Giant Cell Tumor of the Proximal Phalanx: Report of two Cases Treated by Two Different Methods and Review of the Literature.

The hand is an extremely rare site for giant cell tumor (GCT). There are only a few reported cases of GCT including the hand, and even fewer reporting involvement of phalanges. GCTs in small bones are typically more aggressive and have higher local recurrence and rate of metastasis in younger patients compared to long bone involvement, so the treatment is more clinically challenging in the hand. In this study, we present the management of giant cell tumors of the proximal phalanxin two patients treated with two different method; ray resection and arthrodesis using an iliac crest graft. Key words: giant cell tumor, phalanx, hand, recurrence.

A review of the role of infections in the aetiology of haemolysis in patients with sickle cell diseases: pathogenesis, management, and prevention

Background: Sickle cell disease (SCD) is associated with chronic haemolysis, immuno-suppression and susceptibility to infections, which may trigger infection-associated haemolysis (IAH). SCD patients are vulnerable to anaemic effect of IAH due to vicious interaction between pre-existing 'inherited' chronic haemolysis and 'acquired' IAH. IAH in SCD manifests as febrile haemolytic crisis with clinical and laboratory features of severe anaemia or pancytopenia. Clinico-pathological perspectives of IAH in SCD are fragmented. This review presents a comprehensive but concise overview of pathogenesis, management and prevention of IAH in SCD. Methodology and results: Online literature search using search terms such as 'sickle cell disease, viral, bacterial, parasitic, fungal, infections, hyperhaemolytic crisis, haemophagocytic syndrome, severe anaemia, pancytopenia' in various combinations was done on PubMed/Medline, Google, Google-Scholar and Bing. Overall, 112 relevant publications were retrieved, which included 109 peer reviewed journal articles, 2 World Health Organization (WHO) technical reports, and 1 edited text book. A range of bacterial (Bartonella spp, Mycoplasma spp., Mycobacterium avium complex), viral (Dengue, SARS-CoV-2, Parvovirus-B19, Cytomegalovirus, Epstein-Barr virus), parasitic (Plasmodium spp., Babesia spp.), and fungal (Histoplasma spp.) infections were associated with IAH in SCD. There are two broad types of IAH in patients with SCD; infection associated extra-medullary haemolysis (IAEMH) and infection associated intra-medullary haemolysis (IAIMH). While IAEMH is associated with severe anaemia due to intravascular haemolysis caused by red cell invasion, oxidative injury, auto-antibodies, and/or pathogen-haem interaction, IAIMH is associated with haemophagocytic tri-lineage destruction of haematopoietic precursors in the bone marrow. Conclusion: Various microbial pathogens have been associated with IAH in SCD. SCD patients with fever, severe anaemia or pancytopenia should be investigated for early diagnosis and prompt treatment of IAH, which is a lifethreatening haematological emergency for which transfusion therapy alone may not suffice. Prompt and sustainable termination of IAH may require therapeutic combination of transfusion, anti-microbial chemotherapy, and immune modulation therapy. SCD patients should also receive counselling on hygiene, barrier protection against vectors, routine chemoprophylaxis for locally endemic diseases, and immunization for vaccine-preventable infections as a long-term preventive strategy against IAH.

Adequacy and pattern of blood donations in north-eastern Nigeria: the implications for blood safety.

In a retrospective analysis, the quantities, patterns and adequacy of blood donations made, between 1984 and 2006, at the University of Maiduguri Teaching Hospital in north-eastern Nigeria were explored and related to blood safety in the study area. The types of blood donor were reviewed and the annual increments in the number of donations made were estimated and compared with the annual increments in the numbers of in-patients managed at the study hospital. The mean annual increment in the number of blood donations (4%) fell well below the mean annual increment in in-patient numbers (11%). The blood donations received at the hospital fell into four types: voluntary, family-replacement, commercial and pre-deposit autologous donations. Over the study period, the percentage of donations falling into the voluntary and family-replacement categories fell from 31% to 5% and from 49% to 23%, respectively. These falls were matched by increases in the percentages of donations categorised as commercial and autologous, which rose from 20% to 63%, and from 1% to 9%, respectively. By the end of the study period, the quantity of blood being donated at the hospital was grossly inadequate and predominantly derived from family and commercial donors, who were found to be generally inferior, in terms of blood safety, to voluntary donors. There is an urgent need to rectify this situation by setting up a functional and national blood-transfusion service in Nigeria.

