Hemophagocytic syndrome complicating T-cell acute lymphoblastic leukemia with a novel t(11;14)(p15;q11) chromosome translocation

Abstract

A case of hemophagocytic syndrome that developed in a patient with T-cell acute lymphoblastic leukemia (ALL) with a novel chromosome translocation involving 14qll is reported. A 15-year-old boy with T-cell ALL in relapse showed leukemic cells with an abnormal karyotype of 46,XY,-15,t(l1;14)(pl% qll), +der(l5)t(l5;?)(pll;?). Pancytopenia and extensive hemophagocytosis by macrophages in the bone marrow were observed after reinduction chemotherapy and again at the terminal stage. At autopsy, infiltration of such cells was also found in other organs. The findings suggested Occurrence of hemophagocytic syndrome probably associated with cytomegalovirus (CMV) infection. The t(11;14)(p15; q l l ) may be a novel translocation specific for T-cell ALL, and conceivably, the association of T-cell ALL with the histiocytosis in this patient may not have been coincidental.

The mechanism of chromosomal translocation t(11;14) involving the T-cell receptor C delta locus on human chromosome 14q11 and a transcribed region of chromosome 11p15.

“…Identification of chromosomal translocation (t 11; 14) junction The t(11;14) (pl5;qll) abnormality has recently been described in a leukaemia patient (Takasaki et al, 1987) and in a T-cell line RPMI 8402 (Le which was derived from a 16-year-old female patient with acute T-cell leukaemia and has the surface antigen phenotype of an immature thymocyte (Minowada et al, 1982). The DNA of the cell has undergone both TCR,3 and TCR-y gene rearrangement (Greenberg et al, 1986;Lefranc and Rabbitts, 1985;Sangster et al, 1986), and expresses mRNA for 'y and 3 but not TCRa chains (Sangster et al, 1986;Le Beau et al, 1986).…”

Bone marrow biopsy in hemophagocytic syndrome

“…This syndrome can occur as a primary disease i.e., FEL or sporadic hemophagocytic lymphohistiocytosis with welldefined diagnostic clinico-pathological criteria [17] or as a reactive form following an extremely large number of causes either infective [1,3,5,15,20,36,41,56,57,58,59,60,63,72,78], neoplastic [8,10,11,13,14,21,29,33,40,43,45,48,50,51,53,62,65,67,69] or, less frequently, of other origin such as Lupus erythematosus [76], Kikuchi lymphadenitis [73], Kawasaki disease [47], drugs [54] or parenteral nutrition with fat emul- sions [22,61]. Large groups of patients, which are rarely described in the literature, prevalently deal with clinical aspects but include only occasional reports on BMB [2].…”

Pediatric Hemophagocytic Syndromes: A Diagnostic and Therapeutic Challenge

“…In regard to malignancies, HS is mostly encountered in patients with T-cell malignancies, especially T-cell lymphomas [87-89]. It can be present at onset or during the course of treatment and usually implies relapse or escape of the leukemic clone from chemotherapeutic agents [90].…”