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Hypogammaglobulinemia and hemophagocytic syndrome associated with lymphoproliferative disorders
Author(s) -
Ezdinl Ediz Z.,
Kucuk Omer,
Chedid Antonio,
Sinclair Thomas F.,
Thomas Korathu,
Singh Satinder,
Sarpel Suleyman,
Jovanovic Lillina
Publication year - 1986
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19860301)57:5<1024::aid-cncr2820570526>3.0.co;2-h
Subject(s) - hypogammaglobulinemia , pancytopenia , medicine , immunology , hemophagocytosis , lymphoproliferative disorders , antibody , monocyte , histiocyte , lymphoproliferative response , hypergammaglobulinemia , in vitro , lymphoma , biology , bone marrow , peripheral blood mononuclear cell , biochemistry
Four patients with lymphohistiocytic disorders had or subsequently experienced severe hypogammaglob‐ulinemia and pancytopenia due to hemophagocytosis. The percentages of B‐ and T‐lymphocytes and the ratios of helper (OKT4) cells to suppressor (OKT8) cells in the peripheral blood were variably altered. Mitogenic response to pokeweed mitogen and phytohemagglutinin was depressed but could be restored to near normal by the in vitro addition of indomethacin or interleukin‐2. The half‐life of intravenously administered immunoglobulin was markedly shortened. The data indicate that hyperactive monocytes/histiocytes are capable of simultaneously ingesting apparently normal blood cells and immunoglobulin, leading to pancytopenia and hypogammaglobulinemia. The monocytes with suppressor activity (which could be abrogated with indomethacin or interleukin‐2) appeared to additionally contribute to the hypo‐gammaglobulinemia, possibly by interfering with the terminal differentiation of the B‐lymphocytes. Hemophagocytosis occurs frequently in histiocytic and occasionally in lymphoproliferative disorders or viral diseases. More frequent and serial determination of serum immunoglobulin levels in such situations may lead to the discovery of additional cases.