Contributions
Abstract: PB1469
Type: Publication Only
Session title: Bone marrow failure syndromes incl. PNH - Clinical
Background
Hemophagocytic Lymphohistioctosis(HLH) is characterised by fever, splenomegaly, cytopenias,hepatitis, altered mental status and neurological involvement. There is marked increase of Histiocytes in bone marrow.
Aims
To study epidemiology, clinical features, laboratory findings & diagnosis of underlying disease associated with HLH and also to assess their response to the treatment
Methods
We enrolled 53 consecutive patients diagnosed with HLH from January 2012 to march 2015 who satisfied the HLH 2009 diagnostic criteria and were between the age of 1 month and 15 years
Results
Most children belonged to age group 31 days -12 months (47.16%). Mean age was 35 months, male (54.71%) children were slightly more affected than female (45.29%) children . The most common presenting symptom in our series was fever (96.2%) and most common sign was pallor (100%). Bone marrow hemophagocytes were demonstrated in 92.15%. Raised S.Ferritin levels >500ng/ml was seen in 98.11% with mean ferritin levels of 4146ng/ml. Transaminitis was seen in 84.9%.Bicytopenia was noticed in 62.26% and pancytopenia in 35.84%. With respect to etiology, Infection was the most common, noticed in 75% of cases. Dengue was found in 14 patients (26.1%). Primary HLH was seen in 5 (9.5%) of children. Out of 53 children enrolled in the study only 47 children received some form of therapy, with prephase steroid alone therapy (n=31) (65.9%) had a improvement in clinical parameter, however on follow up 3 (9.6%) children died and 1 left against medical advice. Among 16 children who had no improvement with steroids were advised complete HLH protocol, but only 12 (25.5%) children parents gave the consent, during course of therapy 7 children died (14.8% in 47) (58.3% in 12 who completed protocol), 3children discharged against medical advice (6.3%) at the time of completion of protocol only 2 (4.2% in 47) (16.6%) in 12 who completed protocol.
Conclusion
Infection associated HLH has better outcome with early diagnosis and prompt treatment. Primary HLH should be suspected and genetic analysis should be sent for patients <3 yrs, with history of consanguinity and sibling death due to similar condition, those presenting with CNS signs and symptoms & patients who deteriorated rapidly on treatment with Dexamethasone alone
Keyword(s): Bone marrow failure
Abstract: PB1469
Type: Publication Only
Session title: Bone marrow failure syndromes incl. PNH - Clinical
Background
Hemophagocytic Lymphohistioctosis(HLH) is characterised by fever, splenomegaly, cytopenias,hepatitis, altered mental status and neurological involvement. There is marked increase of Histiocytes in bone marrow.
Aims
To study epidemiology, clinical features, laboratory findings & diagnosis of underlying disease associated with HLH and also to assess their response to the treatment
Methods
We enrolled 53 consecutive patients diagnosed with HLH from January 2012 to march 2015 who satisfied the HLH 2009 diagnostic criteria and were between the age of 1 month and 15 years
Results
Most children belonged to age group 31 days -12 months (47.16%). Mean age was 35 months, male (54.71%) children were slightly more affected than female (45.29%) children . The most common presenting symptom in our series was fever (96.2%) and most common sign was pallor (100%). Bone marrow hemophagocytes were demonstrated in 92.15%. Raised S.Ferritin levels >500ng/ml was seen in 98.11% with mean ferritin levels of 4146ng/ml. Transaminitis was seen in 84.9%.Bicytopenia was noticed in 62.26% and pancytopenia in 35.84%. With respect to etiology, Infection was the most common, noticed in 75% of cases. Dengue was found in 14 patients (26.1%). Primary HLH was seen in 5 (9.5%) of children. Out of 53 children enrolled in the study only 47 children received some form of therapy, with prephase steroid alone therapy (n=31) (65.9%) had a improvement in clinical parameter, however on follow up 3 (9.6%) children died and 1 left against medical advice. Among 16 children who had no improvement with steroids were advised complete HLH protocol, but only 12 (25.5%) children parents gave the consent, during course of therapy 7 children died (14.8% in 47) (58.3% in 12 who completed protocol), 3children discharged against medical advice (6.3%) at the time of completion of protocol only 2 (4.2% in 47) (16.6%) in 12 who completed protocol.
Conclusion
Infection associated HLH has better outcome with early diagnosis and prompt treatment. Primary HLH should be suspected and genetic analysis should be sent for patients <3 yrs, with history of consanguinity and sibling death due to similar condition, those presenting with CNS signs and symptoms & patients who deteriorated rapidly on treatment with Dexamethasone alone
Keyword(s): Bone marrow failure