Aplastic anemia with small PNH erythrocyte population
Journal Title: Αρχεία Ελληνικής Ιατρικής - Year 2009, Vol 26, Issue 5
Abstract
A 27-year-old Greek male was presented to the outpatient department because of progressive weakness and fatigue, dyspnea on slight exertion and fever. Fever started seven days before, reached 38.5 oC, often peaking twice daily; there were also chills, sweating, and sometimes dry cough. The administration of ampicillin initially and cefuroxime afterwards had no effect. His past medical history was unremarkable. Physical examination on admission revealed only pallor without any sign of infection. There were no hepatosplenomegaly or lymphadenopathy. The body temperature was 37.6 oC, the pulse rate was 125/min and the blood pressure was 110/70 mmHg. His hematological tests showed severe normochromic and normocytic anemia (Ht 19.8%, Hb 6.4 g/dL), leukopenia (white blood cells 1.2×109/L; differential count: neutrophils 18%, lymphocytes 76%, monocytes 5% and eosinophils 1%) and a mild thrombocytopenia with large platelets (78×109/L) although MPV was within normal range. There were no immature forms of red or white series in the blood smears. The reticulocyte count was 0.1%. Coombs reaction was negative and serum haptoglobin levels were normal as well as the coagulation parameters. The erythrocyte sedimentation rate was 38 mm/1 hr. Serum biochemistry was as follows: SGOT50 IU/L, SGPT 52 IU/L, LDH 263 IU/L, γGT 29 IU/L, ALP 128 IU/L,bibirubin 1.12 mg/dL, Fe 180 μg/dL. Serum proteins, ferritin, B12and folate levels, serum electrophoresis and quantitative analysis of immunoglobulins were within normal range. Chest X-rays revealed no abnormality. Blood and urine cultures were unable to detect a bacterial or fungus infection. IgM antibodies titres for EBV, HCV, HSV and VZV were not elevated while IgM antibodies for CMV were positive (titre: 1/480). The tests for rheumatoid factor, antinuclear antibodies, LE cells and cryoglobulins were negative. Antibodies against brucella were not detected. No malaria parasites were present in the peripheral blood smears. Tuberculin skin test was negative. Stool examination for ova and parasites was negative. The abdominal ultrasound was normal. The bone marrow aspirate showed a marked hypoplasia with erythroblastopenia and reduced number of myeloid elements while small foci of sparse cellularity composed mainly of mature lymphocytes were observed. A bone marrow biopsy was performed and showed only yellowish white material, consisting chiefly of fat, fibrous tissue and polyclonal lymphocytes. The detection of CD55 and CD59 red cell populations, using sephacryl gel test microtyping system, revealed a 10% CD55 erythrocytic negativity. Although no bacteria were detected cefotaxime, gentamycin and metronidazole were administered and the fever was controlled after three days. Four units of packed erythrocytes were transfused for the correction of anemia. After the febrile episode the administration of the appropriate therapy for basic disorder was effective as the blood cell count was near normal values two months later and the clinical condition of the patient was excellent.
Authors and Affiliations
A. SARANTOPOULOS, J. ASIMAKOPOULOS, E. PAPAKOSTAS, T. CHATZILEONIDA, N. TRIKILIDIS, M. MICHAEL, O. KAMPOUROPOULOU
Keywords
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