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Clonal and non‐clonal karyotypically abnormal cells in haemophagocytic lymphohistiocytosis
Author(s) -
KANEKO YASUHIKO,
MASEKI NOBUO,
SAKURAI MASAHARU,
IDO MASARU,
TSDNEMATSU YUKIKO,
MIZUTANI SHUKI,
HATTORI TATSUYA,
SHIMIZU HIROYUKI,
EGUCHI HARUHIKO,
OKA TOSHIAKI,
MIYAKE MUNENORI,
HORIKOSHI YASUO,
SUCHI TAIZAN
Publication year - 1995
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1995.tb03379.x
Subject(s) - biology , immunology , bone marrow , malignancy , epstein–barr virus , cytogenetics , population , karyotype , somatic evolution in cancer , pathology , chromosome , virus , cancer , medicine , genetics , gene , environmental health
We studied chromosomes in bone marrow (BM) or peripheral blood cells of nine patients with haemophagocytic lymphohistiocytosis (HLH); three of them had a family history of HLH and four others underwent concurrent Epstein‐Barr virus (EBV) infection. In addition to a large population of normal mitotic cells, karyotypically abnormal clonal cells were found in two patients, abnormal clonal cells and a nonclonal (single) abnormal cell in one, and nonclonal abnormal cells in three. All the six patients with chromosome abnormalities died of progressive disease; one of them also had EBV infection and EBV‐associated clonal proliferation. Two of three patients with EBV infection and only normal mitotic cells in BM completely recovered from the disease. Although HLH did not show histological and/or haema‐tological evidence of a neoplastic disease, clonal chromosome abnormalities and the fatal clinical outcome found in some of the patients suggest that the disease may be heterogenous and include malignancy. HLH patients with karyotypically abnormal clonal cells in BM should warrant more intensive chemotherapy than that presently being applied to them and should be considered as candidates for BM transplantation.