Sudden blast crisis in patients with Philadelphia chromosome-positive chronic myeloid leukemia who achieved complete cytogenetic remission after imatinib therapy

Cancer ◽  
2006 ◽  
Vol 107 (5) ◽  
pp. 1008-1013 ◽  
Author(s):  
Giuliana Alimena ◽  
Massimo Breccia ◽  
Roberto Latagliata ◽  
Ida Carmosino ◽  
Eleonora Russo ◽  
...  
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Blast Crisis ◽  
Philadelphia Chromosome ◽  
Imatinib Therapy ◽  
Cytogenetic Remission ◽  
Philadelphia Chromosome Positive

scholarly journals Bilateral Serous Retinal Detachment in Lymphoid Blast Crisis of Chronic Myeloid Leukemia

2021 ◽  
pp. 555-561
Author(s):  
Kyohei Umemura ◽  
Kyoko Fujita ◽  
Akiko Jinno ◽  
Ayano Nakamura ◽  
Motohiro Kamei
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Retinal Detachment ◽  
Cell Count ◽  
Myeloid Leukemia ◽  
Blast Crisis ◽  
Serous Retinal Detachment ◽  
Philadelphia Chromosome ◽  
Ocular Involvement ◽  
Blast Cells ◽  
Philadelphia Chromosome Positive

We herein report a patient with Philadelphia chromosome-positive lymphoid blast crisis of chronic myeloid leukemia (CML), who presented with bilateral serous retinal detachment (SRD). A 36-year-old Asian male presented with the symptoms of decreased vision and was found to have bilateral SRD involving fovea. There was no inflammation in the anterior chamber or vitreous. Physical examination showed hepatomegaly and splenomegaly. A blood count revealed white blood cell count of 38.2 × 10<sup>9</sup>/L with 51.5% blast cells. Bone marrow aspirate showed total cell count of 145 × 10<sup>3</sup>/μL with 80.6% blast cells and negative neutrophil myeloperoxidase staining. Cytogenetic analysis using fluorescence in situ hybridization confirmed a 9;22 chromosomal translocation, indicating the presence of the Philadelphia chromosome. Flow cytometry analysis demonstrated expression of CD10, CD19, and positive TdT. According to morphology, immunology, cytogenetics, and molecular criteria, the patient was diagnosed as having Philadelphia chromosome-positive lymphoid blast crisis of CML. Based on the ocular findings and hematological abnormalities, the SRD was considered to be ocular involvement secondary to the blast crisis of leukemia. Two months after starting induction therapy, fundus examination and optical coherence tomography showed complete resolution of bilateral SRD and improved vision. Prompt diagnosis of the disease leads to early systemic chemotherapy and may help restore visual function and improve survival.

Philadelphia Chromosome Positive Acute Myeloid Leukemia: An Aggressive Acute Leukemia with Clinicopathologic Features Distinct from Chronic Myeloid Leukemia in Blast Crisis.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3290-3290
Author(s):  
Chad P. Soupir ◽  
Jo-Anne Vergilio ◽  
Paola Dal Cin ◽  
Alona Muzikansky ◽  
Hagop M. Kantarjian ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Myeloid Leukemia ◽  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Blast Crisis ◽  
Philadelphia Chromosome ◽  
Rare Entity ◽  
Median Response ◽  
Acute Myeloid ◽  
Philadelphia Chromosome Positive

Abstract Philadelphia chromosome positive acute myeloid leukemia (Ph+ AML) is a rare entity reported to comprise 1% of tumors bearing the Philadelphia chromosome. Controversy exists over whether this represents a distinct entity or is merely presentation of patients in the blastic phase of chronic myeloid leukemia (CML). Our study sought to determine the clinicopathologic characteristics of Ph+ AML in comparison to CML in blast crisis. We searched the archival files of Massachusetts General Hospital, Brigham and Women’s Hospital, Dana Farber Cancer Institute, and MD Anderson Cancer Center from 1995 to 2005 for cases of apparent de novo Ph+ AML. After excluding biphenotypic leukemias and cases with any evidence of prior CML or other myeloid neoplasia, we retrieved 18 Ph+ AML cases. 20 cases of documented CML in myeloid blast crisis (CML-MBC) who had not received prior imatinib mesylate therapy or bone marrow transplant were used as a control group. The clinical and laboratory features, bone marrow morphology, immunophenotype, cytogenetics, and type of BCR/ABL transcript were analyzed. The Ph+ AML patients included 13 males and 5 females with a median age of 55 years, which was not significantly different from CML-MBC patients. The median WBC at presentation was 15.7 x 109/L. Compared to CML-MBC patients, the Ph+ AML patients were less likely to have splenomegaly (22% vs. 68%, p=0.008) or peripheral basophilia (median absolute basophil count 0.16 vs. 1.24 x 109/L, p=0.003). The bone marrow in Ph+ AML patients was less cellular (median cellularity 80% vs. 98%, p=0.002) with a lower myeloid:erythroid ratio (1.3 vs. 4.8, p=0.01), but more prominent erythroid and myeloid lineage dysplasia (p=0.04) than that of CML-MBC patients. While cytogenetic abnormalities in addition to t(9;22) occurred in 67% of Ph+ AML cases, the typical additional cytogenetic changes of CML-MBC (+8, +19, iso17q, and +Ph+) were uncommon (22% vs. 87% for CML-MBC, p=0.0004). In 5/6 Ph+ AML cases with multiple clones, the Ph+ was present in all clones, suggesting that this represents an early event in the evolution of these leukemias. RT-PCR revealed a predicted BCR/ABL product of p210 (10/13 cases), p190 (2/13 cases) or p230 (1/13 cases). 6/7 Ph+ AML patients treated with imatinib showed at least a partial hematologic response, but this was of a short duration (median 2.5 months, range 1–6 months) in comparison to the reported median response duration of 10 months in imatinib-responsive CML-MBC. The median survival of the Ph+ AML patients was 10.5 months, similar to that of CML-MBC and AML with adverse cytogenetics. In summary, Ph+ AML is a rare entity distinct from CML-MBC, with an aggressive clinical course and only transient response to imatinib.

Deletion of the 5'ABL Region in Philadelphia Chromosome Positive Chronic Myeloid Leukemia: Frequency, Origin and Prognosis

2003 ◽  
Vol 44 (8) ◽  
pp. 1333-1338 ◽  
Author(s):  
Frédéric Morel ◽  
Chandran Ka ◽  
Marie-Josée Le Bris ◽  
Angèle Herry ◽  
Patrick Morice ◽  
...  
Keyword(s):  
Chronic Myeloid Leukemia ◽  
Myeloid Leukemia ◽  
Philadelphia Chromosome ◽  
Philadelphia Chromosome Positive
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