Journal of Blood Disorders & Transfusion

Abstract

Peculiarities of Biermer Disease in a Senegalese Internal Medicine Department: A 6 Year Prospective Study

Diop Madoky M, Berthe A, Toure PS, Ndiaye FSD, Mahamat Y, Leye MY, Diousse P, Adamson P, Leye A and Ka MM

Summary: Biermer’s disease also called pernicious anemia (PA) presents certain peculiarities among black patient such as early age diagnosis and a frequent acquired melanodermia without obvious causes like adrenal insufficiency to explain it. The anemia can also be multifactorial. Patients and Methods: This was a prospective study between May 2007 and June 2013 at the department of internal medicine of the Thies regional hospital, Senegal. All cases that presented with recurrent anemia without gastro intestinal bleeding were recruited. Cases associated with renal disease, tuberculosis, deficiency of acid folic and patients on treatments like amiodarone or alcolisme were excluded from the study. Results: A number of 28 Patients were retained for the study with a mean age 43 years and the sex ratio M/F at 2.1. Average time to diagnosis was 3 years with extreme ages ranging from 2 to 8 years. The diagnosis was suspected in the presence of Neurologic-anemic syndrome and confirmed by low vitamin B12 blood levels and the presence of anti intrinsic factor antibodies. In certain patients, vitamin B12 therapeutic test was contributive. The clinical manifestations were dominated by lower limbs paresthesia in 24 (85.71% of the patients, sensory Ataxia in 3 (10.71%) of the patients, and combined sclerosis of the bone marrow in 1 (3.57%) patient. Glossitis and melanodermia was equally present in 78.57% of all the cases. Association with beta-thalassemia, Plummer Vinson syndrome (SPV) and hyperthyroidism were observed in 1 case. Gastro intestinal hemorrhage was observed in 3 patients among whom one had hemorrhoids specifically the SPV patient. The other 2 cases were diagnosed with bulbar ulcers. Biermer’s disease was also was also seen by deep vein thrombosis in 2 cases (7.14%) and also by cerebral vascular accident in one patient (3.57%). The Anemia was macrocytic in 19 (67.85%) of the cases, normocytic in 7 (25%), and microcytic in 2 (7.14%) of the cases. The mean hemoglobin level was at 5.8 g/dl. Serum vitamin B12 low in 25 (89.28%) of the patients with a mean value at 50 pg/ml. Anti intrinsic factor antibodies were positive in 20 (71.24%) of the cases. In patients who had gastroscopy, the histology showed features of chronic atrophic gastritis in 71.24% of the cases. The treatment comprised whole blood transfusions and supplementations of iron and vitamin b12 parenterally. In all of our patients, there was regression completely of the melanodermia, glossitis and normalization of the hemoglobin. Conclusion: In our study we note that Biermer’s disease in the black patient could present itself by an acquired melanodermia. The anemia could be normo or microcytic depending on other intrinsic causes.