[Clinical characterization of B cell lymphoma-associated hemophagocytic syndrome].
Malignant lymphoma is a major cause of hemophagocytic syndrome (HPS), in which reactive macrophages, phagocytic red blood cells, white blood cells, and platelets proliferate in bone marrow, liver, and spleen. In contrast to T/NK-cell lymphoma-associated hemophagocytic syndrome (T/NK-LAHS), few cases of B-LAHS have been reported; thus, the clinical characterization of B-LAHS remains to be established. We describe here four cases of B-LAHS that include the following features: (1) HPS was the initial presentation; (2) bone marrow involvement with large-cell lymphomas was noted in all cases, despite lack of remarkable lymphadenopathy; (3) no active infection with Epstein-Barr virus as the etiological agent was confirmed; (4) except for the spleen in one case, primary site of lymphoma could not be determined; and (5) serum IL-6, soluble IL-2 receptor, and IFN-gamma- but not TNF-alpha and IL-1 beta-, were significantly elevated. Such characteristics are peculiar to and different from those usually seen in B-cell lymphoma, suggesting that B-LAHS is a unique clinical entity among B-cell lymphomas.
Kawauchi
K
K
Tokyo Women's Medical University Daini Hospital, Department of Medicine.
Ogasawara
T
T
Yasuyama
M
M
D000368:Aged; D015415:Biomarkers / Q000097:blood; D016207:Cytokines / Q000097:blood; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D016393:Lymphoma, B-Cell / Q000150:complications*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013577:Syndrome
D015415:Biomarkers; D016207:Cytokines