Secondary Hemophagocytic Lymphohistiocytosis: Morphological Findings of Spleen and Bone Marrow with Discussion of Management of an Atypical Case
Journal Title: Journal of Medical Science And clinical Research - Year 2015, Vol 3, Issue 7
Abstract
Hemophagocytic lymphohistiocytosis is syndrome due to abnormal and exaggerated immune response to infectious and non-infectious agents. The most common presentation is fever with palpable splenomegaly and pancytopenia. In this case, a young thin girl presented with moderate grade fever and huge splenomegaly (spleen palpable 24 cms below left costal margin) with pancytopenia with severe normocytic anemia. All investigations including serological markers, parasitic a, fungal and bacterial cultures done to know cause of infection , were negative. However, her bone marrow and splenic aspirates were strongly positive for hemophagocytosis. These morphological findings were also confirmed by biochemical results of high triglycerides and low plasma fibrinogen level. This rare case presented to hematology centre and finally diagnosed as hemophagocytic syndrome and treated with HLH-94 protocol, she became afebrile just after one cycle and her splenic size is decreased gradually and pancytopenia is corrected gradually. Now after completion of six cycles of therapy, at the time of writing of paper , she is asymptomatic from more than one year , however she still has mild splenomegaly and mild anemia, but her WBC and platelet counts are corrected completely. She is leading normal transfusion independent and afebrile life. Importance of FNAC of Spleen and bone marrow aspirate in diagnosis of this disease is especially shown in this paper.
Authors and Affiliations
Subhash Chandra Jha
Keywords
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