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Hereditary spherocytosis: what it is and how it is treated | CION Cancer Clinics
Hereditary spherocytosis is an inherited condition where red cells are round instead of flat, so the spleen destroys them too early. The usual signs are tiredness, pale skin, yellow eyes, a larger spleen and gallstones. Many people with a mild form need only check-ups and blood tests. Folic acid, transfusion or spleen removal are used when it is more severe. Here is what to watch for and what to ask. At CION Cancer Clinics, our haematologist cares for anaemia, bleeding, clotting and inherited blood disorders, with ArogyaSri, CGHS and cashless insurance accepted.
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Medically reviewed by Dr. Basudev PokhrelConsultant Haematologist · last reviewed September 2026, next review due September 2027
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On this page
- What is hereditary spherocytosis, and what does it do?
- What symptoms does hereditary spherocytosis cause?
- How do doctors confirm hereditary spherocytosis?
- What treatment is there, and who needs it?
- What do the words on the report mean?
- What do families often get wrong about it?
- Common questions about hereditary spherocytosis
The short answer
What is hereditary spherocytosis, and what does it do?
Hereditary spherocytosis is an inherited condition where red blood cells are shaped like small balls instead of soft, flat discs. The spleen breaks these stiff cells down too early, which leads to tiredness, yellow eyes and sometimes an enlarged spleen.
Why the shape matters
A healthy red cell bends and folds as it squeezes through the narrow channels of the spleen. A round cell cannot fold, so the spleen traps it and removes it. When cells are removed faster than the bone marrow can replace them, you get anaemia, which means a low haemoglobin. This early breakdown of red cells is called haemolysis.
How it runs in families
In most families it passes from one parent to a child, so a parent, brother or sister often has it too. Sometimes it appears in a child with no family history at all. It is not caught from anyone, and nothing the mother did in pregnancy caused it.
How serious it is varies a lot
Some people never know they have it until a routine blood test. Others need regular care from childhood. Even within one family, one person can be barely affected while another needs treatment.
This page explains the condition in general. It cannot tell you how mild or severe your own or your child's condition is. Only your haematologist can, from your reports and examination.What families notice
What symptoms does hereditary spherocytosis cause?
Most signs come from red cells breaking down too fast. They often come and go, and get worse during an infection.
Tiredness and pale skin
A low haemoglobin means less oxygen reaches the body. Children may tire quickly at play. Adults may feel breathless on stairs or notice pale lips and palms.
Yellow eyes or skin
When red cells break down, a yellow pigment called bilirubin builds up. This jaundice often shows in the whites of the eyes first.
Often seen
- In newborn babies
- During fever or a viral illness
- With dark, tea-coloured urine
A larger spleen
The spleen works harder and can grow. You may feel fullness or a dull ache under the left ribs, or a doctor may feel it on examination.
Gallstones
Extra bilirubin can form stones in the gallbladder, even in teenagers. Pain in the upper right belly after meals is a sign to mention.
Many adults with a mild form have no symptoms at all.Not sure whether this applies to you?
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When it cannot wait
Go to the nearest emergency department the same day, or call 108, if a person with hereditary spherocytosis suddenly turns very pale, is unusually sleepy, breathless or has a racing heart, especially after a viral fever. A newborn whose yellow colour is deepening also needs same-day care. After spleen removal, any fever needs urgent medical review. Tell the team about the diagnosis as soon as you arrive.
Finding out
How do doctors confirm hereditary spherocytosis?
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Family history and examination
The haematologist asks who else in the family has had jaundice, gallstones or spleen removal. They check your eyes, skin and the size of your spleen.
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Complete blood count
This shows your haemoglobin and the size of the red cells. A high MCHC, which means the cells are densely packed with haemoglobin, is a common clue.
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Blood smear
A drop of blood is looked at under a microscope. Small, round cells without the usual pale centre are called spherocytes.
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Tests of red cell breakdown
A reticulocyte count shows how hard the marrow is working. Bilirubin and LDH show how many cells are breaking down.
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A confirming test
An EMA binding test, or sometimes a genetic test, confirms the diagnosis. A Coombs test is usually done to rule out an immune cause, because that is treated very differently.
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Treatment
What treatment is there, and who needs it?
Treatment depends on how severe the condition is. Many people with a mild form need only regular check-ups. The aim is to keep haemoglobin at a level where you can grow, study and work, and to catch problems early.
Folic acid and monitoring
Your marrow makes red cells faster than usual, so it uses more folic acid, a B vitamin. Your haematologist decides whether you need a supplement. Regular blood tests and growth checks in children follow how things are going.
