วิกฤตการสร้างเม็ดเลือดระหว่างมีไข้จากการติดเชื้อไข้หวัดใหญ่สายพันธุ์ บี ในผู้ป่วยโรคฮีโมโกลบินคอนสแตนท์สปริง

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สมชาย อินทรศิริพงษ์
จิราวดี น้อยวัฒนกุล

Abstract

Introduction: Aplastic crisis is an uncommon complication during infection in any chronic hemolytic disease and it has been hardly found in a case of hemoglobin Constant Spring (Hb CS) disease.


Objective: To present a case of aplastic crisis in Hb CS disease during acute febrile illness due to influenza B infection.


Case Report: A 15-year-old Thai woman suddenly developed fever, cough, pallor, and malaise for 5 days. The physical examination revealed temperature 38.6 degree Celsius, and marked anemia with mild splenomegaly. Her blood tests revealed: Hb 6.5 g/dL, WBC 3.9 x109/L, N 54%, L 39%, M 7%, platelet 86 x109/L, MCV 77.7 fL, reticulocyte 0.7%. Hb analysis: Hb (CS)A2A, Hb A2 3.1%, Hb F 1.5%, the PCR tests for alpha thalassemia-1 and -2, and beta thalassemiagenes - all negative but positive for Hb CS genes mutation, ferritin 4,376 ng/mL, direct and indirect anti-globulin tests-negative, influenza type B-positive. She was diagnosed with aplastic crisis during fever, influenza type B and suspected Hb CS disease. She was immediately treated with blood transfusion, oseltamivir and supportive therapies. The fever subsided within two days and she was discharged with regularly oral folic acid and deferiprone therapy. She was well during follow-up every 3 months for four times. The successive blood tests were shown as mean: Hb 9.9 ± 0.3 g/dL, normal WBC and platelet, MCV 77.7 ± 2.0 fL, reticulocyte 5.6 %, ferritin 134.9 ng/mL.


Conclusion: Although a Hb CS disease is usually considered a mild hemolytic anemia, the aplastic crisis that needs the immediate transfusion during fever, can happen as seen in this case.

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รายงานผู้ป่วย (Case report)

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