Journal du sang et de la lymphe

In more than 85 year-old history of the Gordon Research Conference, a conference on the scheme of lymphatic systemwas held for the first time on March 2004, in Ventura, California, USA. Such trends of lymphatic Gordon conference are considered to reflect rapid progress of lymphology inspired by the discovery of new markers of
lymphatic endothelial cells such as LYVE-1, podoplanin, and Prox1, and then the development of the VEGF C/D and VEGF R3-mediated lymphangiogenesis and lymphatic metastasis of carcinoma cells.

We report three cases of myelodysplastic syndrome (MDS), refractory anemia with ringed sideroblasts (RARS), who responded to the anti-malarial chloroquine in combination with recombinant erythropoietin (darbepoetin alfa). All three patients either remained or achieved transfusion independence and increased their hemoglobin level by at least 1 gm/dl. Responses occurred rapidly, becoming clinically noticeable within the first 4-6 weeks from start of therapy. All patients were followed for at least a 6 months period during which they have remained transfusion independent. Chloroquine affects multiple cellular pathways, but the precise mechanism of its action in RARS
patients is unclear.

Hepatitis B Virus (HBV) infection is one of the leading causes of death worldwide. The most important marker for HBV infection is Hepatitis B Surface Antigen (HBSAg). HBSAg rapid screening test methods are the most popular methods used in most developing countries even though the Enzyme-Linked Immunosorbent Assay (ELISA) and Nucleic Acid Testing methods are considered as more reliable methods worldwide. The aim of this study was to compare the analytical sensitivity of a rapid kit and ELISA method in the detection of HBV infection among blood donors. One hundred (100) blood donor samples which tested negative with Rapid screening test kit were re- tested using ELISA method. Out of 100 rapid test negative blood donor samples tested, 9 (9%) tested positive with ELISA method. Thirty blood samples which tested positive with rapid screening test kit were also re-tested using ELISA method. All 30 samples (100%) tested positive with ELISA method. This study shows that the rapid screening test kits are inferior and are associated with more false negatives compared to the ELISA technique. There is need for the Nigerian Government to develop a safe blood donor screening strategy for HBV by combining the use of the less sensitive rapid screening techniques with the more sensitive ELISA and NAT screening method to ensure the safety of blood donation in the country and to limit the risk of transfusion-transmissible hepatitis B infection.

When the patent of a small molecule drug expires, generics may be introduced. They are considered herapeutically equivalent once pharmaceutical equivalence (i.e. identical active substances) and bioequivalence i.e. comparable pharmacokinetics) have been established in cross-over volunteer study. However this generic paradigm cannot be applied to complex drugs as biologics. For copies of biologics EMA, and FDA, have introduced  new regulatory bio-similar pathway which mandate clinical trials to show therapeutic equivalence. However for ome complex drugs, such as iron-carbohydrate drugs, low molecular weight heparins (named Non Biologic Complex Drugs [NBCD]), regulatory guidance is still mostly lacking. In this paper we will discuss therapeutic experiences with these different classes of complex drugs and their specificity, to provide scientific arguments for consideration for a new regulatory framework.

Routine orthopedic specimens provide a unique opportunity to review the bone marrow of patients. The processing of these specimens differs at different hospitals and has been scrutinized lately due to the recent wave of cost consciousness in health care. We review examples of important unexpected findings in such cases, as well as the benefits of review, in light of studies showing the rarity of such findings.

Both truncular and extratruncular forms of lymphatic malformations; as described in the Hamburg classification; remain difficult to treat. Most of these malformations are treated adequately or present with complications (recurrent infection, cellulitis, bleeding, skin ooze) increasing the therapeutic challenge. We present a case with complications of a LM highlighting the need for early intervention for these lesions. A 19 year old male presented with watery discharge from the skin over a left thigh swelling since childhood. He was diagnosed to have an extratruncular Lymphatic Malformation (LM). Treatment was deferred. He presented with necrosis of the skin and subcutaneous tissue after 3 months requiring debridement and split thickness skin graft.

Introduction: The principal object of this study is to represent - in maximum accuracy- the real status of Hodgkin’s lymphoma in west of Algeria, we think also it serves as an adequate indicator for the epidemiological characteristics of HL patients and as an overview of these patients treatment and survival in Algeria.

Patients and methods: From December 2007 to the 1st of January 2013, 668 patients were included in this study, from the age of 15 to 88 years, and both genders. 8 hematology centers participated covering the west of Algeria. All diagnostic procedures and treatment policies were unified between the involved centers, the pilot center (Oran1) adapted a strict system for data collection, control and confirmation, WHO/REAL system was used for histological classification and ECOG system for the performance status. The therapeutic approach consisted of chemotherapy combined or not with radiotherapy according to clinical stage followed when appropriate with intensification protocol and autologous stem cell transplantation.

Results: Of our 668 patients: 53% were males, 47% females, global median age was 36 years [15-88], localized disease was presented in 22% of our study patients, while 78% presented advanced stages, rates of peripheral bulky, Mediastinal Bulky and Non-Bulky disease were: 8%, 45% and 47% respectively. 9% of patients had bone marrow involved by HL. 18% of patients had imaging of affected spleen, while 9% had features of affected liver. 74% of patients had one or more of B-symptoms, 68% of males and 74% of females were anemic, 28% of patients had leukocytosis 65% of all patients had nodular sclerosis subtype. Cumulative Overall Survival was estimated in 352 evaluable patients: 59% (at 60 months), 69% (at 48 months), while 79% of patients were alive after 5 years of follow-up. Cumulative OS for males was: 64% (at 48 months), and Cumulative OS of females: 75% (48 months) P-value>0.05 For patients aged more or less than 45 years, Cumulative OS were 43 and 49 months respectively for the means of OS. With P=0,016 of the 352 evaluable patients: 129 patients had reached CR and been qualified for DFS statistical analysis. Relapse rate was 16%, Cumulative Disease-Free Survival was 69% (at 48 months), no statistically significant difference had been detected between patients aged more or less than 45 years P value = 0.811.

Conclusion: Differences in survival rates between this study and publications maybe explained partially by the very high frequency of negative prognostic factors in our cohort like bulky and advanced disease, B-symptoms, and anemia positivity rates, lack of PET-CT and radiotherapy centers number may had also affected these results, even though we found ourselves very interested in deepening further more in diagnosis, treatment and follow – up modalities standardization and development in west of Algeria in order to raise the level of health services offered to HL patients to meet eventually developed countries survival rates.

Background and Objective: In sub-Saharan Africa, hemophilia remains a huge problem mainly because of ignorance of the disease, limited screening capabilities and access to treatment. This review aims to discuss the characteristics of Cameroonian patients living with hemophilia.

Methods: A retrospective study was done on the data of four main reported studies between 1972 and 2010 in Cameroon. Epidemiological, clinical and biological features of PLWH in Cameroon were analyzed.

Results: The mean age of the patients was between 14 and 16.2 years. All hemophiliacs reported by the studies were male. For most patients, the frequency of bleeding episodes ranged from 2-15 bleeds per year. Chronic joint complications were found in almost all patients in the 2010 study. Hemophilia A was more common than B, representing 88.4% and 87.5% in the 2008 and 2010 studies respectively. All the 37 patients reported in the 2010 study were tested negative for HIV in spite of multiple transfusion histories.

Conclusion: Some features of hemophilia in Cameroon are different from those of other African countries. However, as in many countries, the biggest long term challenge is the constant availability of clotting factors concentrates for patients.