Immune dysfunction in patients with end stage kidney disease; Immunosenescence - Review.

The body's defense against environmental factors is realized by physical barriers and cells of both the innate and adaptive immune systems. Patients with end stage kidney disease (ESKD), especially those treated by hemodialysis, have changes in both the function and the number or percent of different leukocyte subsets. Changes were described at the level of monocytes and lymphocyte subsets, which are associated with immunodeficiencies and pro-inflammatory status correlated with degenerative changes and increased cardiovascular risk.

Platelet Defects in Acute Myeloid Leukemia-Potential for Hemorrhagic Events.

Background And Objectives: In acute myeloid leukemia (AML), extensive bleeding is one of the most frequent causes of death. Impaired activation and aggregation processes were identified in previous studies on platelet behaviour associated with this disease. This study's aim was to examine platelet function in correlation with other haemorrhage risk factors (fever, sepsis, recent bleeding, uraemia, leucocytosis, haematocrit value, treatment).

Quantitative analyses of CD7, CD33, CD34, CD56, and CD123 within the -ITD/-MUT myeloblastic/monocytic bulk AML blastic populations.

The most frequent mutations in acute myeloid leukemia (AML) - -ITD and - are associated with a specific immunophenotype. We evaluated the levels of surface antigens in an uninvestigated AML patient population according to the combination of -ITD/ mutations. Antigen levels were calculated as the geometric mean fluorescence index (MFI) ratio between myeloblasts or monoblasts/monocytes and a negative population for the specific antigen.

FLT3-ITD DNA and mRNA levels in AML do not correlate with CD7, CD33 and CD123 expression.

Introduction: FLT3 internal tandem duplication (ITD) mutations are found in around 25% of all acute myeloid leukaemia (AML) cases and is associated with shorter disease-free and overall survival. Previous reports have shown that FLT3-ITD induces a specific phenotype in leukemic blasts, which is characterized by high levels of CD33 and CD123, and that expression of CD33 and CD123 is directly influenced by the DNA FLT3-ITD/wild-type FLT3 allelic ratio (AR).

Methods: A total of 42 FLT3-ITD and 104 FLT3-ITD-negative AML patients were analysed.

Primary myelofibrosis and pregnancy outcomes after low molecular-weight heparin administration: A case report and literature review.

Rationale: Primary myelofibrosis is encountered with the myeloproliferative diseases and is the least prevalent among women of childbearing age. The prognosis is guided by pancytopenia, leukemic transformation and thrombosis which are the dominant complications.

Patient Concerns: Data regarding protocol management during pregnancy in the context of myelofibrosis are insufficient.

2016 WHO Clinical Molecular and Pathological Criteria for Classification and Staging of Myeloproliferative Neoplasms (MPN) Caused by MPN Driver Mutations in the JAK2, MPL and CALR Genes in the Context of New 2016 WHO Classification: Prognostic and Therapeutic Implications.

The 2016 WHO-CMP classification proposal defines a broad spectrum of JAK2 V617F mutated MPN phenotypes: normocellular ET, hypercellular ET due to increased erythropoiesis (prodromal PV), hypercellular ET with megakaryocytic-granulocytic myeloproliferation and splenomegaly (EMGM or masked PV), erythrocythemic PV, early and overt classical PV, advanced PV with MF and post-PV MF. ET heterozygous for the JAK2 V617F mutation is associated with low JAK2 mutation load and normal life expectance. PV patients are hetero-homozygous versus homozygous for the JAK2 V617F mutation in their early versus advanced stages with increasing JAK2 mutation load from less than 50% to 100% and increase of MPN disease burden during life long follow-up in terms of symptomatic splenomegaly, constitutional symptoms, bone marrow hypercellularity and secondary MF.

Hemorrhagic risk due to platelet dysfunction in myelodysplastic patients, correlations with anemia severity and iron overload.

Platelet function is influenced by changes in membrane fluidity that has an important role in the expression of platelet receptors and in modulating the activity of proteins like phospholipase C or proteinkinase C. In freshly prepared platelets, membrane fluidity modifies the aggregation/agglutination function. Reactive oxygen species (ROS) represent another important parameter involved in platelet receptor activation.

Diagnostic difficulties in acute myeloid leukemia.

We present the case of a patient who presented cells with different morphologic appearance, lymphoblasts on peripheral blood smear, lymphoblasts on bone marrow aspirate and myloblasts on bone marrow biopsy, and immunophenotyping, leading to different stage diagnosis. The final diagnosis was that of acute myeloid leukemia (LAM0).

Therapeutic Options for Immune Thrombocytopenia (ITP) During Pregnancy.

The incidence of ITP during pregnancy is low. When ITP is suspected it is necessary to perform an extended set of clinical and biological investigations in order to determine the etiology of thrombocytopenia, as the diagnosis of ITP is a process of exclusion, because there is no sensitive and specific diagnostic test so far. The treatment for ITP during pregnancy represents a challenge, being necessary in the cases selected according to the obstetrical indications, to the degree of maternal thrombocytopenia and to the extent of the hemorrhagic syndrome, as well as according to the adverse reactions of the treatment on the mother and fetus.

