Πέμπτη 12 Νοεμβρίου 2020

Haemolytic crisis of hereditary spherocytosis

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A 23-year-old man presented to the emergency department with a 2-day history of fever, nausea, conjunctival pallor and new yellowing of the skin. His medical history was unremarkable. Physical examination demonstrated mild jaundice of the sclera and skin and a palpable spleen. Laboratory testing showed a haemoglobin of 6.6 g/L (reference range 13.7–16.8 g/L), total bilirubin of 7.03 mg/dL (0.4–1.5 mg/dL), direct bilirubin of 0.67 mg/dL (0–0.4 mg/dL) and a reticulocyte fraction of 2.8% (0 .67–1.92%). Ultrasonography (US) and enhanced CT revealed splenomegaly and non-obstructing gallstones (figure 1). A peripheral blood smear test showed spherocytosis. (figure 2). He disclosed that his family history was notable for splenectomy in his father due to hereditary spherocytosis. A diagnosis of haemolytic crisis of hereditary spherocytosis was made based on these clinical findings. The patient received 2 units of packed red blood cells soon after the admission. Hereditary spherocytosis is the most...

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