Case report: a 37-year-old male with telangiectasias, polycythemia vera, perinephric fluid collections, and intrapulmonary shunting
Conclusion Our case is the first to describe the combination of polycythemia vera, telangiectasias, perinephric fluid collections, and intrapulmonary shunting. The presentation is highly similar to the previously described TEMPI syndrome, though calls into question the potential importance of the monoclonal gammopathy. Our patient demonstrated a response to treatment with hydroxyurea, while patients with the TEMPI syndrome have shown responses to plasma-cell directed therapies such as bortezomib. (Source: BMC Hematology)
Source: BMC Hematology - July 22, 2014 Category: Hematology Source Type: research

Long-term effect of imiglucerase in Latin American children with Gaucher disease type 1: lessons from the International Collaborative Gaucher Group Gaucher Registry
Abstract Background The clinical characteristics of Latin American children enrolled in the International Collaborative Gaucher Group Gaucher Registry at the time of first enzyme therapy infusion (baseline) were investigated, with special emphasis on long-term outcomes. Methods Inclusion criteria were all Latin American patients with Gaucher disease type 1 who were
Source: BMC Hematology - July 11, 2014 Category: Hematology Source Type: research

CD4 positive/CD8 negative/CD56 positive T cell large granular lymphocyte proliferations; clonal disorders of uncertain significance
Conclusions Flow cytometric immunophenotyping remains an essential tool for the diagnosis of T cell proliferations and helped to clinch the diagnosis in these cases. The aim of this case series is to present clinical and immunophenotypic features of these rare CD4 positive T large granular lymphocyte proliferations that fulfil the WHO defining criteria, however are better regarded as clonal expansions rather than as leukemias in view of their indolent and non progressive nature. (Source: BMC Hematology)
Source: BMC Hematology - July 2, 2014 Category: Hematology Source Type: research

Determination of hematological and immunological parameters among HIV positive patients taking highly active antiretroviral treatment and treatment naïve in the antiretroviral therapy clinic of Gondar University Hospital, Gondar, Northwest Ethiopia: a comparative cross-sectional study
Abstract Background Anemia, leucopenia and thrombocytopenia are the commonest hematological abnormalities resulting from human immunodeficiency virus infection. The use of antiretroviral drugs could positively or negatively affect these disorders. Thus a specific diagnosis and a determination of hematological and immunological parameters are required for initiating and monitoring early treatment to avert disease progression. Therefore, this study aimed to compare hematological and immunological parameters in HIV positive patients taking antiretroviral therapy and those treatment naïve ...
Source: BMC Hematology - March 25, 2014 Category: Hematology Source Type: research

Combined meta-analysis of systemic effects of allogeneic stem cell transplantation and systemic sclerosis
Conclusions We designed a new approach for meta-analysis by combining data from different diseases using common clinical manifestation as a linker. This allowed us to power up the insufficient standalone meta-analysis of aSCT microarray studies, by adding SSc samples to the data pool. This new method has successfully identified novel molecular targets for systemic effects of both aSCT and SSc. We believe that this approach is generalizable and can be applied to an array of diseases with common clinical manifestations. (Source: BMC Hematology)
Source: BMC Hematology - March 22, 2014 Category: Hematology Source Type: research

Inverse correlation between serum interleukin-6 and iron levels among Japanese adults: a cross-sectional study
Conclusion Lower serum iron levels are significantly associated with higher serum IL-6 levels among Japanese adults. (Source: BMC Hematology)
Source: BMC Hematology - February 28, 2014 Category: Hematology Source Type: research

HIV, HBV, HCV and T. pallidum infections among blood donors and Transfusion-related complications among recipients at the Laquintinie hospital in Douala, Cameroon
Conclusion This study confirms the presence of blood-borne infectious diseases in blood donors at the LHD, identifying T. pallidum as the greatest threat to blood safety in the region, and hematuria as the most common immunological complications in blood recipients. (Source: BMC Hematology)
Source: BMC Hematology - February 12, 2014 Category: Hematology Source Type: research

Rapid and reliable detection of α-globin copy number variations by quantitative real-time PCR
Conclusions HBA-CNV is able to detect all known large deletions and duplications affecting the α-globin genes, providing a flexible and simple workflow with rapid and reliable results. (Source: BMC Hematology)
Source: BMC Hematology - January 24, 2014 Category: Hematology Source Type: research

Prevalence and severity of anemia among school children in Jimma Town, Southwest Ethiopia
Conclusion Anemia is a moderate public health problem in the study area. Family income, educational status of parents and inadequate plant and animal food intake are the predictors of anemia. Improving the economic status of the family, women education and health education about balanced animal and plant food consumption are recommended strategies to reduce the burden of anemia. (Source: BMC Hematology)
Source: BMC Hematology - January 16, 2014 Category: Hematology Source Type: research

An adult patient with Nijmegen Breakage Syndrome and Hodgkin's Lymphoma
Conclusion We show that application of standard chemotherapy can lead to long-term disease free survival in patients with a DNA repair disorder. Furthermore, we describe here, to the best of our knowledge, the first adult patient with NBS and HL. (Source: BMC Hematology)
Source: BMC Hematology - January 16, 2014 Category: Hematology Source Type: research

Reviewer acknowledgement 2013
Contributing reviewers The editors of BMC Hematology would like to thank all our reviewers who have contributed to the journal in Volume 13 (2013). (Source: BMC Hematology)
Source: BMC Hematology - January 15, 2014 Category: Hematology Source Type: research

How useful are complete blood count and reticulocyte reports to clinicians in Addis Ababa hospitals, Ethiopia?
Conclusion Overall, most clinicians do not use much of the data provided on routine CBC report. Additional research is needed to understand the issue further. Responsible bodies should promote the appropriate use of CBC/Retics reports by clinicians. (Source: BMC Hematology)
Source: BMC Hematology - December 11, 2013 Category: Hematology Source Type: research

An etiological reappraisal of pancytopenia - largest series reported to date from a single tertiary care teaching hospital
Conclusion Detailed clinical history and meticulous physical examination along with baseline hematological investigations, provides invaluable information in the complete workup of pancytopenic patients, helping in systematic planning of further investigations to diagnose and ascertain the cause, avoiding a battery of unnecessary tests. (Source: BMC Hematology)
Source: BMC Hematology - November 6, 2013 Category: Hematology Source Type: research

Time-to-diagnosis and symptoms of myeloma, lymphomas and leukaemias: a report from the Haematological Malignancy Research Network
Conclusions Pathways to diagnosis are varied and can be unacceptably prolonged, particularly for myeloma and some lymphomas. More evidence is needed, along with interventions to reduce time-to-diagnosis, such as public education campaigns and GP decision-making aids, as well as refinement of existing Referral Guidelines. (Source: BMC Hematology)
Source: BMC Hematology - October 31, 2013 Category: Hematology Source Type: research