08/07/2026
Former Afghanistan fast bowler has been expired due to HLH....
(Hemophagocytic Lymphohistiocytosis). It’s a rare but very serious condition where the immune system goes into overdrive and starts attacking the body’s own cells.
Normally, immune cells like T-cells and macrophages kill infections, then shut off. In HLH, they *don’t shut off*. They keep multiplying and release huge amounts of inflammatory chemicals called "cytokines".Those overactive immune cells also start eating up other blood cells - that’s called "hemophagocytosis"= blood cell eating. Result: Damage to liver, spleen, bone marrow, brain, and other organs.
(1) Primary / Familial HLH/ Genetic. Usually seen in infants/children 38.5°C for >7 days that doesn’t respond to antibiotics
2. Enlarged liver and spleen
3. Low blood counts - low WBC, low platelets, low Hb = anemia
4. High ferritin - very high, often >500, sometimes >10,000
5. Rash, swollen lymph nodes
6. Neurological issues - seizures, confusion, irritability in babies
7. Bleeding, jaundice
Doctors use 8 criteria. Need 5 out of 8:
1. Fever
2. Splenomegaly
3. Cytopenias in 2+ blood lines
4. High triglycerides / Low fibrinogen
5. Hemophagocytosis in bone marrow
6. Low NK cell activity
7. Ferritin >500
8. High soluble IL-2 receptor
Plus genetic testing for primary HLH.
HLH is life-threatening without treatment. Goal = calm the immune system + treat trigger.
Main protocol:
1. HLH-2004 Protocol: Chemo drugs like Dexamethasone + Etoposide
2. Treat the trigger: Antivirals for EBV, antibiotics for sepsis, treat cancer
3. IVIG, steroids, Cyclosporine
4. Bone Marrow Transplant - Curative for primary/genetic HLH
: The mortality rate of HLH is high, the overall prognosis is poor. Secondary HLH - Infection triggered without treatment: >90% mortality, with proper treatment the mortality rate is 50-70% .