The burden of HIV and AIDS on blood bank reserves in northeast Nigeria.

This study was conducted to evaluate the burden of HIV/AIDS on blood bank reserves at the University of Maiduguri Teaching Hospital, Nigeria, during 1995-2005. The number of admissions due to HIV/AIDS rose from 43 in 1995 to 447 in 2005, and the proportion of blood units used in transfusing patients with HIV/AIDS rose steadily from 3% in 1995 to 19% in 2005. The proportion of blood units used in managing obstetric patients was also high and fluctuated between 36% and 50% during the period of study. Controlling the spread of HIV, effective treatment of patients with antiretroviral drugs and setting up a functional national blood transfusion service could lessen the rising burden of transfusion in Nigeria and improve blood availability for maternal and child health care.

Clinical significance of glucose-6-phosphate dehydrogenase deficiency in Nigerian patients with sickle cell disease.

A total of 75 (45 males, 30 females) patients with sickle cell disease (SCD) at the university of Maiduguri Teaching Hospital were screened for G-6-PD deficiency and their steady state levels of haematocrit, reticulocyte count and irreversibly sickled cells (ISC) as well as the occurrence of vaso-occlusive crises were analysed with respect to their G-6-PD status during a 3 year period (1998-2000). None of the female patients was G-6-PD deficient, while (15.6%) male patients had the enzyme deficiency. The patients with G-6-PD deficiency had significantly lower mean level of haematocrit (0.221/l) with higher level of reticulocyte count (15%) as compared to their non-G-6-PD deficient counter parts who had mean values of 0.271/l and 9% for haematocrit count respectively. However, there was no significant difference in mean levels of ISC between G-6-PD deficient (6%) and non-G-6-PD deficient (5%) patients with SCD. Patients with G-6-PD deficiency had significantly lower episodes of vaso-occlusive crises (2.1 episodes/patient). This data would suggest that co-inheritance of G-6-PD deficiency in SCD reduces frequency of crises and improves prognosis.

Urticarial reactions in obstetric transfusion in Maiduguri, north east Nigeria.

A total of 135 obstetric patients who were first-time transfusion recipients at the University of Maiduguri Teaching Hospital were studied with respect to the incidence and clinical severity of urticarial transfusion reactions (UTR) in relation to the number of previous pregnancies. The overall incidence of UTR was 12.6%. Analysing the data with respect to the number of previous pregnancies the incidence of UTR were 0%, 0%, 3.8%, 8.3%, 21.7% and 37.5% among patients with 0.1, 2, 3.4 and 5 previous pregnancies respectively. However, all cases of UTR in the affected patients were clinically mild, manifesting with urticarial rashes and pruritus without any features of anaphylaxis. This data would suggest that the risk of UTR increases with the number of previous pregnancies. Therefore, multiparous patients need closer observation during transfusion therapy.

Bone marrow morphological features in anaemic patients with acquired immune deficiency syndrome in Nigeria.

The morphological features of bone marrow aspiration biopsies performed at the University of Maiduguri Teaching Hospital from 1997 to 1999 (3 years) on 24 anaemic AIDS patients (Table i) were retrospectively reviewed. The marrow was normocellular in 7(29.2%) cases and hypocellular in 17(70.8%) cases. Erythropoiesis was normoblastic in 5(20.8%) cases, micronormoblastic in 8(33.3%) cases and megaloblastic in 11(45.8%) cases. All of the 8(33.3%) cases with micronormoblastic erythropoiesis had no stainable iron stores while the remaining 16(66.7%) cases with either normoblastic or megaloblastic erythropoiesis had increased stainable iron stores. Myelopoiesis was sequential in all cases studied. Megakaryocytes were adequate in all cases. Dysplasia in the form of cytoplasmic vacuolations affecting both erythroid and myeloid precursors was seen in 4(16.7%) cases. Lymphocytes counts were normal in 17(70.8%) cases and increased in 7(29.2%) cases. Plasma cells were increased in all cases. Haemophagocytosis was seen in only 1(4.2%) case. Of the 24 cases studied. 10 and 14 cases had positive and negative history of Chloramphenicol ingestion respectively and the cases with positive history of the drug ingestion had significantly higher frequency (90%) of marrow hypocellularity as compared to the lower frequency of 51.7% seen among cases with negative history of chloramphenicol ingestion. These marrow features were thought to reflect the combined effect of malnutrition and drug (Chloramphenicol) in a background state of advanced chronic disease due to AIDS.