Hemolytic Anaemia

What hemolytic anaemia is — in plain words

A healthy red cell lives about 120 days in your bloodstream before being recycled by the spleen. In hemolytic anaemia, that lifespan is dramatically shortened — sometimes to days or even hours. The marrow tries to keep up, working harder than usual, but eventually falls behind, and anaemia sets in.

The breakdown itself produces some characteristic features: a slight yellowing of the eyes (jaundice) from bilirubin released as red cells break down; darker-than-usual urine; and on blood tests, raised LDH, raised indirect bilirubin, low haptoglobin and a high reticulocyte count.

The two big buckets — acquired vs inherited

Acquired causes

Autoimmune hemolytic anaemia (AIHA)

Your immune system makes antibodies against your own red cells. Two main flavours:

• Warm AIHA — antibodies most active at body temperature. The commonest type. Sometimes idiopathic, sometimes associated with lymphoma, lupus, or certain drugs.
• Cold agglutinin disease — antibodies most active at colder temperatures, often causing symptoms in cold weather. Sometimes follows mycoplasma or EBV infection; sometimes associated with a low-grade lymphoma.

Drug-induced hemolysis

Certain medicines can trigger immune destruction of red cells (penicillins, cephalosporins, methyldopa, some others) or direct oxidative damage. Stopping the drug, when identified, is often curative.

Microangiopathic hemolytic anaemia

Red cells are physically sheared as they pass through small vessels coated with abnormal clots or fibrin. Examples include thrombotic thrombocytopenic purpura (TTP), haemolytic uraemic syndrome (HUS), and disseminated intravascular coagulation (DIC). These are emergencies — the blood film shows characteristic fragmented red cells (schistocytes).

Paroxysmal nocturnal haemoglobinuria (PNH)

A rare acquired stem cell disorder where red cells lack protective surface proteins and are destroyed by complement. Treated with C5 inhibitors (eculizumab, ravulizumab) or newer C3 inhibitors.

Infections and other causes

Malaria — a particularly important cause in South Asia — directly destroys red cells. Severe infections, mechanical heart valves, and large haemangiomas can also cause hemolysis.

"When jaundice and anaemia turn up together, the next test is almost always a Coombs. That one result splits the diagnosis into two very different paths — and very different treatments."

Inherited causes

Thalassaemia

Common in South Asia — a genetic condition where one of the haemoglobin chains is produced in inadequate amounts. Thalassaemia trait usually causes only mild anaemia and is often picked up incidentally; it doesn't need treatment but matters for genetic counselling and pregnancy planning. Thalassaemia intermedia needs careful monitoring and sometimes occasional transfusion. Thalassaemia major is a serious condition needing lifelong transfusions and iron chelation, with stem cell transplant as a potential cure in children with suitable donors.

Sickle cell disease

Abnormal haemoglobin makes red cells deform into a sickle shape under low oxygen, causing chronic hemolysis and painful vaso-occlusive crises. Treated with hydroxyurea, transfusions, careful infection prevention, and increasingly with disease-modifying agents and gene therapy at specialist centres.

G6PD deficiency

An enzyme deficiency, especially common in men of Mediterranean, African and South Asian origin, that leaves red cells vulnerable to oxidative stress. Episodes of hemolysis are triggered by certain medicines (some antimalarials, sulpha drugs, nitrofurantoin), fava beans, and infections. Most patients are well between episodes — the key is knowing what to avoid.

Hereditary spherocytosis and other membrane disorders

Red cells with abnormal shapes are removed early by the spleen. Often diagnosed in childhood or young adulthood; some patients benefit from splenectomy.

How we diagnose hemolytic anaemia

1. CBC, reticulocyte count, peripheral blood smear — the smear is critical and tells us a great deal: spherocytes, schistocytes, sickle cells, malarial parasites, all visible to a trained eye.
2. LDH, indirect bilirubin, haptoglobin — together confirm hemolysis is occurring.
3. Direct antiglobulin test (Coombs test) — positive in AIHA, negative in most other causes. The single most important branching test.
4. Haemoglobin electrophoresis (or HPLC) — diagnoses thalassaemia, sickle cell and other haemoglobinopathies.
5. G6PD level — measured between episodes (immediately after an episode, the level can falsely look normal).
6. Flow cytometry for PNH — sensitive and specific.
7. ADAMTS13 activity — when TTP is suspected; an emergency test.
8. Cold agglutinin titre, viral screen, autoimmune screen — directed by the clinical picture.
9. Bone marrow biopsy — occasionally needed, particularly when an underlying lymphoma is suspected. What it's like →

How we treat it — by cause

Supportive care that applies broadly

• Folic acid supplementation in chronic hemolysis (the marrow is working hard and uses more folate).
• Hydration, particularly during acute crises in sickle cell and G6PD.
• Vaccinations — especially against encapsulated bacteria for patients with hyposplenism or after splenectomy.
• Avoidance of known trigger drugs and infections.
• Genetic counselling for inherited disorders, particularly before pregnancy.

What outcomes look like — honestly

Outcomes depend almost entirely on the cause. Drug-induced hemolysis usually resolves completely when the drug is stopped. Warm AIHA responds to first-line treatment in most patients, though relapse can occur. Inherited disorders are lifelong but very manageable with modern care, and stem cell transplant offers cure for selected younger patients with thalassaemia major or sickle cell disease. The microangiopathies, treated early, can resolve completely; treated late, they are dangerous — speed matters.

Daily life with chronic hemolytic anaemia

Many patients live full lives — work, family, travel — with the condition in the background. The main themes are knowing your triggers, keeping vaccinations up to date, staying well hydrated, attending periodic check-ups, and having a clear plan for what to do if a crisis happens. For thalassaemia and sickle cell, we coordinate closely with transfusion services and, where relevant, with transplant centres.

What to bring to your first visit

• Every blood report you can find, including past episodes of anaemia or jaundice.
• Any haemoglobin electrophoresis, G6PD, Coombs or flow cytometry results.
• A list of all medications taken in the recent weeks.
• Family history of anaemia, jaundice, gallstones, or known blood disorders.
• For couples planning pregnancy, both partners' carrier status if known.