferezy. U obojga chorych wkrótce po zastosowaniu leczenia według protokołu HLH-2004 uzyskano stabilizację stężenia ferrytyny i remisję choroby. U 3. pacjenta HLH wywołało zakażenie wirusem Epsteina-Bárr i zespołu ostrej niewydolności oddechowej 2 (SARS-CoV-2). W pierwszym etapie leczenia pacjent otrzymywał leki zgodnie z protokołem HLH-2004, ale stężenie ferrytyny w surowicy nie ulegało normalizacji. W trakcie leczenia doszło do ponownego zakażenia SARS-CoV-2. Ze względu na oporność na leczenie i brak możliwości kontynuacji terapii według protokołu HLH-2004 zdecydowano o rozpoczęciu leczenia ruksolitynibem, co spowodowało stabilizację stężenia ferrytyny w surowicy i poprawę stanu ogólnego. Pacjenta zakwalifikowano do allogenicznego przeszczepienia szpiku. Podsumowanie: Limfohistiocytoza hemofagocytarna to trudny i interdyscyplinarny problem diagnostyczno-leczniczy. Konieczne jest poszerzanie wiedzy dotyczącej konieczności szybkiej i ukierunkowanej diagnostyki. Stężenie ferrytyny wydawało się szczególnie pomocnym parametrem laboratoryjnym, będącym czynnikiem predykcyjnym skuteczności leczenia. Właściwa diagnostyka i jak najwcześniejsze leczenie może uratować życie pacjenta

Testing Selected Personal Protection Items of Firefighters in Combined Conditions of Mechanical Loads and Temperatures Occurring during Gas LNG Leaks

The article examined selected individual protection used during rescue and fire-extinguishing activities. Fire helmet and special shoes were examined under conditions resembling the operational conditions of LNG gases. The assessment of the equipment consumption was based on strength tests. The main threats come from thermal and mechanical factors. Therefore, firefighters must properly protect their head and legs. At −80 °C, the energy of the impact force of the beater in the headache was, 12.4 J and 15.1 J for points P1 and P2, respectively. The studies showed that cooling the chamber to −80 °C adversely affected the structure of the fire helmet exposed to impact Dynamic. Research work was carried out as part of the Research and Development project No. DOB-BIO9/15/02/2018

Gaucher disease diagnosed after bone marrow trephine biopsy — a report of two cases

The hematologist is at the forefront of specialists to whom patients with Gaucher disease present because of cytopenia and hepatosplenomegaly. Usually, patients with such symptoms have undergone trephine biopsy. We present the cases of two patients in whom Gaucher disease was suspected because of the discovery of Gaucher cells in trephine biopsy, and subsequently confirmed via enzymatic and molecular investigations. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 2, pp. 352–356

Real-World Efficacy of Midostaurin in Aggressive Systemic Mastocytosis

In April 2017 midostaurin was approved by the US Food and Drug Administration for the treatment of patients with aggressive systemic mastocytosis (ASM). So far, very limited real world data on its efficacy is available. Thirteen patients aged from 48 to 79 years, who received midostaurin in the early access program, were included in the study. Midostaurin was used both in first (n = 5) and subsequent lines of treatment (n = 8). The median duration of exposure was 9 months. Most patients (77%, n = 10) had a clinical improvement already as soon as the second month of therapy. Objective response was noted in 4 (50%) of eight evaluated patients. Among responders, we observed a decrease in serum tryptase level (median 74.14%) and bone marrow infiltration by mast cells (median 50%) in the sixth month of treatment. In one case, in the 10th month of treatment, allogenic stem cell transplantation was performed, achieving complete remission. Five patients died, three due to progression of disease, one in the course of secondary acute myeloid leukemia and one due to reasons not related to mastocytosis. Treatment is ongoing in seven patients. We found that midostaurin therapy is beneficial to patients with ASM