ISRN Hematology最新文献

The Notch signaling pathway is an evolutionarily conserved cell signaling system present in most multicellular organisms, as it controls cell fate specification by regulating cell proliferation, differentiation, apoptosis, and survival. Regulation of the Notch signaling pathway can be achieved at multiple levels. Notch proteins are involved in lineage fate decisions in a variety of tissues in various species. Notch is essential for T lineage cell differentiation including T versus B and αβ versus γδ lineage specification. In this paper, we discuss Notch signaling in normal T-cell maturation and differentiation as well as in T-cell acute lymphoblastic lymphoma/leukemia.

The first case is about a man of 60 years old suffering of hypereosinophilic syndrome (HES) developed since 1998. He presented chronic cough, insomnia, and negative parasitical test. We observed hypereosinophilia and fibroblastic hyperplasia at the bone marrow biopsy. Initially, hydroxyurea and α-interferon treatment failed. We proposed to him imatinib mesylate in May 2003. The FIP1L1-PDGFRA gene was detected. The second case is about a man of 34 years old seen in March 2002. First investigation concluded to CML. Progressively, eosinophil cells increased, and complications occurred as oedema syndrome, dyspnoea, and parietal chronic endocarditic fibrosis associated with pericarditis. In addition, a bowel obstruction happened and was cured by surgery. Bcr-abl fusion was negative, and FIP1L1-PDGFRA gene was detected after and imatinib mesylate was given. Actually, endocarditic fibrosis decreased. The two patients are in haematological and cytogenetic remission. We concluded that clonal HES is present in Africa, and imatinib mesylate is effective.

Introduction. Hodgkin lymphoma shows a well-known geographic pattern, but temporal changes have been found recently as well. Patients and Methods. 439 Hodgkin lymphoma patients' clinicopathological and treatment data were processed in calendar periods of approximately ten years. The patients were treated at our department from 1980 until the end of 2008. Results. The first period (1980-89) contained 177 patients, the second (1990-99) 147, and the third (2000-08) 115 Hodgkin lymphoma patients. The mean age of the patients was 40.1, 35.9, and 36.8 years in order. The male/female ratio: 1.42, 1.45, 1.05 in order. Contrary-wise a unimodal age group pattern could have been seen with an incidence peak between 30 and 39 in the past decades. The incidence of classical mixed cellularity histological subtype is decreasing (43.7%, 58.23%, 42.6%, P = 0.0098 (it is only significant in the second period)); classical nodular sclerosis shows an increasing tendency (25%, 27.32%, 34.78%, P = 0.1734). The first incidence peak is predominantly created by classical nodular sclerosis, meanwhile the second peak by classical mixed cellularity. The number of early-stage patients (59.12%) is beyond the advanced stage (40%) in the last decade. Meanwhile, the number of second-stage patients was increasing (25.8%, 26.35%, 49.56%  P < 0.0001) and of patients in third stage was decreasing (53.4 %, 50.67%, 20%  P < 0.0001). The 5- and 10-year overall survival data were progressing: 59.7 %, 77.4 %, and 90.5 % and 44.1 %, 70.6 % and 90.5 % (expected survival) in the last decade. Conclusions. Changes can be explained by the altered nature of Hodgkin lymphoma, the changes in socioeconomic status and the development of diagnostic and therapy methods.

The study describes a case of a 67-year-old female who developed a Stage I E marginal zone lymphoma of the right triceps muscle 1 month after influenza vaccination at the same site. She was treated with single modality, involved field radiation therapy (IFRT) to 4000 cGy in 20 fractions with excellent response and no evidence of disease after one year followup.

