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  • Author: Erzsébet Lázár x
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Abstract

Acute myeloid leukemia (AML) is a cancerous disease affecting the myeloid line of the bone marrow cells. FLT3, also known as CD135, is a proto-oncogene, which, if mutated, leads to different types of cancer. The protein it encodes presents tyrosine-kinase activity, and its intratandem mutation, FLT3-ITD, leads to uncontrolled proliferation of myeloblasts and worse outcomes in AML patients. There are currently several pharmacological agents that can inhibit the effect of either the proteins with tyrosine-kinase activity or the mutated FLT3 gene. We present the case of a 68-year-old patient, smoker, with a history of arterial hypertension, chronic obstructive pulmonary disease, presenting with headache unresponsive to antalgics, dyspnea after physical exertion, and epistaxis, with onset 2 months prior to his presentation. The patient was diagnosed with AML with positive FTL3 mutation for which conventional induction therapy was initiated. Within the next days, the patient presented several complications related to the disease itself or caused by the treatment, which eventually led to his death.

Abstract

Multiple myeloma accounts for 10% of the hematologic malignancies and is characterized by a single clone of plasma cells producing a monoclonal protein. The aim of this review is to summarize the current treatment methods of multiple myeloma. In the last 15 years, the incidence of myeloma has increased in patients younger than 65 years, thus treatment became even more important in order to obtain a long lasting remission or plateau phase. The treatment of this disease is complex and focuses not only on increasing the patients’ survival, but also improving their quality of life.

Abstract

Hematological conditions and their treatments have an increased risk of cardiovascular events, and invasive interventions have a higher risk of periprocedural complications in this group of patients. The aim of this review was to evaluate the risk of invasive interventions in patients with hematologic disorders and to underline the role of noninvasive cardiovascular screening in patients with hematological disorders such as Hodgkin and non-Hodgkin lymphoma, anemia, hemophilia, thrombocythemia, polycythemia vera, and leukemia. Based on present knowledge in the field, our opinion is that the screening of patients with hematological diseases is very important to reduce the morbidity and mortality due to cardiovascular events. Noninvasive assessments are suitable for this purpose with a significantly lower risk compared to invasive interventions.

Abstract

Hematological conditions can lead to serious disturbances in blood rheology, being frequently associated with increased systemic inflammation and increased risk of bleeding. The imbalance between coagulation and thrombolytic factors in patients with acute coronary syndromes may lead to undesirable outcomes, and the success of emergency coronary angioplasty or by-pass grafting may be altered by increased bleeding in coagulopathies such as hemophilia. This paper intends to review the present knowledge in the field of acute coronary syndromes in subjects with hematological and onco-hematological disorders such as thrombotic thrombocytopenic purpura, immune thrombocytopenic purpura, von Willebrand disease, hemophilia, polycythemia vera, erythrocyte disorders, myelodysplastic syndrome, leukemia, lymphoma or myeloma.

Abstract

Background: High-dose chemotherapy and autologous stem cell transplantation have become a standard curative treatment in various hematologic malignancies. Many factors can affect the success of mobilization and hematopoietic stem cell harvesting.

Aim: The aim of this study was to analyze factors that lead to mobilization failure.

Material and Methods: We conducted a retrospective study on 19 patients with failure of stem cell harvesting. All patients were administered high doses of GCS-F (filgrastim, 15 μg/kg/day) and 0.24 mg/kg of plerixafor on day +5 or +10 of harvesting.

Results: The median age of the study population was 51 years (range 35–67) and 52.6% (n = 10) were males. The study group included 4 (21%) subjects with multiple myeloma, 6 (31.5%) with Hodgkin lymphoma, 8 cases (42.1%) with non-Hodgkin lymphoma and 1 patient with chronic lymphocytic leukemia. Each patient received 2.78 (range 1–5) lines of chemotherapy, administered in 11.57 (range 2 to over 20) cycles of treatment.

Conclusion: In hematologic malignancies it is very important to collect stem cells in time, in order to reduce mobilization failure. As we have shown in our studied cases, multiple lines of polychemotherapy with or without radiotherapy lead to mobilization failure.

Abstract

We present the method of immunomagnetic stem cell separation with the ISOLEX 300i device (Isolex® 300i Magnetic Cell Selection System, Nextell Therapeutics Inc. Irvine California 21618 USA) and the results obtained using this method in patients admitted to the Hematology and Bone Marrow Transplantation Clinic of Tîrgu Mureş, Romania. Cell selection has a great importance in separating stem cells from tumor cells, therefore contributing to the success of autologous stem cell transplantation.

