Linfohisitiocitosis Hemofagocitica
Páginas: 2 (262 palabras)
Publicado: 28 de agosto de 2011
HLH
BACKROUND: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon haematologic disorder clinically manifests as fever, splenomegaly and jaundice, withlaboratory findings of lymphocytosis and histiocytosis and has the pathologic finding of hemophagocytosis. We present our case series in a Mexican Hospital in 3years.
Aim: Determine the clinical experience in HLH between 2006 and 2009. Methods: We reviewed charts of all patients diagnosed according to the HLH 2004 criteria inthe Hospital Infantil de Mexico Federico Gomez between 2006 and 2009. We analyzed clinical course and outcome.
Results. Ten patients have been diagnosed withHLH. 40% are female and 60% are male. Birth weight was 3.2Kg (2.4-4). Hematological parameters: Hb 7.2 mg/dl (4.2-13.5); WBC 5171 cel/dl (600-16400); platelets 39200(16000-68000); Cholesterol 173.29 gr/dl (123-323); triglycerides 379.71 mg/dl (31-897). All received HLH 2004 protocol. In tree cases we use gammaglobulin assupport treatment. Two patients were classified as genetic on basis on affected siblings. The other 8 were classified as secondary and we were able to document theinfectious agent. In 2 patients salmonella was present in hemoculture, in 4 Ebstein Barr virus, in one respiratory sintitial virus and in one hystoplasma. Two patientsdied early of sepsis. 30% of the rest have completed treatment but all patients who are in treatment have complete clinical as well as laboratory remission.Conclusions: HLH-2004 criteria has been useful in our center to diagnose and seem to cure patients with low toxicity. Results are premature to establish conclusions.
BACKROUND: Hemophagocytic lymphohistiocytosis (HLH) is an uncommon haematologic disorder clinically manifests as fever, splenomegaly and jaundice, withlaboratory findings of lymphocytosis and histiocytosis and has the pathologic finding of hemophagocytosis. We present our case series in a Mexican Hospital in 3years.
Aim: Determine the clinical experience in HLH between 2006 and 2009. Methods: We reviewed charts of all patients diagnosed according to the HLH 2004 criteria inthe Hospital Infantil de Mexico Federico Gomez between 2006 and 2009. We analyzed clinical course and outcome.
Results. Ten patients have been diagnosed withHLH. 40% are female and 60% are male. Birth weight was 3.2Kg (2.4-4). Hematological parameters: Hb 7.2 mg/dl (4.2-13.5); WBC 5171 cel/dl (600-16400); platelets 39200(16000-68000); Cholesterol 173.29 gr/dl (123-323); triglycerides 379.71 mg/dl (31-897). All received HLH 2004 protocol. In tree cases we use gammaglobulin assupport treatment. Two patients were classified as genetic on basis on affected siblings. The other 8 were classified as secondary and we were able to document theinfectious agent. In 2 patients salmonella was present in hemoculture, in 4 Ebstein Barr virus, in one respiratory sintitial virus and in one hystoplasma. Two patientsdied early of sepsis. 30% of the rest have completed treatment but all patients who are in treatment have complete clinical as well as laboratory remission.Conclusions: HLH-2004 criteria has been useful in our center to diagnose and seem to cure patients with low toxicity. Results are premature to establish conclusions.
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