hemoglobin SC disease

Introduction

Sickling disorders less severe than hemoglobin SS (sickle cell) disease.

Pathology

• splenomegaly rather than splenic atrophy generally occurs
• rare vasoocclusive crises (painful crises)
• rare aseptic necrosis of bone
• overactivity of SLC12A4 may contribute to erythrocyte dehydration

Clinical manifestations

• hematuria (painless)

Laboratory

• complete blood count (CBC)
  • blood hemoglobin variable (10-15 g/dL)
  • MCV >= 75 fL
• peripheral smear
  • fat sickle cells
  • target cells
• reticulocyte count: reticulocytosis (case: 4.5%)
• hemoglobin electrophoresis:
  • Hgb S (50%), Hgb C (50%), Hgb A (0%)
• hemoglobin genotyping

More general terms

• hemoglobinopathy
• hemolytic anemia

Patient information

hemoglobin SC disease patient information