Hemoglobin E disease, transient erythroblastopenia
Journal Title: Αρχεία Ελληνικής Ιατρικής - Year 2015, Vol 32, Issue 6
Abstract
were observed. Two weeks after the fever started, the patient was clinically normal. As hypochromia, microcytosis, numerous target cells and other significant morphologic alterations of red cells were observed, classical electrophoresis excluded β-thalassemia syndromes. Red cell survival was slightly reduced (19 days) and the osmotic fragility was decreased (MCF 3.45 g/L). Hemoglobin variant study by high performance liquid chromatography (HPLC) revealed no hemoglobin A and Hb A2+E of 7.8%.An 18-year-old woman presented to the outpatient clinic because of fever, chills, malaise and symptoms from the upper respiratory and gastrointestinal tract. Five days before her admission she presented sore throat, cough and fever up to 38 oC. She was treated with amoxicillin, without any improvement. Subsequently, abdominal pain with vomiting and diarrhea, weakness and dyspnea on slight exertion were added and, as the fever increased up to 39 oC, the patient came to the hospital. Her past medical history was unremarkable. Physical examination on admission, revealed pale skin and mucosae, a faint, maculopapular rash in the extremities, cervical and supraclavicular microlymphadenopathy (smaller than 0.5 cm in diameter, painless and mobile) and a mild non-tender splenomegaly (3 cm below costal margin). The liver was not palpable. Patient’s temperature was 38.2 oC, while her pulse rate was 98/min and the respiratory rate was 26/min. Fundoscopy and eurological examination were unremarkable. Her hematological profile was as follows: Ht 18.6%, Hb 6.5 g/dL, MCV 62 fL, MCH 21.6 pg, reticulocytes 0.01%, WBC 3.6×109/L (neutrophils 48%, lymphocytes 44%, monocytes 7%, eosinophils 1%) and platelets 158×109/L. The peripheral blood smear is shown in figures 1 and 2. Serum biochemistry was as follows: BUN 0.18 mg/dL, creatinine 1.2 mg/dL, total bilirubin 0.80 mg/dL, SGOT 35 IU/L, SGPT 40 IU/L, alkaline phosphatase 128 IU/L, γGT 25 IU/L, LDH 490 IU/L, uric acid 5.2 mg/dL and total proteins 6.1 g/dL with normal electrophoretic diagram. The bone marrow aspiration findings are shown in figures 3–5. Symptomatic therapy was administered, whereas fever and other symptoms disappeared after three days, while reticulocytosis and a mild leukocytosis
Authors and Affiliations
J. ASIMAKOPOULOS, L. PAPAGEORGIOU, V. TELONIS, A. ZANNOU, P. ARAPAKI, T. GIANNIKOS, E. NIKOLAOU, G. DRYLLIS, I. KONSTANTINOU, M. ANGELOPOULOU, K. KONSTANTOPOULOS
Keywords
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