Abstract
Hemoglobin D is a relatively rare disease first reported in 1951. We present the first reported case of Hemoglobin DC disease. This is a case of a Hemoglobinopathy with DC disease in a woman with a previous diagnosis of Hemoglobin SC disease. A 19-year-old woman presented to the Adult Hematology clinic at a tertiary care hospital in Northwest Louisiana for transition of care from Pediatric Hematology for a diagnosis of Hemoglobin SC disease diagnosed at the age 4. Historical data suggested no avascular necrosis, acute chest syndrome, and very few episodes of pain crisis. She has never taken hydroxyurea. Laboratory work showed persistently normal hemoglobin and white blood cell counts. All sickle cell preparations in the past were negative. Computerized tomography scan of the abdomen was reviewed and showed a spleen grossly normal in size and appearance. Given the incongruent clinical picture for sickle cell disease, repeat hemoglobinopathy evaluation with Capillary electrophoresis and confirmatory acid electrophoresis (to differentiate hemoglobins that co-migrate with Hemoglobin S) showed a probable double heterozygote for Hemoglobin D and C with suspected coexistent alpha thalassemia minor based on red blood cell indices. This case confirms the importance of the required confirmatory method to ensure a correct diagnosis since a misdiagnosis can lead to numerous adverse clinical or psychological effects for patients.
Highlights
A 19-year-old female presented for transition of care from Pediatric Hematology to Adult Hematology
She was diagnosed with Hemoglobin SC disease with coexistent α thalassemia at the age 4 on cellulose acetate hemoglobin electrophoresis
Disease such as acute chest syndrome and pain crisis, presence of intact spleen, and normal hemoglobin was what made the original diagnosis of sickle cell anemia questionable
Summary
A 19-year-old female presented for transition of care from Pediatric Hematology to Adult Hematology. She was diagnosed with Hemoglobin SC disease with coexistent α thalassemia at the age 4 on cellulose acetate hemoglobin electrophoresis She reported two episodes of possible vasoocclusive pain crisis in the past, with no history of any sickle cell anemia complications including acute chest syndrome, splenic sequestration, and avascular necrosis. Disease such as acute chest syndrome and pain crisis, presence of intact spleen, and normal hemoglobin was what made the original diagnosis of sickle cell anemia questionable. DD disease are typically clinically and o hematologically silent or may present with -c mild hemolytic anemia As a result, these patients do not seek medical attention.[8] n Hemoglobin D acquires o clinical significance when it occurs in N combination with either β thalassemia or Figure 2.
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