Circulating endothelial cells are increased in chronic myeloid leukemia blast crisis

We measured circulating endothelial precursor cells (EPCs), activated circulating endothelial cells (aCECs), and mature circulating endothelial cells (mCECs) using four-color multiparametric flow cytometry in the peripheral blood of 84 chronic myeloid leukemia (CML) patients and 65 healthy controls; and vascular endothelial growth factor (VEGF) by quantitative real-time PCR in 50 CML patients and 32 healthy controls. Because of an increase in mCECs, the median percentage of CECs in CML blast crisis (0.0146%) was significantly higher than in healthy subjects (0.0059%, P<0.01) and in the accelerated phase (0.0059%, P=0.01). There were no significant differences in the percentages of CECs in chronic- or active-phase patients and healthy subjects (P>0.05). In addition, VEGF gene expression was significantly higher in all phases of CML: 0.245 in blast crisis, 0.320 in the active phase, and 0.330 in chronic phase patients than it was in healthy subjects (0.145). In conclusion, CML in blast crisis had increased levels of CECs and VEGF gene expression, which may serve as markers of disease progression and may become targets for the management of CML.

Imatinib mesylate as first‑line therapy in patients with chronic myeloid leukemia in accelerated phase and blast phase: A retrospective analysis.

INTRODUCTION: Imatinib is a bcr‑abl tyrosine kinase inhibitor which has revolutionized the treatment for chronic myeloid leukemia (CML). Even though there is much data on CML chronic phase, there is limited data on imatinib‑naïve advanced phase CML. MATERIALS AND METHODS: We retrospectively analysed 90 patients with advanced phase CML (accelerated phase [AP]: 51 and blast crisis [BC]: 39), patients who received imatinib as frontline therapy. RESULTS: The median age of presentation in CML‑AP and CML‑BC were 32 years (12‑61) and 39 years (8‑59), respectively. Imatinib at 600 mg/day was initiated within 2 weeks of diagnosis. Median time to complete hematological response in both CML‑AP and CML‑BC was 3 months (CML‑AP: 1‑9 months and CML‑BC: 1‑14 months). At 6 months 30 (59%) CML‑AP and 15 (38%) CML‑BC patients achieved major cytogenetic response (MCyR), of them 24 (47%) and 10 (25.6%) being the complete cytogenetic response, respectively. At a median follow‑up of 41 months, the median overall survival in CML‑AP was 61 months, but in CML‑BC it was 14 months. The median progression‑free survival and event‑free survival were 30 months and 23 months in CML‑AP and 14 and 12 months in CML‑BC, respectively. On univariate analysis, performance status (PS), spleen size, and MCyR predicted survival in AP, whereas in BC, platelet count, PS, and early MCyR were predictive. Non‑hematologic and hematologic adverse events were observed in 80% and 60% of patients, respectively. Dose was reduced in 10% of patients for grade IV toxicity and interrupted in 30% for grade III toxicity. CONCLUSION: Front‑line imatinib is an option in advanced phases of CML especially in CML‑AP in low‑resource countries, where stem cell transplantation and alternate TKIs are not available.

Bilateral granulocytic sarcoma (chloroma) of the breast in CML in blast crisis: a case report.

A 35 year old lady was diagnosed as having chronic myeloid leukemia in May 1999 and thereafter started on chemotherapy. Three years later the patient presented with bilateral breast masses. FNAC from both the breast lesions showed leukemic infiltration (granulocytic sarcoma). The peripheral blood picture showed blastic transformation. Breast is an uncommon site for development of granulocytic sarcoma. We present this case because of its unusual location and bilateral nature.

Histological and clinical evolution patterns of chronic myelocytic leukemia.

Trephine biopsies of 101 chronic myelocytic leukaemia (CML) patients were analysed to study the relationship between initial and subsequent histological features vis-a-vis clinical behaviour of the disease. The patients with blast crisis at presentation were excluded. At diagnosis 62 (61.4%) patients revealed granulocytic-megakaryocytic (gran-meg) proliferation whereas granulocytic (gran) proliferation was found in 39 (38.6%) patients. Gran pattern at diagnosis was associated with shorter survival and early evolution into blast crisis (36.8%) in 12 months, although the difference in the total incidence of blast crisis between the two histological groups was not statistically significant. Myelofibrosis was detected in more number of cases on follow up (89.1%) as compared to the initial biopsies (80.2%). However myelofibrosis did not correlate with initial cellular composition, overall survival or the phase of CML (P > 0.05). Transition from one histological type to another was observed in 15 out of 60 (25%) cases while remaining in the chronic phase.

Role of immunophenotyping in characterisation of blast crisis of chronic myeloid leukemia--a study of 25 cases.

The blast cell populations of 25 patients of chronic myeloid leukemia in blast crisis (CML-BC) were studied for morphological, cytochemical and immunophenotypic features. The patients were divided into 6 broad groups based upon the pattern of surface marker positivity-myeloblastic, mixed myeloblastic, megakaryoblastic, mixed lineage, lymphoid and undifferentiated blast crisis. Myeloperoxidase (MPO), Sudan Black B (SBB) and Chloroacetate esterase (CAE) stains showed 100% specificity for the myelomonocytic lineage but the sensitivity was low. Periodic acid Schiff (PAS) stain was neither specific nor sensitive for the lymphoid lineage. Immunophenotyping as compared to morphologic and cytochemical assessment, was seen to be most useful for assigning a lineage to leukemic cells in CML-BC.

Destructive bone lesions in chronic granulocytic leukemia.

Bone involvement in Chronic Granulocytic Leukemia (CGL) is rare. We describe here five such patients who presented with severe localized bone pain with involvement of femur neck, skull, tibia-upper end and spine. Lesions were osteolytic in three of them. Blast crisis was present in four of them. Survival was poor in all except one who presented in chronic phase. Bone involvement in CGL carries a poor prognosis.

Erythroleukaemic blast crisis in chronic granulocytic leukaemia.

A rare instance of erythroleukaemic blast crisis in chronic granulocytic leukaemia is described in a 22 years old male. A combination of morphology and a battery of cytochemical stains helped us to make this diagnosis.

Leucemia basofílica / Basophilic leukemia

Se presenta el caso de un paciente masculino de 18 años con diagnóstico de leucemia granulocítica crónica con tres años de evolución en su fase crónica y desarrollo de crisis blástica con presencia de blastos con caracterización basofílica (basofiloblastos), positivos a las tinciones citoquímicas de azul de tolouidina y peroxidasa. A su ingreso se le demostró un cariotipo 46XY Ph-1+ y en la crisis blástica presentó trisomía de los romosomas 8 y 9, presencia de isocromosoma del brazo largo del 17, pérdida del cromosoma Y en algunas células, y persistencia del cromosoma Ph1+. Con todos estos datos clínicos y de laboratorio se concluyó el diagnóstico de leucemia basofílica secundaria a LGC con diferenciación basófilo-mieloblástica no descrita con anterioridad. Se hace una breve revisión de la literatura sobre esta rara variedad de leucemia