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ASH Image Bank (2001); doi:10.1182/ashimagebank-2001-100198
Copyright © 2001 by the American Society of Hematology.
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Full Case Study

Blast Crisis of Chronic Myelogenous Leukemia

Peter Maslak, MD



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  		Figure 1. Blast Crisis of Chronic Myelogenous Leukemia Figure 1. Blasts noted in the peripheral blood. These appear deeply basophilic with a thin rim of cytoplasm (100X MacNeal tetrachrome).

 


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  		Figure 2. Blast Crisis of Chronic Myelogenous Leukemia Figure 2. Blast, characterized by a high nucleocytoplasmic ratio, pictured next to a granulocyte in the peripheral blood.

 


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  		Figure 3. Blast Crisis of Chronic Myelogenous Leukemia Figure 3. Bone marrow aspirate shows increased blasts. (40X MacNeal tetrachrome).

 


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  		Figure 4. Blast Crisis of Chronic Myelogenous Leukemia Figure 4. High-power view of blasts in the aspirate. Multiple nucleoli are faintly discernible (100X MacNeal tetrachrome).

 


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  		Figure 5. Blast Crisis of Chronic Myelogenous Leukemia Figure 5. Bone marrow biopsy is replaced by blasts (40X Giemsa).

 


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  		Figure 6. Blast Crisis of Chronic Myelogenous Leukemia Figure 6. Dual parameter plot of forward versus side scatter separates cells by size and granularity and defines the blast population.

 


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  		Figure 7. Blast Crisis of Chronic Myelogenous Leukemia Figure 7. Blasts co-express CD13 and CD19 (right upper quadrant).

 


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  		Figure 8. Blast Crisis of Chronic Myelogenous Leukemia Figure 8. Patient's peripheral blood returned to normal following Gleevec (40X MacNeal tetrachome).

 


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  		Figure 9. Blast Crisis of Chronic Myelogenous Leukemia Figure 9. Hypocellular bone marrow with maturing myeloid and erythroid elements (40X MacNeal tetrachrome).

 

   Clinical Summary
TOP
 Clinical Summary
Diagnosis
 Discussion
 Differential Diagnosis
 
The patient is a 47-year-old man who presents to the hematology clinic for a second opinion regarding further therapy for a diagnosis of chronic myelogenous leukemia (CML). The patient was in his usual state of health until 18 months ago when he presented with a white blood cell count (WBC) of 40 k/ml on a routine complete blood cell count (CBC). The diagnostic work-up included a bone marrow aspirate and biopsy and demonstrated that the patient had CML. Karyotype revealed 100% Ph+ cells.

Shortly afterwards, the patient was enrolled on an ECOG (Eastern Cooperative Oncology Group) protocol and received alpha interferon in combination with subcutaneous Ara-C for 10 days each month. The patient tolerated the regimen with only a minor increase in his transaminases. A repeat bone marrow with cytogenetic testing done following 6 months of therapy showed a 60% reduction in the Ph+ population. However, subsequent bone marrow evaluations at the 12- and 18- month mark showed an increase in the Ph+ population with 60% and 75% of the cells positive. Despite this, the interferon/Ara-C was continued.
Two weeks prior to evaluation, the patient had a repeat bone marrow and was found to have 13% blasts. He was told he had accelerated phase disease and was referred for evaluation.

The patient reported no new complaints. He denied fever, chills, fatigue, and new onset cough or night sweats. The physical examination revealed a 47-year-old man in no acute distress. There was no adenopathy. No organomegaly was appreciated. CBC was performed and showed: WBC- 3.2 k/ml, hemoglobin (Hgb)- 11.6 g/dl, hematocrit (Hct)- 33.6%, mean corpuscular volume (MCV)- 94 fl, mean corpuscular hemoglobin (MCH)- 32.5 g/dl, and platelest- 142K/ml. Machine-generated differential revealed: neutrophils- 66.6%, lymphocytes- 23.5%, eosinophils-1.5%, basophils-0.9%, and large unclassified cells (LUCs)- 4.1%. Review of the peripheral smear demonstrated that the LUC's were circulating blasts (Figure 1 and Figure 2).
Bone marrow aspiration and biopsy were performed. The aspirate was hypercellular with an increased number of blasts (66%). The blasts varied in size with thin to moderate cytoplasm (Figure 3, Figure 4, and Figure 5). Megakaryocytes were also increased and some appeared dysplastic. Flow cytometry demonstrated the presence of CD34, CD33, CD13, CD19, and CD10 suggesting mixed lineage disease (Figure 6 and Figure 7). Cytogenetics revealed that in addition to the Philadelphia chromosome (t(9;22), the patient had acquired +8 and t(12;14).

Treatment options were discussed. The patient was started on imatinib mesylate (Gleevec) 600 mg po QD. He developed some mild periorbital edema but tolerated the therapy well. He required no transfusions while taking the medication. CBC following 4 weeks of therapy revealed: WBC- 2.8 k/ul, Hgb- 9.3 g/dl, Hct- 26.9%, and platelets- 147k/ul. No circulating blasts were detected (Figure 8). Repeat bone marrow was performed at this time and showed a hypocellular aspirate without increased blasts (Figure 9). Biopsy showed maturation with a normal number of megakaryocytes.

The patient was maintained on Gleevec. HLA typing showed that neither of the patient’s two siblings were identical and an unrelated search was initiated.

Sex
Male

Age
47


   Diagnosis
TOP
 Clinical Summary
 Diagnosis
Discussion
 Differential Diagnosis
 
Chronic myelogenous leukemia- blast phase


   Discussion
TOP
 Clinical Summary
 Diagnosis
 Discussion
Differential Diagnosis
 
Chronic myelogenous leukemia is a myeloproliferative disorder with distinct biology and a well-defined clinical course. The disease is characterized by the presence of the Philadelphia chromosome which results from a balanced translocation between chromosomes 9 and 22. While the majority of patients present in chronic phase, the natural history of the disorder is progression to the fulminant clinical picture of blast crisis. This phase is defined by more than 30% blasts in either the blood or the bone marrow. It may be accompanied by chromosomal changes which occur in addition to the Philadelphia chromosome. While the majority of blastic transformation is toward a myeloid lineage, approximately one-third of patients develop a blast crisis that is of lymphoid lineage. However, the co-expression of myeloid and lymphoid markers is not uncommon.

Treatment for blast crisis is suboptimal. Interferon is inactive. Patients who have a myeloid blast crisis are often treated with acute myelogenous leukemia- (AML-) type chemotherapy. The response rate is only 20% to 30% with few long term survivors. While patients who develop a lymphoid blast crisis have a higher complete remission (CR) rate, their responses are often short-lived and the overall prognosis remains grim. Stem cell transplantation, once the patient has been able to achieve CR, may provide the best hope for cure in selected patients. The recent introduction of imatinib mesylate (Gleevec) has provided some hope in treating this uniformly fatal disorder. Hematologic response rates of approximately 30% have been reported in untreated patients with myeloid blast crisis. Resistance, however, remains a problem and most patients will relapse with blastic disease within a few months.


   Differential Diagnosis
TOP
 Clinical Summary
 Diagnosis
 Discussion
 Differential Diagnosis
 
acute leukemia, other myeloproliferative disorders


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Related ASH-SAP Chapter:space logo
Chapter 8: Myeloproliferative disorders

This Article
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Right arrow Articles by Maslak, P.
Related Collections
Right arrow Myeloproliferative Diseases
Right arrow Related ASH-SAP Chapter
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Right arrowRelated ASH Education Book Articles
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Copyright © 2001 by the American Society of Hematology.