WE REPORT THE FIRST CASE OF COSEGREGATION OF TWO HAEMOGLOBINS (HBS): HbG-Philadelphia [α68(E17)Asn → Lys] and HbDuarte [β62(E6)Ala → Pro]. The proband is a young patient heterozygous also for β°-thalassaemia. We detected exclusively two haemoglobin variants: HbDuarte and HbG-Philadelphia/Duarte. Functional study of the new double variant HbG-Philadelphia/Duarte exhibited an increase in oxygen affinity, with a slight decrease of cooperativity and Bohr effect. This functional behaviour is attributed to β62Ala → Pro instead of α68Asn → Lys substitution. Indeed, HbG-Philadelphia isolated in our laboratory from blood cells donor carrier for this variant is not affected by any functional modification, whereas purified Hb Duarte showed functional properties very similar to the double variant. NMR and MD simulation studies confirmed that the presence of Pro instead of Ala at the β62 position produces displacement of the E helix and modifications of the tertiary structure. The substitution α68(E17)Asn → Lys does not cause significant structural and dynamical modifications of the protein. A possible structure-based rational of substitution effects is suggested.

The hematological reference values are very important for diagnostic orientation and treatment decision. The aim of this study was to establish hematological reference values for healthy adults in Togo. A total of 2571 voluntary blood donors participated to this study. Only 1349 subjects negative for HIV, HBV, HCV, malaria, and without hemoglobin abnormalities in electrophoresis and hypochromia on blood smear, were definitively retained for the study. Median hemoglobin level was higher in males than females (15.1 g/dL versus 13.0 g/dL, p = 0.000). Median total WBC (4.2×10(9)/L) and absolute neutrophil counts (1.6×10(9)/L) were similar by gender. The median lymphocyte counts in males and females were, respectively, 2.1×10(9)/L and 2.2×10(9)/L (p = 0.11). The median platelet count was lower in males than females (236×10(9)/L versus 247×10(9)/L, p = 0.004). Our median values for RBC parameters differ from those of African countries probably because of our inclusion criteria which eliminate most cases with iron deficiency and/or thalassemia.

We report the case of a 37-year-old woman who had a relapse of acute myeloid leukemia (AML) during treatment for chronic graft versus host disease (cGVHD) after allogeneic bone marrow transplantation. She was originally suspected of having autoimmune pancreatitis. Relapse of AML often occurs at extramedullary sites. Whereas the pancreas is rare as an organ of AML relapse, physicians should be aware that enlargement of the pancreas could be a sign of relapsed AML when excluding autoimmune pancreatitis, particularly during active cGVHD after allogeneic stem cell transplantation.

During the past decade, intravenous iron supplementation to ESA (erythropoiesis-stimulating agent) therapy has emerged as an option to augment hemoglobin response in anemic cancer patients. In this paper, the results of seven published randomized clinical trials assessing the role of iron supplementation to ESA therapy in the hematology/oncology setting will be discussed. The pathogenetic mechanisms behind functional iron deficiency, a major reason for ESA hyporesponsiveness in cancer, will also be described.

The distribution and adverse effects, especially to optic and acoustic nerves, of cobalt released from a hip arthroplasty and its association with albumin were studied. The analysis of cobalt was performed in plasma, whole blood, urine, and cerebrospinal fluid by inductively coupled plasma mass spectrometry (ICP-MS). The fraction of albumin binding the metal was determined by colorimetric assay using dithiothreitol (DTT). In all the biological matrices very high levels of cobalt were measured, but contrary to expected, a higher concentration in whole blood than in plasma was observed. The determination of altered albumin confirmed this hypothesis. This evidence might indicate an alteration in the binding of cobalt to albumin and a consequent increase in the concentration of the diffusible (free) fraction of the metal. This appears an interesting starting point for further investigations for identifying and better understanding cobalt neurotoxicity, apparently not so frequent in occupational medicine and clinical practice.

Sickle cell disease is one of the common hemoglobinopathies in the world. It can affect any part of the body and one of the most common and an early organ to be affected in SCA is the spleen. It is commonly enlarged during the first decade of life but then undergoes progressive atrophy leading to autosplenectomy. This however is not the case always and sometimes splenomegaly persist necessitating splenectomy for a variety of reasons including acute splenic sequestration crisis, hypersplenism, massive splenic infarction and splenic abscess. Splenic complications of SCA are known to be associated with an increased morbidity and in some it may lead to mortality. To obviate this, splenectomy becomes an essential part of their management. This review is based on our experience in the management of 173 children with various splenic complications of SCA necessitating splenectomy.