Systemic hemophagocytosis masking the diagnosis of large cell non-Hodgkin lymphoma.
An 11-year-old female presented with clinical features suggestive of malignant histiocytosis: fever, weight loss, subcutaneous nodules, pulmonary infiltrates, adenopathy, and hepato-splenomegaly. On biopsy, lymph node and bone marrow demonstrated necrosis and extensive hemophagocytosis with no definitive evidence of malignancy: the subcutaneous nodules, however, demonstrated large-cell non-Hodgkin lymphoma. This clinicopathologic picture has been reported in adults, but not in children. Although serum G-CSF, M-CSF, and TNF levels were not elevated in this child, it is possible that other cytokines induced either directly or indirectly by the subcutaneous lymphoma resulted in hemophagocytosis.
Sandlund
J T
JT
Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
Roberts
W M
WM
Pui
C H
CH
Crist
W M
WM
Behm
F G
FG
D001853:Bone Marrow / Q000473:pathology; D002648:Child; D016207:Cytokines / Q000276:immunology; D003937:Diagnosis, Differential; D005260:Female; D005334:Fever / Q000175:diagnosis; D016179:Granulocyte Colony-Stimulating Factor / Q000097:blood; D054747:Histiocytic Sarcoma / Q000175:diagnosis*; D006801:Humans; D008168:Lung / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000276:immunology; D016173:Macrophage Colony-Stimulating Factor / Q000097:blood; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis; D015431:Weight Loss
D016207:Cytokines; D014409:Tumor Necrosis Factor-alpha; D016179:Granulocyte Colony-Stimulating Factor; D016173:Macrophage Colony-Stimulating Factor