HEREDITARY PYROPOIKILOCYTOSIS Flashcards
1
Q
pathology and genes
A
- Autosomal Recessive
- Severe Haemolytic Anaemia worse in Neonatal period- Enhanced RBC fragmentation on heating in vivo
2
Q
investigations
A
Moderate-Severe AnaemiaPolychromasia
MicrospherocytesBizarre Poikilocytes with bud-like projections
Target CellsHowell Jolly
BodiesThrombocytosis
Platelet Anisocytosis
3
Q
clinical features
A
Asymptomatic, DAT Negative, Anaemia, Reticulocytosis, Jaundice, Gallstones, Splenomegaly, FMH NOT Hypochromic/Microcytic
4
Q
management
A
Supportive Care Splenectomy
