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Congenital afibrinogenaemia (CA), is a rare inherited bleeding disorder characterised by complete deficiency of fibrinogen in the plasma. Blood clotting tests are indefinitely prolonged in patients. The mode of inheritance is autosomal recessive. Typically patients present with excessive cord bleeding after birth with intracerebral haemorrhages reported in childhood. Other manifestations include musculoskeletal haemorrhages, mucocutaneous bleeds with poor wound healing reported occasionally. In females, menorrhagia, repeated early pregnancy loss and post-partum haemorrhages are common. We present a four year old female who initially presented with severe cord bleeding after birth, warranting a blood transfusion. Currently she experiences recurrent epistaxis, easy bruising and excessive post-traumatic haemorrhages. All her clotting times are markedly prolonged. Her plasma fibrinogen and fibrinogen antigen are undetectable. An older sibling died from excessive cord haemorrhage after birth. Bleeds in CA respond very favourably to fibrinogen concentrates, cryoprecipitate and fresh plasma. To date, 242 cases of CA have been reported worldwide, none of them in Kenya. Our aim in reporting this case is to document the disorder, and also to raise the index of suspicion of the condition.

Histoplasmosis, caused by two varieties of dimorphic fungi, Histoplasma capsulatum variant capsulatum and Histoplasma capsulatum variant duboisii is a systemic fungal infection. It has a worldwide distribution and is shown to be more prevalent in North America and Central America. Both variants occur in Africa. Disease spectrum ranges from asymptomatic primary infection to disseminated disease in immunocompromised patients. Since the upsurge of acquired immune deficiency syndrome (AIDS) and despite the availability of high active anti-retroviral therapy (HAART) many patients still present with opportunistic infections of which histoplasmosis is one. Four cases are presented; two infants and two adults. All had disseminated disease with multiple organ involvement and the disease was not suspected clinically. Diagnosis was made incidentally on bone marrow aspirate cytology. The two adult cases were HIV positive, one with CD4 counts of 132 cells/microlitre and was not on HAART. The other was on HAART but the CD4 had not been determined. One of the infants tested HIV negative and the others status was unknown. A high index of suspicion is required for diagnosis as the disease may mimic tuberculosis(TB) and other causes of hepatosplenomegaly such as visceral leishmaniasis. Laboratory diagnosis includes culture, direct staining, antigen and antibody detection. Antibody detection may give false negative in the immunocompromised patient. The infection responds well to antifungal agents (amphotericin B is the drug of choice) and life long maintenance therapy may be required in AIDS especially if CD4 counts remain less than 150 cells/microlitre. Histoplasmosis should be a differential diagnosis in immunosuppressed patients with unexplained fever, weight loss, hepatosplenomegaly and chest findings especially if not responding to anti-TB treatment.

Objectives: To determine the prevalence and to characterise Gaucher's disease in terms of socio-demographic data, clinical presentation, and management as seen at Kenyatta National Hospital., Design: A retrospective record based study., Setting: Kenyatta National Hospital, a referral and teaching hospital., Main Outcome Measures: Prevalence (number of cases seen a year), of Gaucher's disease, sociodemographic data, clinical presentation, mode of diagnosis and treatment modalities of Gauchers disease., Results: Nine patients were studied, four males and five females giving a prevalence of 0.9 cases seen a year and a M:F ratio of about 1:1. The most common presentation was splenomegaly in nine (100%) cases and hepatomegaly in seven (78%) patients, neurological and bone symptoms were rare, in one (11%) cases and in two (22%) cases respectively. Diagnosis was mainly on basis of presence of Gaucher cells in bone marrow and splenic aspirate as enzyme assay was unavailable. Management was mainly supportive and enzyme therapy was only available for two (22%) patients. Anaemia was the most common complication with seven (78%) patients and one death occurred due to osteomyelitis. Only four (44%) patients were followed up for a period of four years., Conclusions: Gaucher's disease is a rare condition at the Kenyatta National Hospital (KNH). The presentation of most patients is organomegaly, (hepatosplenomegaly) and best fits the type 1 or non-neuronopathic Gaucher's disease. Neurological manifestations are rare. Management of this condition at the KNH is mainly supportive and enzyme therapy still remains out of reach for most patients.

