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Successful donor-lymphocyte infusion for extreme immune-hemolysis following unrelated BMT in a patient with X-linked chronic granulomatous disease and McLeod phenotype

Bone Marrow Transplantation volume 42, pages 219–220 (2008)Cite this article

Large gene deletions in male subjects at Xp21.1 may cause contiguous gene syndromes by loss of hemizygosity for genes located in this region. Among them are CYBB and XK causing X-CGD and McLeod syndrome. Absence of the XK-encoded membrane glycoprotein Kx abolishes or weakens expression of the highly polymorphic and antigenic Kell antigens, resulting in the rare McLeod phenotype blood group. Carriers are at risk for severe alloimmunization when exposed to non-McLeod erythrocytes. Here, we report a boy diagnosed with X-CGD at 3 years of age. Genomic mapping detected a 148 kb deletion at Xp21.1 spanning exons 2–3 of XK and all exons of CYBB and DYNLT3. Despite antibiotic and antimycotic prophylaxis, he suffered liver abscesses from Burkholderia xenovorans and pulmonary aspergillosis that required surgery. His direct antiglobulin test was negative pre transplant and there was only minimal nonimmune hemolysis due to acanthocytosis.

HLA-matched unrelated replete BM with 4.7 × 106 CD34+ cells/kg from a Kell-negative donor was performed at the age of 10 years in 1999. Recipient and donor blood groups were A rhesus positive (CCD.ee, McLeod) and rhesus positive (CCD.ee, kk). Conditioning consisted of BU (16 mg/kg), CY (adjusted dose 50 mg/kg because of a transiently decreased creatinine clearance of 30 ml/min/1.73 m2 during conditioning, reflecting amphotericin-nephrotoxicity) and rabbit antithymocyte globulin (ATG-Fresenius, 120 mg/kg). For GVHD prophylaxis, he received MTX and CsA. Before engraftment, six units of Kell-negative packed RBC (PRBC) were given. He was discharged on day 30 on CsA without signs of GVHD.

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Authors and Affiliations

  1. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

    U Kordes, M A Hassan, K Beutel, H Kabisch & R Schneppenheim

  2. Department of Transfusion Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

    T M C Binder & T H Eiermann

  3. Department of Pediatric Hematology and Oncology, University Medical Center, Heidelberg, Germany

    B Hassenpflug-Diedrich

  4. Institute of Legal Medicine, Charite-Humboldt-University, Berlin, Germany

    M Nagy

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  1. U Kordes
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  2. T M C Binder
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  5. M A Hassan
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Correspondence to U Kordes.

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Kordes, U., Binder, T., Eiermann, T. et al. Successful donor-lymphocyte infusion for extreme immune-hemolysis following unrelated BMT in a patient with X-linked chronic granulomatous disease and McLeod phenotype. Bone Marrow Transplant 42, 219–220 (2008). https://doi.org/10.1038/bmt.2008.159

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