Glucose 6-phosphate dehydrogenase (G6PD) deficiency is one of the commonest human enzymopathies: it is due to inherited mutations of the X-linked gene G6PD. G6PD deficiency makes red cells highly vulnerable to oxidative damage, and therefore susceptible to hemolysis. Over 200 G6PD mutations are known: about one-half are polymorphic and therefore common in various populations. Some 500 million persons with any of these mutations are mostly asymptomatic throughout their lifetime; however, any of them may develop acute and sometimes very severe hemolytic anemia when this is triggered by ingestion of fava beans, or by any of a number of drugs (e.g. primaquine, rasburicase), or more rarely by infection. About one-half of the G6PD mutations are instead sporadic: rare patients with these mutations present with chronic non-spherocytic hemolytic anemia. Almost all G6PD mutations are missense mutations, causing amino acid replacements that entail deficiency of G6PD enzyme activity because they compromise the stability of the protein, or because the catalytic activity is decreased, or through a combination of both mechanisms: thus, genotype-phenotype correlations have been reasonably well clarified in many cases. G6PD deficiency correlates remarkably, in its geographic distribution, with past/present malaria endemicity: indeed, it is a unique example of an X-linked human polymorphism balanced through protection of heterozygotes from malaria mortality. Acute hemolytic anemia can be managed effectively provided it is promptly diagnosed. Reliable diagnostic procedures are available, with point of care tests becoming increasingly important where primaquine and its analogue tafenoquine, recently introduced, are required for the elimination of malaria.

Acquired aplastic anemia (AA) is a rare disease, with an estimated frequency-in Europe and in North America-of about 2 cases per million population per year. 1 There are reports of AA from North Africa, 2 West Africa, 3 East Africa, 4 and Southern Africa, 5 but no data on the frequency of this condition.

Introduction: Sickle Cell Disease (SCD) causes significant morbidity and mortality particularly in sub-Saharan Africa (SSA) where it contributes to early childhood deaths. There is need to standardize treatment guidelines to help improve overall SCD patient health outcomes. We set out to review existing guidelines on SCD and to set minimum standards for management of SCD for the different referral levels of healthcare.Methods: A standards of care working group (SoC-WG) was established to develop the SoC recommendations. About 15 available SCD management guidelines and protocols were reviewed and themes extracted from them. The first draft was on chosen themes with 64 major headings and subtopics. Using a summarised WHO levels of referral document, we were able to get six different referral levels of healthcare. The highest referral level was the tertiary facilities whilst the lowest level was the home setting. Recommendations for SCD management for the regional, district, sub-districts, health posts and CHPs compounds were also drafted.Results: The results from this review yielded a guidelines document which had recommendations for management of SCD on 64 topics and subtopic for all the six (6) different referral levels.Discussions: Every child with SCD need to receive comprehensive care that is coordinated at each level. This recommendation is unique in terms of the availability of recommendations for different levels of care as compared to the traditional guidelines which is more focused at the tertiary levels. Patients can access care at any of the other lower referral hospitals and be managed with recommendations that are in keeping with institutional resources at that level. When such patients need care that requires expertise that is not available at that level, the recommendations will be to refer to the appropriate referral level where those expertise are available. This encourages patients to have good clinical care nearer their homes but also having access to specialist screening modalities and expertise at the tertiary hospitals if need be. With this, patient are not limited to a specific referral level when interventions cannot be instituted for them.Conclusion: This SoC recommendations document is a useful material that can be used for consistent standards of treatment in SSA.

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Background Hematological malignancies of the lymphoid lineage such as multiple myeloma, lymphomas and lymphoid leukemia are a burden to the world especially in Tanzania; anaemia is one of the presenting symptoms of these conditions and is found in almost 100% of the patients at diagnosis. Of particular importance Immune Hemolytic Anemia (IHA) which has a poor response to treatment and subsequently poor prognosis when it is associated with lymphoid malignancies. The aim of this study is to determine the magnitude of immune hemolytic anemia in lymphoid malignancies in Tanzania Methods This was a cross sectional study that recruited 86 patients with lymphoid malignancies attending both Ocean Road Cancer Institute (ORCI) and Muhimbili National Hospital (MNH). The clinical features were captured using a well-structured questionnaire. Laboratory results for Full Blood Count (FBC), reticulocytes count, total, indirect and direct bilirubin and Lactate Dehydrogenase (LDH) were retrieved from the laboratory reporting system. Additionally, a blood sample for the Direct Antiglobulin Test (DAT) was taken from all participants. This test was performed at the Central Pathology Laboratory (CPL) of MNH. Data analysis was performed using Statistical Package for the Social Sciences (SPSS 20.0) and Microsoft excel 2010. Data analysis was performed using South Texas Art Therapy Association (STATA 15.0). Results IHA, indicated by a positive DAT, was found in 10% of patients with lymphoid malignancies. Analysis of DAT among patients never exposed to chemotherapy showed a prevalence of 22%. Among 9 IHA cases, 2 were CLL (Chronic Lymphoid Leukaemia), 2 were MM (Multiple Myeloma), 3 was NHL (Non-Hodgkin Lymphoma), 1 was HL (Hodgkin Lymphoma) and 1 SLL (Small Lymphocytic Lymphoma). All 9 cases had decompensated hemolytic anemia. Conclusion IHA is a real complication of chronic lymphoid malignancies and DAT should be included in tests done during the course of management.

Background Hematological malignancies of the lymphoid lineage such as multiple myeloma, lymphomas and lymphoid leukemia are a burden to the world especially in Tanzania; anaemia is one of the presenting symptoms of these conditions and is found in almost 100% of the patients at diagnosis. Of particular importance Immune Hemolytic Anemia (IHA) which has a poor response to treatment and subsequently poor prognosis when it is associated with lymphoid malignancies. The aim of this study is to determine the magnitude of immune hemolytic anemia in lymphoid malignancies in Tanzania Methods This was a cross sectional study that recruited 86 patients with lymphoid malignancies attending both Ocean Road Cancer Institute (ORCI) and Muhimbili National Hospital (MNH). The clinical features were captured using a well-structured questionnaire. Laboratory results for Full Blood Count (FBC), reticulocytes count, total, indirect and direct bilirubin and Lactate Dehydrogenase (LDH) were retrieved from the laboratory reporting system. Additionally, a blood sample for the Direct Antiglobulin Test (DAT) was taken from all participants. This test was performed at the Central Pathology Laboratory (CPL) of MNH. Data analysis was performed using Statistical Package for the Social Sciences (SPSS 20.0) and Microsoft excel 2010. Data analysis was performed using South Texas Art Therapy Association (STATA 15.0). Results IHA, indicated by a positive DAT, was found in 10% of patients with lymphoid malignancies. Analysis of DAT among patients never exposed to chemotherapy showed a prevalence of 22%. Among 9 IHA cases, 2 were CLL (Chronic Lymphoid Leukaemia), 2 were MM (Multiple Myeloma), 3 was NHL (Non-Hodgkin Lymphoma), 1 was HL (Hodgkin Lymphoma) and 1 SLL (Small Lymphocytic Lymphoma). All 9 cases had decompensated hemolytic anemia. Conclusion IHA is a real complication of chronic lymphoid malignancies and DAT should be included in tests done during the course of management.