Discovery of Type 3 von Willebrand Disease in a Cohort of Patients with Suspected Hemophilia A in Côte d'Ivoire.

BACKGROUND: Type 3 von Willebrand disease (VWD) is the most severe form of VWD, characterized by a near-total absence of von Willebrand factor (vWF), leading to a massive deficiency in plasmatic factor VIII (FVIII). VWD may be confused with hemophilia A, sometimes leading to misdiagnosis. The purpose of this work was to finalize the biological diagnosis of patients with FVIII activity deficiency in Abidjan in order to guide the best type of management. METHODS: We conducted a cross-sectional descriptive study from June 2018 to April 2019. Forty-nine patients, all of whom had lower FVIII levels or had been referred for a bleeding disorder, were monitored in the clinical hematology service. The pro-coagulant activity of coagulation factors was performed in Abidjan. The assays for von Willebrand antigen and activity were performed at Nîmes University Hospital in France. RESULTS: The mean age of patients was 13.8 years (1 - 65) and 86% were Ivorian. FVIII deficiency was discovered during a biological checkup, circumcision or post-traumatic bleeding, in 33%, 31% and 29% respectively. The FVIII deficiency of patients was classified as severe (89.8%), moderate (8.2%) and mild (2%). Only one patient had a quantitative deficiency of von Willebrand factor (vWF: Ag <3%) with undetectable von Willebrand factor activity (vWF: Ac) and an FVIII level <1%. CONCLUSIONS: Not all of the congenital deficiency of FVIII are represented by hemophilia A. It was crucial to assess the Willebrand factor of these patients followed in Côte d'Ivoire for whom hemophilia A had been suspected.

Influence of the Clinical Status on Stress Reticulocytes, CD 36 and CD 49d of SSFA 2 Homozygous Sickle Cell Patients Followed in Abidjan.

Background and Objectives. Interactions between sickle cells involving CD 49d, CD36, and the vascular endothelium may initiate vasoocclusion leading to acute painful episodes and multiple organ failure. Materials and Methods. We selected 60 SS patients who had never been treated by hydroxyurea. We performed a total blood count. We identified with immunophenotyping by flow cytometry total reticulocytes their distribution according to the degree of maturity (mature, intermediate, very immature) and CD 36(+) and CD 49d(+) antigens. Stress reticulocytes corresponded to the sum of intermediate and immature cells. Results. Subjects in crisis had more total reticulocytes and very immature reticulocytes than subjects in stationary phase (P < 0.05). During the crisis, total CD 36(+) reticulocytes (214 870 ± 107 584/ µ L versus 148 878 ± 115 024/ µ L; P < 0.05) and the very immature CD 36(+) reticulocytes (28.9 ± 7.9% versus 23.0 ± 6.4%; P < 0.05) increased. The clinical status had no impact on CD 49d(+) reticulocytes. Conclusion. The rates of stress reticulocytes in general and those expressing CD 49d and CD 36 were very high. The clinical status had an influence on CD 36(+) reticulocytes. The expression of adhesion molecules is only one of the parameters involved in sickle cell disease crisis.

Imatinib Mesylate Effectiveness in Chronic Myeloid Leukemia with Additional Cytogenetic Abnormalities at Diagnosis among Black Africans.

Imatinib mesylate provides good results in the treatment of CML in general. But what about the results of this treatment in CML associated with additional cytogenetic abnormalities at diagnosis among black Africans? For this, we retrospectively studied 27 cases of CML associated with additional cytogenetic abnormalities, diagnosed in the department of clinical hematology of the University Hospital of Yopougon in Côte d'Ivoire, from May 2005 to October 2011. The age of patients ranged from 13 to 68 years, with a mean age of 38 years and a sex ratio of 2. Patients were severely symptomatic with a high Sokal score of 67%. CML in chronic phase accounted for 67%. The prevalence of additional cytogenetic abnormalities was 29.7%. There were variants of the Philadelphia chromosome (18.5%), trisomy 8 (14.8%), complex cytogenetic abnormalities (18.5%), second Philadelphia chromosome (14.8%), and minor cytogenetic abnormalities (44.4%). Complete hematologic remission was achieved in 59%, with 52% of major cytogenetic remission. The outcome was fatal in 37% of patients. Death was related in 40% to hematologic toxicity and in 30% to acutisation. The median survival was 40 months.

Interest in Determining the CD34+ CD38- Phenotype in the Diagnosis and Prognosis of Acute Leukemia in Abidjan - Côte d'Ivoire.

