HLH is one of those conditions that often hides in plain sight- a rare, aggressive and potentially fatal hyperinflammatory autoimmune syndrome. HLH can be primary (genetic) or secondary (causes-infection, autoimmune diseases or malignancy). What makes it challenging is that the symptoms overlap with many common illnesses (nutritional deficiencies, aplastic anemia, infections causing marrow suppression, toxin exposure, malignancies like-leukemia, lymphoma). This makes the diagnosis of HLH difficult and the consequences escalate rapidly. Hence, leading to delay in
adequate management & care to the patient.
Risk Factors-
-Chronic infections
-immunosuppression
-underlying systemic diseases
Signs & symptoms-
-persistent high grade fever (of unknown origin)
-hepatosplenomegaly
-jaundice
-cytopenias
-elevated ferritin
-sometimes coagulation abnormalities
Patients often appear extremely unwell without a clear cause - a red flag every clinician must pay attention to.
Diagnosis relies on fulfilling specific criteria and after ruling out all common causes. Because time is crucial, starting treatment before all criteria are met often helps in better prognosis.
Management revolves around dampening the immune storm using corticosteroids, immunosuppressants and addressing underlying triggers.
In one of the case I came across, was a 60 year old man with diabetes and past history of liver abscess, presented with month long fever, jaundice, weakness and hepatosplenomegaly. Initial treatment failed, but bone marrow findings and significantly elevated ferritin eventually pointed to HLH after ruling out all other causes. In his case trigger was found to be Histoplasmosis, a fungal infection. Early initiation of dexamethasone and itraconazole led to gradual recovery, underscoring how timely recognition can save a life.
HLH may be rare, but awareness is the first step towards prevention of delayed diagnosis and better prognosis.
MBH/PS
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