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Hereditary spherocytosis

Sphaerocytosis hereditaria · Vrozená sférocytóza

What it is

An inherited fault in the red cell's skeleton makes cells round and fragile, so the spleen destroys them early — the commonest inherited anaemia in people of northern European descent (about 1 in 2,000).

Symptoms & signs

  • Anaemia, tiredness
  • yellowing (jaundice), dark urine
  • enlarged spleen
  • gallstones, even in children
  • sudden anaemia with parvovirus infection

🚩 Get help if…

  • call 112 / 911 now Very pale, breathless or faint, especially with a viral illness
  • call 112 / 911 now Fever over 38.5 °C after the spleen was removed
  • see a doctor soon Yellow skin or eyes getting worse
  • see a doctor soon Pain in the right upper belly after meals (gallstones)

Where it strikes

Medical treatment

  • Blood smear, EMA binding test
  • folic acid in moderate and severe disease
  • transfusion in crises
  • Spleen removal for severe disease — ends most destruction; lifelong infection risk, vaccines needed trials support
  • partial splenectomy in young children
  • gallbladder removal if stones

Natural & lifestyle

  • Folate-rich foods
  • seek care fast for fever after splenectomy

Emerging & experimental

  • Partial splenectomy techniques
  • gene studies

Alternative, off-label & anecdotal

Approaches used outside the guidelines, with what their supporters report and what testing has found so far.

  • Iron supplements — not needed; harmful with iron overload from transfusions anecdotal / traditional
  • Vitamin E — antioxidant; untested anecdotal / traditional

ICD codes