Objectives: To determine the prevalence of malaria in donor units in a low and a high endemic region in Kenya and evaluate the cost effectiveness of recipient anti-malarial prophylaxis and pre-transfusion screening (using an automated method) as options to prevent post transfusion malaria., Design: A descriptive cross-sectional study., Setting: Two regional blood banks, Nairobi and its environs (National Blood Transfusion Services, Nairobi) a low malaria endemic region and western region (National Blood Transfusion Services, Kisumu) high malaria endemic region., Subjects: All the donated units were included in the study for analysis, during the duration of study, from the two study sites., Main Outcome Measures: Prevalence of malaria in donor units in low endemic area (Nairobi) and high endemic area (Kisumu). Cost per case prevented for the two options, Option I Prophylactic administration of anti-malarial (sulfadoxine pyrimethamine SP) drugs to recipients, and Option II pre-transfusion screening using an automated technique., Results: A malaria prevalence of 0.67% was found in Nairobi and its environments (low endemic) and 8.63% for Kisumu and its environments (high endemic area). The cost analysis showed a cost per case prevented of Ksh.105 (US$1.4) adult, Ksh.52.5 (US$0. 69) and paediatric for the option of recipient prophylaxis using an SP based drug. The cost escalated to Ksh.592 (US$7.79) adult Ksh.444 (US$5.84) paediatric if the prophylaxis was upgraded to the recommended artemisinin derivative (ACT-artemisinin based combination) and for the option of pre-transfusion screening using an automated technique the cost was Ksh.2.08 (US$0.03)., Conclusion: The prevalence of malaria in donors showed the expected regional variation in the low and high endemic areas and was comparable to data obtained elsewhere. If malaria positive donor units were to be excluded from the national blood supply, an estimated 5% (compared to 1.3% for human Immunodeficiency virus, 3.6% for hepatitis B virus and 1.3% for hepatitis C virus) would be wasted. The cost per case prevented of transfusion-associated malaria is considerably higher for recipient antimalarial prophylaxis than pre-transfusion screening using an automated technique. The cost escalates by five to seven times if the newer artemesinin based combination antimalarial drugs are adopted.

Objectives: To determine the status of blood donor haematology in two regional sites in Kenya and to assess the potential role of automated haematology in National blood bank process control., Design: A cross sectional descriptive study., Setting: Two regional blood banks--Nairobi and its environs (Blood Transfusion Services, Nairobi) and Western Region (National Blood Transfusion Services, Kisumu)., Main Outcome Measures: Distribution, mean, median, and 95% percentile ranges of haemoglobin (Hb), red cell parameters (red cell count, haematocrit, MCV, MCH and MCHC), total and differential white blood cell (WBC) counts, and platelet counts in the two donor populations., Results: A significant number of donations (16.5% in Kisumu and 3.4% in Nairobi) showed haemoglobin levels below the recommended National Blood Transfusion Service (NBTS) guideline of 42g/unit. Compared to Kisumu, Nairobi donors had significantly (p < 0.001) higher Hb, MCV and MCH values while the red blood cell counts and MCHC values were similar (p > 0.05). A low MCV (< 78 fl) was observed in 12.4% and 3.4% of Kisumu and Nairobi donors respectively. Both populations showed similar but significant frequencies (Kisumu, 21.3%; Nairobi, 18.7%) of mild neutropenia (< 1.5 x 10(9)/1), while eosinophilia (> 0.5 x 10(9)/1 in the tropics the cut off is > 0.6 x 109) was more prominent in Kisumu donors (18.8% versus 8.5%). Platelet counts were also significantly lower in Kisumu donors, with the prevalence of thrombocytopenia (< 150 x 10(9)/1) being considerably higher (15.9% versus 3.7%)., Conclusions: A significant number of Kenyan donors showed abnormal haematology profiles that may indicate underlying pathology. Such abnormalities are not detected by current blood transfusion services screening practices and there may be a need to strengthen donor selection criteria to protect both donors and recipients.

Granulocytic sarcoma (GS) is a rare extramedullary solid tumour composed of malignant immature cells of the granulocytic series. It may herald, accompany or signal acute myeloid leukaemia (AML) or chronic granulocytic leukaemia (CGL). GS may also occur in patients with myelodysplastic syndromes (MDS) where it is a sign of imminent disease progression. Three cases of GS are presented; the first one involving the pancreas and preceding AML, the second case affecting uterine cervix in stable phase CGL and the third case is GS of the breast accompanying AML. Any site of the body may be involved by the GS, and morbidity depends on the local organ/tissue affected in addition to the attending primary leukaemia or MDS. Treatment of GS involves surgery, radiotherapy and chemotherapy. The objective of this communication is to enhance awareness in personnel providing health care. Further, early diagnosis and treatment affects overall outcome.