Macrophage Activation Syndrome: A case series; From Sepsis to Malignancy: Early diagnosis and treatment

Abstract

INTRODUCTION: Haemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome(MAS) is a syndrome of fulminant cytokine storm leading to multiorgan dysfunction and high mortality rate. HLH may be Familial or Primary(fHLH) and Secondary (sHLH).FHLH is due to mutation in gene coding for perforin or NK cell of CD8 lymphocytes. sHLH may be associated with hematological malignancies, autoimmune disorders like SLE,Still’s diseae, kawasaki disease, infections and sepsis of various etiology starting from bacteria ,viral protozoal ,fungal and zoonotic infection. Main presenting features are fever, hepatospleenomegly, cytopenia,high ferritin level, high serum triglyceride and haemophagocytosis in bone marrow spleen or lymphnode. MATERIAL AND METHOD: In this series we describe Five cases ,(two cases of SLE,one B-Cell lymphoma, one case of scrub typhus,one case of kleibsiella pneumoniae presenting as sepsis ) with their clinical, laboratory investigations, management and outcome with special correlation of ferritin level and HS score with the prognosis. All patients managed with I.V.Methyl prednisolone 30 mg/kg/day for three consecutive days and out come is assessed. CONCLUSION: MAS in SLE and sepsis is a life threatening unrecognised condition, early diagnosis and treatment can increase the survival rate by many folds . a drop of ferritin level by 15% after 48 hrs of treatment is a surrogate marker of good prognosis. From H scoring cut off value 169 corresponds to sensitivity of 93% and specificity of 86%.