Assessment of changes in membrane properties of platelets from patients with chronic myeloid leukaemia in different stages of the disease.

Patients with chronic myeloproliferative leukemia (CML) have frequent haemorrhage and/or thrombosis in their medical history. The mechanisms of these major and life-threatening complications remain unclear. Membrane organization influences many of the unique cellular functions and is strongly correlated, among other factors, to the membrane lipid composition; it may be evaluated by following up the membrane fluidity and aggregation properties of the platelet.

Sudden blast crisis in a chronic myeloid leukemia patient during imatinib therapy.

Imatinib mesilate (IM) is the first line therapy for chronic myeloid leukemia (CML) patients in chronic phase. Although it offers a complete cytogenetic response (CCyR) in a majority of patients, there still are some rare cases in which a sudden blast crisis (SBC) evolves. The mechanism of this unexpected event is not yet completely understood.

Refractory anemia with ring sideroblasts associated with marked thrombocytosis: case report and literature review.

"Refractory anemia with ring sideroblasts and thrombocytosis" (RARS-T) is a rare disease, a provisional entity, with a controversial status in the 2008 revised WHO classification. Even at present time, RARS-T is a matter of debate whether it is a distinct clinicopathological entity or more likely a constellation of clinical and pathological features of two well-defined myeloid neoplasms, myelodysplastic syndrome and myeloproliferative neoplasm. Perhaps none of the clonal disorders illustrates better the challenges presented by the current classification of myeloid neoplasms, than this clinical entity with overlapping features of both refractory anemia with ring sideroblasts and essential thrombocythemia.

Platelet dysfunction in acute leukemias and myelodysplastic syndromes.

The hemorrhagic and thrombotic diathesis represents a frequent complication in myelodysplastic syndromes (MDS) and in acute leukemias. They are correlated with the number of the platelets, but also with their qualitative disorders, such as membrane glycoprotein changes. The latter are revealed by many platelet studies including flow-cytometry and comprise modified activation, secretion and aggregation patterns.

Chronic myeloid leukemia--from the National to the European Registry--limited experience of a single center.

Chronic myeloid leukemia (CML) is a hematopoietic stem cell disorder in which the diagnosis is confirmed by detection of a genetic marker: Philadelphia (Ph) chromosome in almost 90% of cases. Some of Ph1 negative patients, nevertheless, test positive for the abnormal gene, or the abnormal protein associated with this chromosome, when more sensitive studies, such as PCR or FISH are used and nowadays the diagnosis of CML is based, not only on cytogenetic, but also on molecular analysis. The better understanding of the CML biology provided by the latest researches requires a deeper knowledge about the epidemiologic data in each geographic area, so the compiling of a National and/or European Registry for CML patients, that represents one of the aims of this study, became a stringent matter in our days; it can offer valuable data concerning the real incidence of this disease in Romania and can provide the basics for establishing long-term budgetary strategies.

Molecular markers guide diagnosis and treatment in Philadelphia chromosome-negative myeloproliferative disorders (Review).

The Philadelphia negative chronic myeloproliferative neoplasms are hematological disorders with several diagnostic challenges. Due to recent molecular findings, the WHO classification of Tumors of Hematopoietic and Lymphoid Tissue 2008 reorganized the field of chronic myeloproliferative diseases. Thus, specific molecular markers provide important information for current diagnostic strategies.

Preliminary data on the involvement of B, C and D hepatitis viruses in the etiopathogenesis of chronic lymphoproliferative syndromes in Romania.

Unlabelled: THE AIMS OF THE STUDY: Evaluation of the prevalence of HBV, HCV, HDV infection in patients with chronic lymphoproliferative diseases (CL), identification of the most involved viral genotypes, correlation between viremia dynamics and CL evolution, detection of molecular mechanisms implicated in CL pathogenesis, identification of lymphocytic receptors for viral antigens and biologic markers for early diagnosis of CL.

Methods: We present preliminary results of the first year of our research grant. This is a prospective, analytic, observational study in patients diagnosed with CL and HBV, HCV, HDV chronic infection.

An update on the platelet dysfunction in chronic myeloproliferative syndromes.

The thrombotic and hemorrhagic diathesis represents a frequent complication in myeloproliferative disorders (CMPD). They are correlated with the number of platelets, but also with their qualitative disorders, such as membrane glycoprotein changes. The latter are revealed by many platelet essays including flow-cytometry and include modified activation, secretion and aggregation patterns.

Myeloma cells with asurophilic granules--an unusual morphological variant--case presentation.

We present the case of an 80-year-old man who was admitted for anemia, back pain and progressive weakness. After a workup of clinical and laboratory data, the final diagnosis was multiple myeloma. The bone marrow aspirate revealed 53% myeloma cells with peculiar and rare morphological features: numerous large asurophilic--bright red granules--mucopolizaccharides and immunoglobulins secreted and accumulated in the endoplasmic reticulum, typically known as Russel bodies.