BACKGROUND: In Côte d'Ivoire, acute leukemias account for 12.5% of hematological malignancies. Acute leukemias are due to an anomaly of the stem cell characterized among other things by the expression of CD34(+) CD38(-) surface markers. This CD34(+) CD38(-) phenotype as well as other factors such as tumor syndrome, high leukocytosis and blasts are considered as important factors of poor prognosis. We therefore proposed to investigate the prognostic value of the expression of CD34(+) CD38(-) markers in acute leukemias in Abidjan. METHODS: We selected 23 patients aged 33 years on whom we performed Complete Blood Count, bone marrow aspiration and immunophenotyping. To search for myeloperoxydase, smears of blood or bone marrow were stained with benzidine and revealed by the use of Hydrogen peroxide. Acute leukemias were then identified and distributed using the score proposed by the European Group for the Immunological characterization of Leukemias. The definitive diagnosis was made by combining morphological characters that serve as the basis for the French-American-British classification as well as cytochemical and immunophenotypic characters. RESULTS: According to the cytological and immunophenotypic classifications, the acute lymphoid leukemia 2 and B IV predominated. 52.2% (12/33) of patients were CD34(+) CD38(-). This phenotype was found in almost all cytological immunophenotypic types. The medullary invasion by blasts (reflection of the tumor mass) of the total sample of CD34(+), CD34(+) CD38(-) patients and those not expressing CD34(+) was respectively 79.4%, 81.25%, 83.3% and 74.8%. CONCLUSION: There was therefore no correlation between medullary blasts and the expression of CD34(+) CD38(-). To the factors we selected it would have been necessary to associate the study of cytogenetic and molecular anomalies to better understand the role of CD34(+) CD38(-) phenotype, concerning prognosis.

Characteristics and Results of the Treatment of Multiple Myeloma in the Subject under the Age of 65 at the University Hospital of Yopougon in Abidjan, Côte d'Ivoire.

We retrospectively studied 30 cases of multiple myeloma in patients under the age of 65, diagnosed from 1991 to 2005 in the clinical hematology department of the University Hospital of Yopougon that is a hospital incidence of 2.9 cases/year. The age of patients ranged from 34 to 64 years, with a mean age of 49 years and a sex ratio of 1.73. The professional activity was variable with 3% of radiographers and 10% of farmers. Clinically, the dominant sign was bone pain in 83% of cases. Myeloma was secretory in 93% of cases. It was Ig G-type in 86%, kappa-type in 66% of cases. 86% of patients were anemic, 20% had creatinine >20 mg/L, and 10% had serum calcium >120 mg/L. Geodes were found in 80% of cases. 53% were at stage III of DURIE and SALMON. Complications were infectious (33%), renal (20%), and hemorrhagic (7%). Chemotherapy regimens were VAD (10%), VMCP (30%), and VMCP/VBAP (60%) with 47% of partial responses, 33% of stable disease, and 7% of very good quality partial responses. The outcome developed towards death in 37% and causes of death were renal in 46% of cases. The median survival was only 5.1 months.

[Homozygous sickle cell disease in Ivory Coast adults]. / Drépanocytose homozygote chez l'adulte ivoirien de plus de 21 ans.

Sickle cell disease is a genetic disease characterized by the synthesis of an abnormal haemoglobin called haemoglobin S. It is the most frequent of the hereditary anomalies of haemoglobin and occurs most commonly in individuals of African descent. Various treatments have considerably improved its prognosis, prolonging the survival of patients, especially those with the most severe, homozygous form. The objective of thisstudy is to describe the epidemiologic, clinical, and laboratory characteristics as well as the disease course and available treatments in adults (aged 21 years or older). This retrospective, descriptive, analytic and non-comparative study included 48 adults of both sexes with homozygous sickle cell disease. Their mean age was 26.1 years (range: 21 to 56 years, and sex ratio 1.3. In all, 70.8% had clinical anaemia, 83.3% were subicteric or icteric and 8.3% had hepatomegaly. Spleen size was normal in 41.7% of patients, and atrophic in 37.5%. No case of splenomegaly was noted and 8.3% had been splenectomised. Haemoglobin rates ranged from 4 g/dL to 12.7 g/dL with an average of 9.5 g/dL, haemoglobin S levels from 83 to 93% with an average of 85.3%, and haemoglobin F levels from 3.5 to 17% with an average of 10.6%. The percentage with fewer than three crises (vasooclusive or haemolytic or both) in a year was 68.7%; 27.1% had from three to five crises, and 4.2% more than five. Disease complications included anaemia in 43.7%, infections in 18.8% and ischaemia in 16.7%; 20.8% had no complications. Age at the beginning of treatment was younger than 5 years in 56.25%, from 5 to 10 years in 29.2%, and older than 10 years in 14.6%. Medical follow-up was regular for 68.7% and irregular for 31.2%. Vaccination was up to date in 58.3. Most patients (83.3%) adhered to their maintenance treatment. In all, 41.7% had not had any blood transfusions, 54.2% had had one or two transfusions, and 4.2% three or more. We compared the patients aged 26 years or younger with those older than 26 and studied the influence of age on different disease variables. Age did not affect the frequency of crises (p = 0.368) or of infections (p = 0.116), the rates of haemoglobin (p = 0.221), haemoglobin S (p = 0.44), or haemoglobin F (p = 0.35), or complications (p = 0.56). Nevertheless, we noted that the frequency of crises, infections, and anaemic complications were higher among the younger patients. Early treatment, regular medical follow-up, maintenance treatment and vaccination have all improved the prognosis of homozygous sickle cell disease considerably. These patients have reached adulthood with relatively few chronic complications.

