Seynabou Fall

Light Chain Deposition Disease in Black African, Report of Three Cases from Senegal

Background: Although there are numerous publications on multiple myeloma in black people, it seems sub-Saharan literature particularly Senegalese’ one is silent about Light chain deposition disease (LCDD) which is linked to immunoglobulin light chain deposition in glomerulus. The authors report the first three observations of LCDD collected in Nephrology department of Aristide Le Dantec hospital in Dakar, Senegal. Cases: we report three cases a man and two women of 61, 69 and 47 years old respectively, admitted to the Nephrology department of Aristide Le Dantec hospital for rapidly progressive renal failure in one case and a nephrotic syndrome in 2 other cases. The renal biopsy showed a nodular glomerulosclerosis and immunofluorescence microscopy, revealed deposition of IgG light chains suggesting LCDD. On the other hand, the diagnosis of multiple myeloma of IgG kappa type in 2 cases and IgG lambda type in the other one was done. A combination of chemotherapy (Mephalan Prednisone) and hemodialysis was instituted for all 3 cases. The evolution was marked by the appearance of an end stage renal disease in 2 cases and the third one was expired due to an infected bed sore secondary to a pathological fracture of the neck of the femur. Conclusion: Although the LCDD is rare, the prognosis of this syndrome seems to be poor as more than half of the patients die or progress to ESRD within 2 years.

Profil diagnostique et évolutif du myélome multiple au Sénégal: étude monocentrique de 2005 à 2016

Introduction Accessibility to innovative multiple myeloma therapies is limited in sub-Saharan Africa. This study aimed to describe the diagnostic and evolutionary features observed during treatment of our patients with myeloma. Methods We conducted a retrospective, descriptive, analytical study (2005 - 2016) of patients with myeloma included in the study based on International Myeloma Working Group (IMWG) Criteria (2003,2014) at the Hopital Aristide Le Dantec (Senegal). Results We collected data from 136 medical records (69 men, 67 women) of patients with an average age of 59 years ± 10.1 years, who were less than 65 years of age in 69.1% of cases. Tell-tale signs included bone pain (96.3%), renal failure (36.8%), infection (23.5%), pathological fracture (17.6%), spinal cord compression (16.9%) and malignant hypercalcaemia (16.2%). Isotopic antiglobulin test showed that anti-IgG could be detected in 61.3% of cases and Kappa in 65% of cases. Patients were classified stage III (59.4%) and I-II (40.6%)of the index staging system. The median survival of patients under conventional traitement (Méphalan-Prédnisone: 67.6%, innovative: 5.9%) was 20 months (1-78 months). Survival rates are better in the absence of neurological and infectious complications and for patients with score I-II of the index Staging System. Conclusion In our study, multiple myeloma was frequently diagnosed before age 65, at advanced stage of tumor mass. Early detection and access to adequate therapies could improve overall survival.Introduction Les thérapeutiques innovantes du myélome multiple sont peu accessibles en Afrique subsaharienne. Le but de cette étude est de décrire les particularités diagnostiques et évolutives observées dans notre pratique de prise en charge des myélomateux. Méthodes Une étude rétrospective (2005 - 2016) descriptive à visée analytique, mené à l’hôpital Le Dantec (Sénégal) a concerné les myélomateux inclus selon les critères de l’International Myeloma Working Group (2003, 2014). Résultats Ont été colligés 136 dossiers (69 hommes, 67 femmes) de patients d’âge moyen 59 ans ± 10,1 ans et qui ont un âge inférieur à 65 ans dans 69,1% des cas. Les signes révélateurs ont été des douleurs osseuses (96,3%), une insuffisance rénale (36,8%), une infection (23,5%), une fracture pathologique (17,6%), une compression médullaire (16,9%), et une hypercalcémie maligne (16,2%). L’isotype a été IgG dans 61,3% des cas et Kappa dans 65% des cas. Les malades ont été classés stade III (59,4%) et I-II (40,6%) de l’index staging system. Sous traitement conventionnel (Méphalan-Prédnisone: 67,6%, innovant: 5,9%), la survie médiane a été de 20 mois (1-78 mois). La survie est meilleure, en l’absence de complications neurologiques, infectieuses et au score I-II de l’Index Staging System. Conclusion Dans notre étude, le myélome multiple est fréquemment diagnostiqué avant 65 ans, au stade de forte masse tumorale. La survie globale peut être améliorée par un dépistage précoce et un accès aux thérapeutiques adéquates.

Torsion chronique d’une rate baladeuse chez un adolescent: à propos d’un cas

Wandering or migrating spleen is a rare anomaly which is usually described in children. Complications, which include pedicle torsion, are common and can be life-threatening. We report the case of a 17 year-old patient with a long past medical history of epigastric pain suffering from wandering spleen with chronic torsion of the pedicle. The clinical picture was marked by spontaneously painful epigastric mass, evolved over the past 48 hours. Abdominal ultrasound objectified heterogeneous hypertrophied ectopic spleen in epigastric position and a subcapsular hematoma. Doppler showed a torsion of splenic pedicle which was untwisted 2 turns and a small blood stream on the splenic artery. Abdominal CT scan with contrast injection showed a lack of parenchymal enhancement of large epigastric ectopic spleen and a subcapsular hematoma. The diagnosis of wandering spleen with chronic torsion of the pedicle complicated by necrosis and subcapsular hematoma was confirmed. The patient underwent splenectomy. The postoperative course was uneventful. We here discuss the contribution of ultrasound and CT scan in the diagnosis of wandering spleen with chronic torsion of the pedicle.

Hematologic and immunologic signs of the lupus disease: the experience of the Dakar's hospital

INTRODUCTION The systemic erythematosus lupus (SEL) or lupic disease is a systemic auto-immune pathology, characterized primarily by the presence of antibodies directed against native antibodies anti-DNA. The circumstances of discovery are variable and polymorphic. The hematologic signs and the immunological disorders constitute criteria of diagnosis of lupic disease. METHODOLOGY It is a multicentric retrospective study from January 1, 1997 to September 30, 2006. Patients were followed up in Internal medicine of Dakar. We appreciate the hematologic and immunological aspects appreciate their prognosis prevalence and their implications with the course the lupic disease. RESULTS 142 lupic patients were included with 125 women and 17 men; the sex ratio is 0.13. The average age was 34 years with extremes of 6 and 72 years. Our patients had hematologic manifestations average in 32,4 % of the cases and immunological in 76,8 % of the cases. The immunological tests showed the presence, of antinuclear antibodies in 97,9 % of the cases, of native antibody anti-DNA in 45,7 % of the cases, the anti-ECT in 86,95 % (with the anti-RNP in 78,3 % of the cases, anti-Sm in 56,5 % and of anti-SSA in 87 % of the cases). Antibodies anti-DNA and anti-ECT were associated with the hematologic demonstrations respectively in 92,0 % and 95 % of the cases (p = 0,08). Total survival in 96 % of the cases is estimated to 7 years. CONCLUSION The circumstances of discovery of the lupic disease are variable. The hematologic signs constitute criteria diagnosis of lupic disease. The accessibility of the hematologic and immunological assessment is necessary for an early diagnosis and an early treatment.