Hemophagocytic Syndrome Associated with Hematologic Malignancies

Abstract

Hemophagocytic syndromes may occur in patients of all age groups. Secondary HS is more frequent than primary (familial) and is usually described in patients with an underlying immune disorder. This clinicopathological entity is the result hemophagocytosis of hemopoetic cells due to activation of morphologically benign macrophages in the bone marrow. Clinical symptoms include fever, hepatosplenomegaly, severe cytopenias, dyslipidemia, and frequent coangulopathy. The prognosis is dismal. Hematologic malignancies are often involved in HS, which may present at any disease phase. Non-Hodgkin lymphomas and less frequently Hodgkin disease have been associated with HS. Lymphoma associated hamophagocytic syndrome (LAHS) accounts for 40-50% of HS where an underlying condition can be defined. NK/T and T peripheral lymphomas are responsible for 80% of LAHS. As far as B-cell lymphomas are concerned, intravascular usually present with LAHS. The pathogenesis of HS is not fully understood, but it seems to differ between T- and B-cell lymphomas. EBV is thought to have an important part in the pathogenetic procedure, since it has been detected both in Hodgkin and Non-Hodgkin lymphomas presenting with HS. Treatment decisions depend upon the underlying condition and its phase. However the most acceptable treatment option is currently immunochemotherapy followed by myeloablative stem cell transplantation.