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Recurrence of lymphoid blast crisis over 20 years after successful sibling allo-SCT for CML: short lived complete cytogenetic response to imatinib

Bone Marrow Transplantation volume 44, pages 267–268 (2009)Cite this article

Unlike other therapies for CML, allogeneic hematopoietic SCT (HSCT) is a potentially curative treatment. Survival at 20 years post sibling allogeneic HSCT in low-risk groups is 49%.1 Relapse post transplant is a significant cause of mortality, the majority occurring within 2 years of transplant and the incidence becoming less frequent over time.1 Presentation in blast crisis (BC) is uncommon but carries a grave prognosis. BC is less responsive to conventional chemotherapy, and survival post HSCT for BC is lower due to higher relapse rates and treatment-related mortality. Five year survival is approximately 18%.1 We present a patient with CML in lymphoid BC at diagnosis. She was successfully treated by matched sibling HSCT but relapsed in lymphoid BC after 20 years.

This 41-year-old Caucasian woman, with a short history of fatigue, bone pains and fever, sought medical attention in August 1985. Blood analysis showed pancytopenia with circulating blasts. BM morphology and cytochemistry suggested lymphoid BC of CML. Further investigations confirmed these blasts to be TdT positive but were negative for non-specific esterase, periodic acid Schiff and Sudan Black staining. Cytogenetic analysis showed a Ph translocation, t(9;22),(q34;q11), in all cells analyzed. She obtained a complete morphologic remission from her initial therapy (CY, doxorubicin, VCR, prednisone and L-asparaginase) and then proceeded to a non-T-cell-depleted allogeneic HSCT from her HLA-identical brother in October 1985. The conditioning regimen used was CY (120 mg/kg), VCR, L-asparaginase and TBI delivered in a single fraction of 500 cGy. GVHD prophylaxis was with CYA and oral prednisone. Day-100 post transplant, BM biopsy confirmed complete morphologic remission, but cytogenetic analysis revealed ongoing mixed chimerism (60% male vs 40% female) and persistent Ph+ cells, still accounting for 40% of remaining host hematopoiesis. Immunosuppression was abruptly withdrawn in April 1986, and transition to full donor chimerism (100% male) with extinction of the Ph+ cell line had occurred by May 1986 as indicated by repeat BM biopsy and cytogenetic analysis. From June 1986 until 2006 she remained well with excellent quality of life. All investigations between 1986 and 1990 suggested continued cytogenetic remission with 100% donor hematopoiesis. After 1990, follow-up was predominantly by her local physician and cytogenetic/molecular studies were not performed; blood counts in 2002 were normal.

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  1. Division of Hematology, Vancouver General Hospital, Vancouver, British Columbia, Canada

    D A Irvine & J D Shepherd

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  1. D A Irvine
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Correspondence to J D Shepherd.

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Irvine, D., Shepherd, J. Recurrence of lymphoid blast crisis over 20 years after successful sibling allo-SCT for CML: short lived complete cytogenetic response to imatinib. Bone Marrow Transplant 44, 267–268 (2009). https://doi.org/10.1038/bmt.2009.11

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