HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SYNDROME: HOSPITAL AMPANG EXPERIENCE
Wong Chin Hau
Hospital Ampang <
Background: Haemophagocytis lymphohistiocytosis (HLH) is a rare but life threatening immune dysregulatory disorder with various presentations. It can be familial or secondary to variety of triggers.
Materials and methods:This is a retrospective study of all cases treated at Hospital Ampang from 1st January 2007 till 31st December 2014. Cases are traced through Hospital Information System.
Results:Total 17 cases were reported including15 new cases and 2referral cases for allogeneic stem cell transplant. Patient’s age ranges from 16 to 61 year old. Median age is 42.9 year old. Duration from onset of symptoms till diagnosis ranges from 3 days to 6 months. 12 cases fulfilled at least 5 out of 8 diagnostic criteria based on HLH 2004 Trial, 2 cases fulfilled 4 criteria, 2 cases fulfilled3 criteriaand 1unknown. This unknown case isa transplant referral from other hospital. Frequency of diagnostic criteria is as follow: fever >38.5C16/16 (100%), Haemoglobin <9g/dL11/16 (68.8%), platelet 100x109/L14/16 (87.5%), neutrophil <1x109/L 11/16 (68.8%), splenomegaly9/16 (37.5%), fasting hypertriglyceridemia>265mg.dL10/14 (71.4%), hypofibrinogenaemia<150mg/dL4/13 (30.8%), ferritin > 500ng/ml16/16 (100%). Hepatitis/ liver: 14/16 patients (87.5%). 2 cases have unexplained low GCS level and 1 case has bilateral proximal lower limb weakness. Renal failure:8/17 patients (47%). 7/8 patients need haemodialysis support. 11 cases were reported secondary to infection (EBV (4), EBV with aspergillosis (2), Cytomegalovirus (1), CMV with non-tuberculous mycobacterium (1), CMV with concomittent aspergillosis, mycobacterium tuberculosis, Riketsial, and mycoplasma infection (1) ,klebsiella (1), and leptospirosis (1)). 3 cases are associated with malignancies (Subcutaneous Panniculitis like T cell lymphoma (1), hairy cell leukaemia (1), and suspected lymphoma (1)). 3 cases have unknown causes. 6 cases were treated with HLH 2008 protocol. Various chemotherapyregimens are used for other cases include dexamethasone+vincristine (1), dexamethasone+ Vinblastine+ cyclosporine (2), etoposide+ vinblastine (1), vinblastine (1),Rituximab+ cyclosporine+dexamethasone (2),dexamethasone+ cyclosporine+ cyclophosphamide (1). 1 patient died before treatment.One of thetransplant referral who has relapse, treated and is in remission was successfully transplanted. Another case has refractory disease anddied during conditioning chemotherapy. Mortality rate is 77.78% (14/ 17 cases). 3 survivors include the transplanted patient, a 28 year old lady with unknown cause and treated with HLH 2004 protocol and 58 year old man with EBV and treated with vinblastine. Both do not haveinfection, hepatitis, or renal failure. Both of them do not relapse.All 3 patients who have unexplained neurological symptoms died. All 7 patients who required haemodialysis support and all 14 patients with hepatitisdied.
Conclusion:HLHhas poor prognosis andprompt diagnosis is difficult due to non-specific presentation. Strict adherence to HLH 2004 Trial diagnostic criteria will lead to underdiagnoses as certain criteria only presence when the disease progresses.Most of our cases are secondary HLH. EBV is the commonest infective cause (63.6%) and overall cause (41.1%). Hepatitis is common presentation. Hepatitis, neurological involvement and renal failure requiring haemodialysis carry poorer prognosis.
Keywords: HLH, allogeneic stem cell tranplant