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Szende Jakab, Erzsébet Lázár, István Benedek, Judit Beáta Köpeczi, Annamária Pakucs and István Benedek

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.

Open access

István Benedek, Erzsébet Lázár, Johanna Sándor-Kéri, Szilárd Bíró, Szende Jakab and István Benedek

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.

Open access

Enikő Kakucs, I Benedek, Erzsébet Benedek, Judit Beáta Köpeczi, Aliz Tunyogi and Monica Istrati

Abstract

Introduction: Autologous haemopoietic stem cell transplantation (SCT) is an important treatment modality for patients with acute myeloid leukemia with low and intermediate risk disease. It has served advantages over allogenic transplantation, because it does not need a matched donor, there is no graft versus host disease, there are less complications and a faster immune reconstitution than in the allo-setting. The disadvantage is the lack of the graft versus leukaemia effect.

Materials and methods: In the Bone Marrow Transplantation Unit Tîrgu Mureș 14 patients with acute myeloid leukemia received an autologous SCT. Mobilization of the stem cells was performed using chemotherapy and granulocytic colony stimulating factor. The conditioning regimen for SCT consists in monotherapy with busulfan (Bu) 16 mg/kg, BuCy: busulfan in combination with Cyclophosphamide (CY) 120 mg/ kg or BuMel: Busulfan in association with Melphalan (Mel) 140 mg/m2.

Results: The median patient age was 36 years (range 20-55), 9 (64%) were males and 5 (36) were females and the median time interval from diagnosis to autologous SCT was 9 months (range 3-25). All the patients were transplanted successfully, all of them achieved a sustained neutrophil count (> 0.5 G/L), median time 11 days (9-15) and platelet count (> 20 G/L) median time 14 days (10-19) after transplantation.

Conclusions: We conclude that autologous stem cell transplantation is an effective treatment in acute myeloid leukemia with the possibility of long survival, particularly in patients with standard risk disease

Open access

Judit Beáta Köpeczi, Erzsébet Benedek, Enikő Kakucs, Aliz Tunyogi, Monica Istrati and Istvan Benedek

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), recently classified among the acute myeloid leukemia and related precursor neoplasms, is a rare hematological malignancy with highly aggressive clinical course. We report a case of a 55 year-old female patient who presented spontaneous rupture of the spleen. The histopathological diagnosis without immunohistochemistry was splenic marginal zone non-Hodgkin lymphoma (NHL). The patient received 4 courses of polychemotherapy. Due to the unfavorable evolution the spleen was histopathologically reexamined and immunohistochemistry was performed in another laboratory. The diagnosis of NHL was excluded. Undifferentiated malignant cell proliferation, possible myeloid was suspected. Due to the discrepancy between diagnoses, a third immunohistological examination was performed in a third laboratory. NHL was excluded and myeloid, NK-cell or plasmacytoid dendritic cell leukemia was suspected. The patient was admitted to our clinic after 8 months from the initial diagnosis. Immunophenotyping by flowcytometry from the bone marrow showed 23% blasts positive for CD4, CD56, CD123, HLA-DR, CD38, CD11b, CD2, and negative for lineage specific markers of B-, T-lymphoid, NK- or myelomonocytic cells. The final diagnosis was BPDCN in leukemic phase. Due to the diversity of the clinical presentation, morphology and immunophenotype of BPDCN the diagnosis of this rare malignancy remains challenging. Immunophenotyping by flowcytometry is superior to immunohistochemistry because of the availability of a larger panel of antibodies and the possibility to identify the intensity of antigen expression. The atypical forms of BPDCN should be recognized early in order to manage properly the patients with this aggressive disease

Open access

Balázs Oltean-Péter, Szilamér Korodi, István Benedek, Erzsébet Lázár, Johanna Kéri, Annamária Pakucs, István Kovács, Lehel Bordi, Adriana Mitre, Imre Benedek, Theodora Benedek and István Benedek