Abstract

In Western countries, chronic lymphocytic leukemia (CLL) is one of the most diagnosed leukemia types among elderly patients. CLL is described as an indolent lymphoproliferative disorder, characterized by the presence of a high number of small, mature B-cells in the peripheral blood smear, with a particular immunophenotype (CD5, CD19, CD23 positive and CD20 dim positive) and accumulation in the bone marrow and lymphoid tissue (e.g., lymph nodes, spleen). The experience of the past decades showed that CLL is clinically very heterogeneous; while some patients present a chronic clinical evolution, with a prolonged survival, in which the treatment can be delayed, others suffer from a more aggressive form, which must be treated early and is associated with many relapses. This observation led to several genomic studies that have mapped the genetic modifications involved in the disease conformations, including del(13q14), del(11q), or trisomy 12. On the other hand, certain genetic mutations such as del(17p13)–p53, NOTCH1 mutation, or ZAP70/CD38 increased expression are associated with worse clinical outcome. In order to apply the right treatment strategy, the RAI and BINET staging systems should be considered, which are based on clinical and laboratory assessment, on genetic mutations that may influence the resistance to chemotherapy, as well as the patient’s age and comorbidities. The aim of this manuscript was to present the therapeutic approaches of CLL, in order to attempt to answer the following question: to treat, or not to treat? This clinical update focuses on the managements of CLL patients in the 21st century.

Abstract

Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder, which can involve the hematopoietic stem cell or early progenitor cells, without the loss of their capacity to differentiate. Typically, CML has three clinical phases: a chronic phase, an accelerated phase, and an aggressive transformation in blast crisis, analogous to acute leukemia. The following article presents the case of a 49-year-old patient diagnosed with Philadelphia-negative CML in blastic transformation, where after multiple conventional acute leukemia induction chemotherapy regimens an unrelated allogeneic hematopoietic stem cell transplant was performed.

Abstract

Background: There are several histologic variants and clinical subtypes of diffuse large B cell lymphoma, which includes the primary mediastinal large B cell lymphoma (PMBL). In the last 10 years the incidence of diffuse large lymphomas grew significantly.

Case report: We present the case and evolution of an aggressive life-threatening mediastinal B cell lymphoma with respiratory insufficiency, diagnosed in the 27th week of pregnancy. After 4 courses of R-CHOP the clinical status has somewhat improved, but the dyspnea, the facial and neck oedema and the trouble of speech persisted. After the patient was admitted to our hospital, she received DHAP regimen followed by mobilization with G-CSF. Before transplantation we administered another 3 courses of DHAP chemotherapy with spectacular results. We performed autologous hematopoietic stem cell transplantation preceded by BEAM chemotherapy. At present, 5 years post-transplant the patient is well, with no metabolically active disease on the PET-CT performed 3 months ago.

Conclusion: We can conclude that even in very complicated DLBCL cases, with a very good, efficient medical-team work we can salvage lives, in our case both of the mother and the child’s. Even in partially chemo-refractory cases like in the presented one, salvage chemotherapy followed by autologous transplantation can lead to a successful treatment.

Abstract

A 62 year-old caucasian male was admitted in our pulmonary hypertension expert center with initial diagnosis of pulmonary veno-occlusive disease for validation and specific treatment approach. Routine examinations revealed no apparent cause of pulmonary hypertension. Patient was referred for a thorax contrast enhanced multi-slice computed tomography which revealed extensive bilateral thrombi in pulmonary lower lobe arteries, pleading for chronic post embolic lesions. A right heart catheterization and pulmonary angiography confirmed the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). Following the local regulations, the patient underwent thrombophilia screening including molecular genetic testing, with positive findings for heterozygous for VCORK1 -1639G>A gene single nucleotide polymorphism, PAI-1 4G/5G and factor II G20210A gene. With heterozygous genetic profile of 3 mutations he has a genetic predisposition for developing a thrombophilic disease which could be involved in the etiology of CTEPH. Familial screening was extended to descendants; the unique son was tested with positive results for the same three genes. Supportive pulmonary hypertension drug therapy was initiated together with patient self-monitoring management of oral anticoagulation therapy. For optimal control of targeted anticoagulation due to a very high risk of thrombotic state the patient used a point-of-care device (CoaguChek®XS System, Roche Diagnostics) for coagulation self-monitoring.