Waldenström macroglobulinemia (WM), according to the 2017 World Health Organization classification, is defined as the co-occurrence of lymphoplasmacytic lymphoma involving the bone marrow with monoclonal gammopathy of the IgM class regardless of the concentration of monoclonal protein. It is a rare lymphoproliferative disease with distinctive clinical features. Diagnostic characteristics in WM have changed significantly with the discovery of two molecular markers: MYD88 and CXCR4. The mutational status of these markers both affects clinical presentation and has shown therapeutic implications. The choice of treatment in WM is closely dependent on the patient’s age, risk of treatment-related neuropathy, and risk of immunosuppression or secondary malignancies. The therapeutic landscape has broadened in recent years, and the approvals of ibrutinib and zanubrutinib represent a significant step forward toward better management of the disease.

This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.

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.

Eosinophilia is defined as an absolute peripheral blood eosinophil count > 0.5 GL. Most often, its intensity is mild. Eosinophilia usually accompanies other diseases such as allergic, rheumatological, infectious, or oncological. The signs depend on the underlying diseases. After starting treatment of the underlying disease, the eosinophil count returns to the reference range. The diagnosis of eosinophilia starts by excluding the secondary causes. Clonal eosinophilia is a rare disease in which eosinophils are part of a tumor clone. The clonality is confirmed by molecular biology methods. The most common eosinophilic infiltration includes skin, lungs, and heart. The symptoms are not connected with a count of eosinophils in blood or bone marrow. The manifestation can be mild but also severe, life-threatening like venous thromboembolism. This article presents a case of 36-year-old man with eosinophilia caused by Toxocara canis infection manifested by systemic symptoms, erythroderma, and liver failure.

Plasma cell myeloma (PCM) is a hematologic malignancy that derives from mature B cells. The prognosis of patients with PCM is highly dependent on the presence of cytogenetic aberrations. Determination of cytogenetic risk enables informing patients about their prognosis and allows for individual choice of therapy. In Poland, cytogenetic risk assessment is a fully reimbursed procedure, and it is recommended to perform such an examination in every diagnosed patient. Therapy of patients with high cytogenetic risk should be planned with consideration of tandem autotransplantation of hematopoietic cells in eligible patients. In patients with refractory or relapsed PCM, treatment with ixazomib in combination with lenalidomide and dexamethasone appears to remove cytogenetic risk.

Immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by an isolated platelet count of less than 100,000/μL caused by destruction and reduced production of platelets. Systemic steroid therapy is a mainstay of first-line treatment; however, additional treatment lines are usually necessary due to the very high recurrence rate after therapy completion and steroid resistance. Standard treatment options for steroid resistance or steroid dependence include splenectomy, rituximab or thrombopoietin receptor agonists. The article presents current data on the diagnosics and therapy of ITP, considering the limited access to some forms of treatment in Poland.

Ostra białaczka szpikowa (AML) to złożona choroba o dynamicznym przebiegu, związana z szeregiem nabytych, kumulujących się zmian genetycznych. W ostatnich latach dokonano znaczących postępów w zakresie zrozumienia patogenezy oraz możliwości diagnostycznych i terapeutycznych. Aktualne klasyfikacje uwzględniają zaburzenia cytogenetyczno-molekularne, wśród nich obecność między innymi mutacji w obrębie FLT3 — transbłonowej kinazy tyrozynowej, regulującej proliferację i różnicowanie się komórek krwiotwórczych na wczesnym etapie rozwoju. Mutacja FLT3 jest wykrywana w około 30% przypadków nowo rozpoznanej AML. Wyróżnia się dwa podtypy mutacji: wewnętrzną tandemową duplikację ITD (internal tandem duplication) lub mutację punktowe domeny kinazowej TKD (tyrosine kinase domain). Obecność mutacji FLT3-ITD high ratio wiąże się z niekorzystnym rokowaniem. Efekty standardowej terapii są niesatysfakcjonujące, dlatego rekomenduje się włączanie tej grupy chorych do badań klinicznych. Do nowych strategii leczenia pacjentów z AML zalicza się inhibitory kinaz tyrozynowych, wśród których wyróżnia się inhibitory I i II generacji. Midostaurynę, niespecyficzny inhibitor kinaz, jako pierwszą zarejestrowano do leczenia skojarzonego pacjentów z nowo rozpoznaną AML FLT3-dodatnią w 2017 roku. W pracy przedstawiono doświadczenia Kliniki Hematologii i Transplantacji Szpiku w Poznaniu w stosowaniu inhibitorów kinazy tyrozynowej FLT3 na podstawie opisu dwóch przypadków chorych z nowo rozpoznaną AML.

Many systemic diseases can manifest in the oral mucosa. Leukaemia is the most common neoplastic disease of white blood cells. Common symptoms of leukaemia in the oral cavity include mucosa pallor, bleeding gums, gingival enlargement, ecchymosis, oral infections and ulcerations. A dentist should know how to recognize the first signs of leukaemia and may be responsible for a prompt referral to an adequate professional to improve patient outcomes. A dentist must participate in a patient’s process of treatment when the plan includes complex therapy of the oral cavity. In many cases, this procedure enables the implementation of appropriate therapy and the possibility of recovery and can even save the patient’s life. The study aimed to present the cooperation between the dentist and haematologist in the example of a 52-year-old female with oral symptoms of leukaemia.

Chronic lymphocytic leukaemia (CLL) is the commonest B-cell malignancy and leukaemia in western countries. It mainly affects the elderly population with a median age of 72. Overall survival varies from 2 to 15 years and depends on clinical and pathological risk factors like age, clinical stage and the presence of genetic mutations with TP53 aberrations conferring the worst prognosis. Indications for therapy include active and symptomatic disease and advanced clinical stage assessed in the Rai or Binet staging system. CLL therapy has been revolutionized in the last years due to the introduction of novel agents — small molecule inhibitors interfering with signalling pathways. These agents proved to be of greater efficacy than immunochemotherapy in both untreated and relapsed patients and led to the significant improvement in survival of CLL patients. These novel agents have been incorporated into new recommendations for CLL treatment. They have also improved the survival of high-risk CLL patients with TP53 aberrations and complex karyotype although their prognosis is still inferior as compared to other patients’ subgroups. Questions concerning optimal sequencing novel agents and the timing of allogeneic bone marrow transplantation remain still open for high-risk group CLL patients.

In Poland, 800–900 new cases of classical Hodgkin's lymphoma (cHL) are diagnosed annually. Despite relatively good results of first-line treatment, 20–25% of patients relapse and the chances of their cure become much smaller. The paper presents the consensus of the Polish experts on the management of patients with relapsed and refractory cHL in 2020, taking into account generally accepted international recommendations, approved indications and the Polish reimbursement conditions. The proposed recommendations were voted by all authors. The recommendations that were agreed by the majority are presented in the paper. For each recommendation, the most important information supporting its legitimacy and information justifying the dissenting opinions of the authors of the consensus were quoted.