Abstract

Recent studies demonstrated that despite restoration of the sinus rhythm, patients with a positive history of atrial fibrillation (AF) are still at risk of thromboembolic events. The primary objective of this study is to identify new imaging-derived biomarkers provided by modern imaging technologies, such as cardiac computed tomography angiography, delayed enhancement magnetic resonance imaging, or speckle tracking echocardiography, as well as hematological biomarkers, associated with the risk of intracavitary thrombosis in patients with AF, in order to identify the imaging-derived characteristics associated with an increased risk of cardioembolic events. Imaging data collected will be post-processed using advanced techniques of computational modeling, in order to fully characterize the degree of structural remodeling and the amount of atrial fibrosis. The primary endpoint of the study is represented by the rate of thromboembolic events. The rate of cardiovascular death, the rate of major adverse cardiovascular events, and the rate of AF recurrence will also be determined in relation to the degree of structural remodeling and atrial fibrosis.

Open access

István Benedek, István Benedek, Judit Beáta Köpeczi, Johanna Sándor Kéri, Annamária Pakucs, Szende Jakab and Erzsébet Lázár

Abstract

Plasma cell leukemia (PCL) is one of the most aggressive monoclonal gammopathies, being characterized by the presence of more than 20% of plasma cells in the peripheral blood and an absolute number of these cells of more than 2×109, with different morphology, from young elements to mature cells. The incidence of PCL varies between 2–4% among multiple myeloma (MM) patients. In comparison with MM, PCL appears more often in younger patients. The following article describes the case of a 49-year-old female patient diagnosed with PCL which needed urgent control of the clinical manifestations due to its irreversible complications. Urgent autologous stem cell transplantation is recommended in this group of patients.

Open access

Árpád Bzduch, István Benedek, Szilárd Bíró, Johanna Sándor-Kéri, Erzsébet Lázár and István Benedek

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.

Open access

Erzsébet Benedek Lázár, Judit Beáta Köpeczi, Aliz Beáta Tunyogi, Enikő Kakucs, Alina Cătană and I Benedek

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.

Open access

István Benedek, Erzsébet Lázár, Judit Beáta Köpeczi, István Benedek, Aliz Beáta Tunyogi, Szende Jakab and Annamária Pakucs

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.

Open access

Judit Beáta Köpeczi, I Benedek, Erzsébet Benedek, Enikő Kakucs, Aliz Tunyogi, Monica Istrati and Aranka Kurtus

Abstract

Introduction: Plasmacytoid dendritic cell leukemia is a rare subtype of acute leukemia, which has recently been established as a distinct pathologic entity that typically follows a highly aggressive clinical course in adults. The aim of this report is to present a case of plasmacytoid dendritic cell leukemia due to its rarity and difficulty to recognize and diagnose it.

Case report: We present a case of a 67 year-old man who presented multiple subcutaneous lesions on his face, neck, chest and upper extremities with reddish-brown, brown colour. In the bone marrow aspirate 83% of the blast cells were found. Immunophenotypically the blasts were positive for CD4, CD56, CD123 (high intensity), CD36, CD22, CD10 (10.42%), CD33, HLA-DR, CD7 (9.24%), CD38 (34.8%) and negative for CD13, CD64, CD14, CD16, CD15, CD11b, CD11c, CD3, CD5, CD2, CD8, CD19, CD20, CD34. The skin biopsy showed lymphohistiocytoid infiltration in the dermis. The patient was diagnosed with acute plasmacytoid dendritic cell leukemia and received polychemotherapy with rapid response of skin lesions and blastic infiltration of the bone marrow. After 3 courses of polychemotherapy the cutaneous lesions reappeared and multiplied. The blast infiltration in the bone marrow increased to 70%. A more aggressive polychemotherapy regimen was administered, but the patient presented serious complications (febrile neutropenia) and died in septic shock 8 months after the initiation of treatment.

Conclusions: Immunophenotyping of blasts cells is indispensable in the diagnosis of plasmacytoid dendritic cell leukemia. The CD4+, CD56+, lin-, CD123 ++high, CD11c-, CD36+, HLA-DR+, CD34-, CD45+ low profile is highly suggestive for pDCL. The outcome of plasmacytoid dendritic cell leukemia is poor. Despite the high rate of initial response to treatment, early relapses occur and the patients die of disease progression.