A 9 year and 7 month old Korean ,girl with hereditary elliptocytosis has Presented.
She has complained of growth retardation, severe anemia, abdominal distension and
intermittent fever since 2 year of age. On a(Emission, pallor and generalized jaundice
were marked and: Hb: 6.5 gm%, Hct: 19%, R.B.C.: 2.04 millions/Cumm, reticulocyte: 8%
and elliptocyte: 99% on peripheral blood smear were noted. Father's blood also revealed
80% elliptocytes, but mother intact. Peripheral blood smears of three siblings showed
normal blood picture. Survival half of erythrocytes and external counting of organs were
measured with Cr-51 tagged red blood cell and resulted T 1/2: 8.5 days and S/L ratio:
2.4:1 which were between range of indication of splenectomy. On 50 days after
admission, an abrupt crisis was occurred and then emergency splenetomy was don. On
follow-up check at 5 months after splenectomy, peripheral blood showed almost normal
except slight elliptocytosis. Pathogenesis and pertinent literatures were briefly reviewed.
|