university of BLIDA
Contributions
Type: Publication Only
Background
Hemophagocytic syndrome ( HS) is a clinical-biological entity characterized by exaggerated and uncontrolled immune response. It can be primary or secondary. Serious illness that can develop life-threatening and imposing the initiation of urgent specific treatment.
The existence of a family history, consanguinity and an early start is very suggestive of primitive forms
and reports to various forms of immunodeficiencies.
We relating 6 cases of primary HS hospitalized in our department over a 4 year period.
Aims
records of patients with HS
Methods
Retrospective study from December 2009 to December 2014, 6 children with primary HS were collected, the diagnosis was made on the criteria of histiocyte society, 2007.
Results
There are 4 boys and 2 girls with an average age of 23 months (range 2 months - 6 years).
The clinical picture is dominated by fever and poor general condition (100% of cases), organomegaly (hepato splenomegaly) control tissue infiltration by histiocytes, was found in all cases. Neurological involvement is present in 3 patients.
The pigmentation abnormality is found in 3 cases.
Very contributive biology revealed a healthy cytopenia at least 2 bloodlines (100% of cases), fibrinogen is decreased in 83% of cases associated with a lower TP. Ferritin sensitive and specific test HS is greater than 500 microg / l in 5 cases. Medullary smear showed hemophagocytosis and confirms the diagnosis in all cases.
The underlying etiologies were: 3 cases of syndrome Chediak - Higashi (CHS), 2 cases of familial hemophagocytic lympho- histiocytosis probable, probable case of Purtilo syndrom type 2.
These surges haemophagocytosis were triggered by EBV infection in 3 cases.
The combination of corticosteroids and immunosuppressive have been established in all cases, and in 03 cases an anti CD20, no child has received marrow transplant.
The evolution was fatal for 4 children in an array of multi-visceral failure (2 CHS and 2 family HLH).
Summary
Primary HS remains a serious disease with a poor prognosis, 70 % mortality was observed in our series. Hope lies in the realization of a bone marrow transplant for our patients in Algeria
Session topic: Publication Only
Type: Publication Only
Background
Hemophagocytic syndrome ( HS) is a clinical-biological entity characterized by exaggerated and uncontrolled immune response. It can be primary or secondary. Serious illness that can develop life-threatening and imposing the initiation of urgent specific treatment.
The existence of a family history, consanguinity and an early start is very suggestive of primitive forms
and reports to various forms of immunodeficiencies.
We relating 6 cases of primary HS hospitalized in our department over a 4 year period.
Aims
records of patients with HS
Methods
Retrospective study from December 2009 to December 2014, 6 children with primary HS were collected, the diagnosis was made on the criteria of histiocyte society, 2007.
Results
There are 4 boys and 2 girls with an average age of 23 months (range 2 months - 6 years).
The clinical picture is dominated by fever and poor general condition (100% of cases), organomegaly (hepato splenomegaly) control tissue infiltration by histiocytes, was found in all cases. Neurological involvement is present in 3 patients.
The pigmentation abnormality is found in 3 cases.
Very contributive biology revealed a healthy cytopenia at least 2 bloodlines (100% of cases), fibrinogen is decreased in 83% of cases associated with a lower TP. Ferritin sensitive and specific test HS is greater than 500 microg / l in 5 cases. Medullary smear showed hemophagocytosis and confirms the diagnosis in all cases.
The underlying etiologies were: 3 cases of syndrome Chediak - Higashi (CHS), 2 cases of familial hemophagocytic lympho- histiocytosis probable, probable case of Purtilo syndrom type 2.
These surges haemophagocytosis were triggered by EBV infection in 3 cases.
The combination of corticosteroids and immunosuppressive have been established in all cases, and in 03 cases an anti CD20, no child has received marrow transplant.
The evolution was fatal for 4 children in an array of multi-visceral failure (2 CHS and 2 family HLH).
Summary
Primary HS remains a serious disease with a poor prognosis, 70 % mortality was observed in our series. Hope lies in the realization of a bone marrow transplant for our patients in Algeria
Session topic: Publication Only