Blood transfusion
A transfusion may be needed in infancy, during a sudden drop in haemoglobin, or in pregnancy. Most people with a mild form never need regular transfusions.
Removing the spleen
Removing the spleen, called splenectomy, stops the early breakdown of red cells. It is considered for moderate or severe forms. It does not suit very young children, or most people with a mild form, because life without a spleen brings a lifelong higher risk of serious infection. Vaccines, and sometimes preventive antibiotics, are needed before and after. The gallbladder may be removed at the same time if there are stones.
Never start or stop folic acid, antibiotics or any medicine on your own. Your treating team sets the plan.On your report
What do the words on the report mean?
- Spherocytes
- Small round red cells seen on the smear. They also appear in other conditions, so they are a clue, not a diagnosis.
- Reticulocytes
- Young red cells. A high count means the marrow is working hard to replace cells being lost.
- Unconjugated bilirubin
- The yellow pigment released when red cells break down. It causes the yellow eyes.
- EMA binding test
- A lab test that measures a protein on the red cell surface. A low result supports the diagnosis.
- Negative DAT or Coombs
- No antibodies were found on the red cells, so an immune cause is less likely.
Commonly believed
What do families often get wrong about it?
"Yellow eyes mean it is a liver problem."
In hereditary spherocytosis the yellow colour usually comes from red cells breaking down, not from liver damage. Treating it as hepatitis with home remedies can delay the right tests. Ask for a blood smear and reticulocyte count.
"More iron tonic will fix the anaemia."
This anaemia is not caused by a shortage of iron. Extra iron without a proven need does not help and can build up in the body. Take iron only if your doctor has found a deficiency.
"Removing the spleen is always the answer."
It helps people with a severe form, but it carries a lifelong infection risk. For most people with a mild form, check-ups are safer than surgery.
"It is a blood cancer."
It is not. Hereditary spherocytosis is an inherited condition of the red cell membrane. It does not turn into leukaemia.
Questions we are asked
Common questions about hereditary spherocytosis
Can my child live a normal life with hereditary spherocytosis?
Most children with a mild or moderate form go to school, play sport and grow into working adults. What matters is regular follow-up, knowing the warning signs, and quick care during infections. Your haematologist can explain how your child's own form is likely to behave, because this varies even within families.
Should my other children and I be tested?
Often yes. Because it usually passes from parent to child, brothers, sisters and parents may have a mild form without knowing it. A blood count and smear is a simple starting point. Ask the haematologist which family members should be checked, and bring their old reports if they have any.
Is hereditary spherocytosis the same as thalassaemia?
No. Both are inherited and both can cause anaemia, but they are different conditions. Thalassaemia affects how haemoglobin is made. Spherocytosis affects the outer layer of the red cell. The tests, the treatment and the advice for family planning are different, so the exact diagnosis matters.
Can I get pregnant if I have it?
Yes, many women with hereditary spherocytosis have healthy pregnancies. Haemoglobin can fall during pregnancy, so tell your obstetrician and haematologist early. They will plan closer blood tests and decide if you need a supplement or a transfusion. Your baby may also be checked for jaundice after birth.
Will removing the spleen stop all the problems?
It usually stops the early breakdown of red cells, so haemoglobin rises and jaundice settles. The cells stay round, though, because the gene does not change. You will need vaccines and must treat every fever seriously for life. Ask your haematologist whether the gain is worth that risk for you.
Does food or diet make any difference?
A balanced diet with green leafy vegetables, dals and fruit supports general health, but no food changes the shape of the cells. Avoid tonics and herbal products that promise to fix the blood. Ask before taking any supplement, including iron, because it may not be what you need.
Why does my child turn yellow every time there is a fever?
Infections speed up red cell breakdown, so more bilirubin is released and the eyes turn yellow. It usually settles as the illness passes. If the child also becomes very pale, drowsy or breathless, do not wait. Take them to an emergency department the same day.
Can CION help if we already have a diagnosis?
Yes. CION's haematology team, led by Dr. Basudev Pokhrel, can review your reports, explain what they mean and plan follow-up. Where surgery or specialised tests are needed, the team coordinates with qualified centres and tells you what to ask. Bring every blood report you have, including old ones.
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Sources
- MedlinePlus Genetics (US National Library of Medicine) — Hereditary spherocytosis
- NHLBI — Hemolytic anemia
- NHS — Spleen problems and spleen removal
- American Society of Hematology — Anemia
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
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