Korean J Lab Med.  2006 Aug;26(4):255-262. 10.3343/kjlm.2006.26.4.255.

Erythroleukemic Blast Crisis of Chronic Myeloid Leukemia

• Chung HJ
• Chi HS
• Seo EJ
• Jang S
• Park CJ
• Lee KH

• KMID: 2238832
• DOI: http://doi.org/10.3343/kjlm.2006.26.4.255

Abstract

Erythroleukemic blast crisis of chronic myeloid leukemia (CML) is very rare. We report two cases of erythroleukemic blast crisis of CML resistant to imatinib treatment. Both patients made a rapid progression to blast crisis 6 and 4 months after diagnosis while being treated with imatinib 400 mg/day. Bone marrow aspiration revealed predominant erythroid precursors with 65.4% and 54.8% each. There were significant proportions (more than 20%) of myeloblasts among non-erythroid cells. Immunophenotyping revealed expression of glycophorin A confirming erythroleukemic blast crisis. The karyotyping result of patient 1 was 46,XX,t(9;22)(q34;q11.2)[3]/52,idem,+8,+12,+18,+21,+22,+der(22)t(9;22)[17] and that of patient 2 was 46,XX,inv(3)(q21q26.2),t(9;22)(q34;q11.2)[20]. Patient 1 showed no response to imatinib and BMS-354825 in the following bone marrow study. She died of septic shock as a complication of an infection after 69 days of blast crisis. Patient 2 received allogeneic bone marrow transplantation (BMT) in the cytogenetically no response state, but she also died of graft-versus-host disease 9 weeks after BMT. The poor prognosis and rapid progression of disease in both cases were correspondent to most of the reported cases. During the course of the disease of the two patients, we monitored the BCR-ABL chimeric mRNA with real-time quantitative polymerase chain reaction (RT-PCR), and it was found useful in predicting the imatinib response and progression to blast crisis of CML. Although both of our cases showed the typical bad prognosis and findings of erythroleukemic blast crisis of CML, the karyotypes were different from the expected type of t(3;21)(q26;q22). But the relationship between additional changes of EVI1 on chromosome 3q26 shown in case 2, and progression to the erythroleukemic blast crisis need further investigation.

Keyword

Chronic myeloid leukemia; Erythroleukemic blast crisis; Imatinib resistance

MeSH Terms

Blast Crisis*
Bone Marrow
Bone Marrow Transplantation
Diagnosis
Glycophorin
Graft vs Host Disease
Granulocyte Precursor Cells
Humans
Immunophenotyping
Karyotype
Karyotyping
Leukemia, Myelogenous, Chronic, BCR-ABL Positive*
Polymerase Chain Reaction
Prognosis
RNA, Messenger
Shock, Septic
Dasatinib
Imatinib Mesylate
Glycophorin
RNA, Messenger

Figure

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  Fig. 1. Hematologic findings at erythroleukemic blast crisis in patient 1 [(A) PB ×1,000 Wright stain, (B) BM biopsy ×200 Hematoxylineosin stain, (C) BM smear ×1,000 Wright stain, (D) BM smear, ×1,000 Periodic acid-Schiff stain]. Leukemic blasts show large size, high N/C ratio, round nucleus with fine nuclear chromatin, 2–4 large prominent nucleoli, basophilic cytoplasm with perinuclear halo and occasional small cytoplasmic vacuoles.

• 

  Fig. 2. Hematologic findings at erythroleukemic blast crisis in patient 2 [(A) PB ×1,000 Wright stain, (B) BM biopsy ×200 Hematoxylineosin stain, (C) BM smear ×1,000 Wright stain, (D) BM smear, ×1,000 Periodic acid-Schiff stain]. Leukemic blasts show large size, high N/C ratio, round nucleus with fine nuclear chromatin, 1–2 large prominent nucleoli and basophilic cytoplasm with perinuclear halo.

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  Fig. 3. Immunophenotyping of blasts at erythroleukemic blast crisis. (A) Blasts of patient 1 express glycophorin A and coexpression of CD13 and CD7. (B) Blasts of patient 2 coexpress glycophorin A and CD33.

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  Fig. 4. Karyotyping at Erythroleukemic Blast crisis. (A) 46,XX,t(9;22)(q34;q11.2)[3]/52,idem,+8,+12,+18,+21,+22,+der(22)t(9;22)[17] in patient 1. (B) 46,XX,inv(3)(q21q26.2),t(9;22)(q34;q11.2)[20] in patient 2.

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  Fig. 5. Quantitation of BCR-ABL chimeric mRNA compared with percentage of total blasts of bone marrow nucleated cells in patient 1.

Reference

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