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We have studied the expression of cytokine receptors CD25 (IL-2R alpha, 55 kD), CD116 (hGM-CSFR, 145 kD), CD117 (CSFR, 145 kD), CD120a (TNFR, 55 kD), CD120b (TNFR, 75 kD), CD121a (IL-1R, type I, 80 kD), CDw123 (IL-3R), CD124 (IL-4R, 140 kD), CD126 (IL-6R, 80 kD), CD127 (IL-7R, 75 kD), CDw128 (IL-8R), CD130 (gp130 subunit), CDw131 (common beta), CD132 (IL-2Rgamma), CD134 (OX40) and also CD95 (Fas antigen) on the lymphoid leukaemic cells. Cells from peripheral blood or bone marrow of 24 patients with disorders in lymphoid lineage mostly included acute lymphoid leukaemias (with a high leukocyte count and percentage of blasts) were analysed for the expression of surface membrane molecules by the immunofluorescence method evaluated by flow cytometry. The findings indicate that some monoclonal antibodies have a reactivity against cytokine receptors of pathological cells in individual cases, but with very variable qualitative and quantitative expression (number copies/cell). The lymphoid leukaemic cells demonstrate unique cytokine receptor profiles, which reveal the great diversity of immunophenotypes within the main functional characterisation of T and B lymphoproliferative malignancies.

In 68 patients with different leukemias the expression of the following adhesion molecules was examined: CD11a, CD18, CD54, CD44, CD58, and CD59. While in normal individuals all these molecules are broadly expressed on leukocytes, in patients with leukemias the following deviations were observed: (a) at least one of the examined molecules was missing in 64/68 cases (94%); in 12/68 cases (18%) both molecules LFA-1 and ICAM-1 were missing, in 37/68 (54%) either LFA-1 or ICAM-1, and in 15/68 cases (22%) adhesion molecules other than LFA-1 or ICAM-1 were missing; (b) the expression of CD11a/CE18, CD58, CD59 on leukemic cells was heterogeneous, without any clear correlation to the subclass of leukemia; (c) in the majority of cases, CD54 (45/68; 66%) and CD44 (36/68; 53%) were missing, however showing a tendency of expression on leukemic cells with more mature immunophenotype.

In 67 cases of newly diagnosed blood malignancies, NonT-ALL, T-ALL, AMLL, AML, CML, CLL, HCL, PLL, MDS, B splenic lymphoma, AUL, as well as in 9 cell lines (U937, HEL, Jurkat, HL60, UHKT2, KG1, Raji, K562, REH), we have analysed the expression and distribution of 2 relatively incompletely studied antigenic markers from the CD nomenclature: CDw12 and CD17, individually and in combination with well characterized ones. We present our data for the usefulness of these molecules in immunodiagnosis of leukemias and lymphomas.

In 72 patients with blood malignancies (leukemias), the expression and distribution of the "B-lineage" antigen CD38, was analyzed, individually and in combination with CD19, CD10, HLA-DR, CD13, CD14, CD33, CDw65, CD2 and CD7. The expression of CD38 on the surface of leukemic cells was totally different from its expression on normal hematopoietic cells. Its positivity in myeloid malignancies was as follows: In patients with acute myeloid leukemia in 21/28 cases-75% (probability of expression 0.68 +/- 0.2, p < 0.05) and in patients with chronic myeloid leukemia in 4/6 cases-66%. In lymphoproliferative malignancies the CD38 antigen was expressed as follows: In patients with acute non-T lymphoblastic leukemia in 12/16 cases-75% (probability of expression 0.7 +/- 0.2, p < 0.05) and in patients with chronic B lymphocytic leukemia in 6/8 cases-75%. CD38 was also found positive in patients with acute mixed lineage leukemia.

Juvenile myelomonocytic leukemia (JMML) is a clonal myeloproliferative disorder of early childhood. In all, 21 patients with JMML who received donor leukocyte infusion (DLI) after allogeneic hematopoietic stem cell transplantation (HSCT) for either mixed chimerism (MC, n=7) or relapse (n=14) were studied. Six patients had been transplanted from an HLA-matched sibling and 15 from other donors. Six of the 21 patients (MC: 3/7 patients; relapse: 3/14 patients) responded to DLI. Response rate was significantly higher in patients receiving a higher total T-cell dose (?1×107/kg) and in patients with an abnormal karyotype. None of the six patients receiving DLI from a matched sibling responded. Response was observed in five of six patients who did and in one of 15 children who did not develop acute graft-versus-host disease following DLI (P=0.01). The overall outcome was poor even for the responders. Only one of the responders is alive in remission, two relapsed, and three died of complications. In conclusion, this study shows that some cases of JMML may be sensitive to DLI, this providing evidence for a graft-versus-leukemia effect in JMML. Infusion of a high number of T cells, strategies to reduce toxicity, and cytoreduction prior to DLI may improve the results.Leukemia (2005) 19, 971-977. doi:10.1038/sj.leu.2403721 Published online 31 March 2005 [ABSTRACT FROM AUTHOR]

The International Prognostic Scoring System (IPSS) for myelodysplastic syndrome (MDS) is based upon weighted data on bone marrow (BM) blast percentage, cytopenia, and cytogenetics, separating patients into four prognostic groups. We analyzed the value of the IPSS in 142 children with de novo MDS and 166 children with juvenile myelomonocytic leukemia (JMML) enrolled in retro- and prospective studies of the European Working Group on childhood MDS (EWOG-MDS). Survivals in MDS and JMML were analyzed separately. Among the criteria considered by the IPSS score, only BM blasts<5%and platelets>100×109/l were significantly associated with a superior survival in MDS. In JMML, better survival was associated with platelets>40×109/l, but not with any other IPSS factors including cytogenetics. In conclusion, the IPSS is of limited value in both pediatric MDS and JMML. The results reflect the differences between myelodysplastic and myeloproliferative diseases in children and adults.Leukemia (2004) 18, 2008-2014. doi:10.1038/sj.leu.2403489 Published online 21 October 2004 [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to an article about JAK2 V617F mutation in juvenile myelomonocytic leukemia.

Presents a letter to the editor of the journal "Leukemia," describing a study which evaluated the prognostic impact of myeloid antigens in uniformly treated patients.