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Haemophagosytic limphohistiosytosis (HLH) in adults - clinical features, triggering diseases, prognostic factors and outcomes: Report of thirty-three cases

Liliya Yankova Komsalova & Luis Morillas

Objective: To analyse clinical features, triggering diseases, treatment strategies and prognostic factors in patients with secondary haemophagosytic limphohistosytosis (SHLH).

Methods: We retrospectively analysed thirty-three patients with positive haemophagocytosis bone marrow biopsies, all collected between 1995 and 2015 from two different hospitals.

Results: The average age was 44.39 years with a man/women ratio 1.06/1. The underlying diseases were as follows: Autoimmune diseases (n=11), liver or kidney transplant (n=9), haematological malignancies (n=5) infection (n=5) and solid organ cancer (n=3). The average time from hospitalisation to death was 49.95 days (49.95 ± 39.608). Three different prognostic factors were separately analysed: overall mortality, severe disease (less than two months to death) and extremely severe disease (less than one month to death). Risk factors associated to overall mortality were age >35 years (p<0.011), severe cytopenias such as anaemia (p<0.002), bicytopenia (p<0.007) and pancytopenia (p<0.025), ongoing lung involvement (p<0.012) and sepsis (p<0.044). Risk factors for severe disease were underlying treatment with corticosteroids alone (p<0.013), severe anaemia (p<0.002), neutropenia (p<0.027) and pancytopenia (p<0.016). Severe hypofibrinogenemia (p<0.039), ongoing lung involvement (p<0.022) and a late start to specific treatment (p<0.047) were highly indicative for severe disease, whereas underlying autoimmune disorder (p<0.003) and the simultaneous use of immunosuppressant at the onset (p<0.007) seemed to act as a protective factor for severe disease. Risk factors for extremely severe disease were underlying solid organ cancer (p<0.043), severe cytopenias such as thrombocytopenia (p<0.016), bicytopenia (p<0.016) and pancytopenia (p<0.009), and a late start to specific treatment (p<0.043).

Conclusions: Patients with secondary HLH might have a different prognosis according to the triggering disease. Underlying autoimmune disorder might be related to a better prognosis and malignancy might indicate high mortality. Early recognition and specific treatment is essential for the patient’s survival whereby tight suspicion is necessary for an attempt at curative therapy to be made.