[Maternal and neonatal iron status in Ivory Coast]. / Métabolisme du fer et valeurs érythrocytaires du nouveau-né ivoirien: relation avec le statut en fer de la mère.

Despite the high prevalence of neonatal anemia in Côte d'Ivoire, complete blood counts and iron studies have not been adequately explored. The authors studied complete blood counts (from peripheral blood mononuclear cells), hemoglobin electrophoresis results, and serum iron, ferritin and transferrin levels in 40 newborns and their mothers. The neonatal results (mean +/- SD) were: hemoglobin: 14.96 +/- 2.24 g/dl; serum iron: 16.88 +/- 7.29 micromol/l; total iron-binding capacity (TIBC): 39.88 +/- 14.85 micromol/l; transferrin: 2 +/- 0.65 g/l; ferritin: 116.20 +/- 105.25 microg/l; and hemoglobin electrophoresis: 22.5% of infants showed some hemoglobinopathy (FAC, FAS, FSA(2)). Maternal serum iron levels were positively correlated with the newborns' TIBC (r = 0.362, p<0.05), maternal ferritin with neonatal transferrin (r = 0.374, p<0.05), maternal transferrin coefficient of saturation (CS) with neonatal TIBC (r = 0.554, p<0.01). These results suggest a high prevalence of iron deficiency in mothers and a consequent potential risk of iron deficiency in their newborns in the absence of iron supplementation.

Reduced levels of T-cell subsets CD4+ and CD8+ in homozygous sickle cell anaemia patients with splenic defects.

T-lymphocyte subsets were studied in two patient groups: (1) 50 patients with homozygous sickle cell anaemia (SCA) (mean age 12 (range 3-32) years old) in good health at the time of the study who showed no infectious complication. (2) 50 patients (mean age 13 (range 4-29) years old) with normal haemoglobin rate. The global response revealed a significant increase in levels of CD3+ (P=0.04) and CD8+ (P=0.04) cells when compared with the control group, there was no significant difference in levels of CD4+ cells (P=0.05) between the two groups. However, there was a relationship between T-cell subpopulation levels and spleen status. The average values of T-cell subsets (CD4+ and CD8+) in patients with SCA-induced splenic defects (asplenic, splenomegaly or splenectomized patients) were significantly reduced when compared to SCA patients with normal spleens and the control groups. These data show that T-cell activity was reduced in patients with splenic defects. A correlation between splenic status and a perturbed host defence system in patients with SCA suggests that monitoring T-cell subsets might have prognostic value in the course of sickle cell disease.

[The impact of chemotherapy on haematological and biochemical profiles during malignant blood diseases in Abidjan, Côte d'Ivoire]. / Impact de la chimiothérapie sur les profils hématologiques et biochimiques au cours des hémopathies malignes à Abidjan (Côte d'lvoire).

Our research concerned the impact of chemotherapy on the haematological and biochemical profiles of patients diagnosed with malignant blood diseases and receiving treatment in Abidjan. The study covered 57 patients, 26 of whom were receiving treatment. Burkitt's lymphoma was the most common type of malignant blood disease encountered (33%). The proportion of men was slightly higher, at 54%, and the average age of patients was 26. Hyperleucocytosis, anaemia and medullar blastosis were the most common blood disorders. The tumours arising from hyperleucocytosis and medullar blastosis caused increases in proteins from inflammatory reaction. The increase was moderate for alpha 1 globulins and haptoglobin and high (at least twice the reference levels) for C Reactive Protein (CRP) and orosomucoid. Full remission was only achieved in the cases of Burkitt's lymphoma, in which the haematological and biochemical parameters reached near-normal levels following treatment. In cases of chronic myeloid leukaemia the treatment lowered the hyperleucocytosis but the high rate of CRP might indicate that the disease was reaching a more acute phase. In the cases of acute leukaemia, chemotherapy did not achieve full remission: the alpha 1 globulins, including orosomucoids, were the most sensitive proteins to treatment. Even though the rate of CRP was lowered, it remained high in all cases of acute leukaemia. Neither haematological nor biochemical data proved superior to the other in monitoring the effectiveness of the treatment or the gradual return of the disease. It would be beneficial to combine them in order to obtain a clearer assessment of the effectiveness of chemotherapy.