Veteran Afghanistan pacer Shapoor Zadran died on Tuesday (July 7), a day before his 39th birthday, after battling a rare immune disorder called Hemophagocytic Lymphohistiocytosis (HLH) syndrome. He was under treatment at a hospital in New Delhi since January.

The left-arm fast bowler played 44 ODIs and 36 T20Is for Afghanistan between 2009 and 2020. The Afghanistan Cricket Board (ACB), while announcing his death on Tuesday, said Zadran was “one of the foundation-laying figures of Afghanistan cricket”.

With an aggressive approach, the 6’2″ imposing bowler with flowing hair brought a different flavour to the Afghanistan bowling attack. He had once famously said that he liked to be an angry state of mind while bowling to intimidate batsmen.

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Zadran recorded his career-best figures of 4/24 against the Netherlands on his ODI debut in August 2009, and claimed an overall 43 wickets in 44 matches for Afghanistan. He was Afghanistan’s most successful bowler in the 2015 ODI World Cup, taking 10 wickets.

The bowler’s death has brought HLH under focus. Here is all you need to know about this condition.

What is HLH?

HLH is a hyperinflammatory syndrome which compromises the immune system. It is a condition wherein the immune system becomes dangerously overactive, causing white blood cells to build up and attack the body’s own tissues and organs, including the liver, spleen, and bone marrow.

Symptoms

The most common physical symptoms of HLH are a persistent, high fever and an enlarged spleen or liver, which can cause noticeable abdominal swelling or pain. Because the immune system is severely overactive, symptoms often mimic a massive, unmanageable infection and quickly affect multiple areas of the body. Here are some common symptoms:

• Prolonged high fever that does not respond to standard antibiotics.
• Abdominal swelling or pain caused by an enlarged spleen or liver.
• Widespread skin rashes across the trunk and limbs.
• Jaundice.
• Swollen lymph nodes in the neck, armpits, or groin.

Since HLH forces the immune system to destroy the body’s own blood cells, there can be distinct secondary symptoms, including:

• Extreme fatigue, weakness, and pale skin due to anaemia.
• Easy bruising, nosebleeds, or bleeding gums.
• Frequent or worsening infections.
• Neurological changes such as irritability and extreme sleepiness.
• Severe headaches and neck stiffness.
• Altered mental state, confusion, or trouble walking and staying balanced.
• Seizures or loss of consciousness in severe, advancing cases.
• Critical warning signs include respiratory distress, uncontrolled bleeding, and coma.

What causes HLH?

The underlying triggers for HLH can be genetic (primary) or acquired (secondary). While Primary HLH is caused by inherited genetic mutations passed down from parents, Secondary HLH happens in patients with structurally normal genes who experience an overwhelming external trigger that pushes the immune system into an uncontrollable loop.

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Secondary HLH triggers include severe infections (50 per cent of cases) such as the Epstein-Barr virus (EBV), tuberculosis, severe sepsis; malignancies (28 per cent) such as lymphomas and leukemias, autoimmune diseases (12 per cent), and medical interventions such as organ or bone marrow transplants, as well as certain therapies used to treat cancer.

Can HLH be cured?

Yes, but the treatment plan and the long-term success rate depend entirely on whether the disease is Primary or Secondary. Since the condition progresses rapidly, achieving a cure requires immediate, aggressive treatment to calm the immune system before irreversible organ damage occurs.

The only cure for Primary HLH is reportedly a stem cell transplant. For a permanent cure of Secondary HLH, doctors must eliminate the trigger. This means treating the underlying viral infection, managing the autoimmune flare-up, or putting the triggering cancer into remission.

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If HLH is caught early, the body can tolerate the intense therapies. If it is diagnosed late, the primary cause of death is typically multi-organ failure or severe, uncontrollable infections rather than the HLH itself.

Unfortunately for Shapoor Zadran, his body faced severe secondary strain, including a sharp drop in blood cell counts, dengue fever, and repeated severe infections including tuberculosis, while undergoing intensive therapy. He died from severe complications of advanced-stage HLH.