A restrospective study carried out on 309 patients with chronic myelogenous leukemia treated from 1990 to 10/2000 showed that; -88 cases (28.3%) converted to the blast crisis. - 89.77% of these cases developed acute myeloblastic leukemia (AML) and 9.09% developed ALL.-The percentage of male and female were 55.68% and 44.31% - 45.45% of these converted cases occurred in the age group of 30-50. The youngest age was 10 months and the oldest age was : 72 years old. Mean of duration of the chronic phase was 2.5 years.
Leukemia, Myeloid, Chronic ; Leukemia, Myeloid, Chronic-Phase

Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Chronic-Phase ; drug therapy ; Protein-Tyrosine Kinases ; antagonists & inhibitors

A localized extramedullary tumor mass composed of immature cells has been reported in association with acute myeloid leukemia, myeloproliferative disorders, myelodysplasia in blast transformation or chronic myeloid leukemia with trilineage hematopoiesis, as well as in patients with no known hematological disorder. Although this tumor may involve anywhere in the body and give rise to a variety of signs and symptoms, there are several case reports of extramedullary tumor in Korea which described the involvement of the gastrointestinal tract. We report the occurrence of gastric extramedullary tumor and lymphoid blast crisis in a patient with complete remission after lymphoblastic transformation of chronic myelogenous leukemia. Endoscopic biopsy for gastric elevated lesion showed diffuse lymphoblast infiltration with TdT (terminal deoxynucleotidal transferase) and CD20 positive immature cells.
Biopsy ; Blast Crisis ; Gastrointestinal Tract ; Hematopoiesis ; Humans ; Korea ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Leukemia, Myeloid, Acute ; Lymphocyte Activation ; Myeloproliferative Disorders ; Sarcoma, Myeloid

The aim of this study was to investigate the effect of nonmyeloablative peripheral blood stem cell transplantation in treatment of chronic myeloid leukemia in chronic phase (CML-CP) and accelerated phase (CML-AP). 24 patients with CML including 16 in CML-CP and 8 in CML-AP were treated with nonmyeloablative conditioning regimen for peripheral blood stem cell transplantation (PBHSCT). The conditioning regimen included fludarabine (30 mg/m(2)x6 d), busulphan [4 mg/(kg.d)x2 d] and CTX [350 mg/(m2.d)x2 d] combined with or without Ara-C. The donors were HLA-identical (n=20) and 5/6 antigen-matched (n=4). The dynamic observation of hematopoietic recovery in all patients was carried out. The results indicated that all the patients were successfully engrafted. The mean time for increase of the number of neutrophils to more than 0.5x10(9)/L and platelet more than 20x10(9)/L were 13 days and 11.5 days respectively. Out of 12 patients, 9 patients showed complete donor chimerism and 3 patients showed mixed chimerism at 30 days after transplantation. At 180 days after transplantation, 18 survival patients showed complete donor chimerism. 18 patients remained alive after a median follow-up length of 24 months (4-48 months). 2 cases died of severe acute GVHD and 1 case died of chronic GVHD, 2 cases died of interstitial pneumonia and 1 case died of relapsed. In conclusions, nonmyeloablative peripheral blood stem cell transplantation is an effective therapeutic method for CML patients in chronic phase and accelerated phase.
Adult ; Female ; Graft vs Host Disease ; prevention & control ; Humans ; Leukemia, Myeloid, Accelerated Phase ; therapy ; Leukemia, Myeloid, Chronic-Phase ; therapy ; Male ; Middle Aged ; Peripheral Blood Stem Cell Transplantation ; methods ; Transplantation Conditioning

Granulocytic sarcoma is an uncommon tumor composed of immature cells of the granulocytic series, occurring in patients with acute myelogenous leukemia and blast crisis of chronic myelogenous leukemia. It may be rarely preceded by an acute myelogenous leukemia and follows an unformly fatal course. We report a case of granulocytic sarcoma of extradural space presenting as the spinal cord compression without any evidence of leukemia in the peripheral blood. The tumor was located extraduraly in the C6-C7 area compressing the dural sac and spinal cord. After left hemilaminectomy of C6 and C7, this mass was removed completely. Postoperatively, symptoms and signs were improved. At the postoperative 10th day, the patient lost consciousness and peripheral blood study showed the findings of acute myelogenous leukemia. Thereafter, the patient received chemotherapy with Ara-C and Daunorubicin, but hematologic remission was not obtained completely. After 4 months, he revisited our hospital owing to fever, and the septic shock was encountered. The patient died of sepsis.
Blast Crisis ; Consciousness ; Cytarabine ; Daunorubicin ; Drug Therapy ; Fever ; Humans ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Sarcoma, Myeloid* ; Sepsis ; Shock, Septic ; Spinal Cord ; Spinal Cord Compression ; Spine*

Granulocytic sarcoma is a localized tumor mass composed of immature cells of the granulocytic series. It can develope in patients with acute myelogenous leukemia or impending blast crisis of chronic myelogenous leukemia. Granulocytic sarcoma during the course of chronic myelogenous leukemia has beeen noted to occur in lymph node, bone, skin, breast, peritoneum, testis, ovary, gastrointestinal tract, kidney, brain. We reported a case of granulocytic sarcoma causing spinal cord compression in chronic myelogenous leukemia with a brief review of literature.
Blast Crisis ; Brain ; Breast ; Female ; Gastrointestinal Tract ; Humans ; Kidney ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Leukemia, Myeloid, Acute ; Lymph Nodes ; Ovary ; Peritoneum ; Sarcoma, Myeloid* ; Skin ; Spinal Cord Compression* ; Spinal Cord* ; Testis

BACKGROUND: The coexistence of t(9;22)(q34;q11.2) and inv(16)(p13q22) chromosomal abnormalities is extremely uncommon, and only a small number of such cases have been reported. Here, we characterized 7 cases of hematologic malignancy exhibiting t(9;22) and inv(16) coexistence. METHODS: We reviewed the cytogenetic data for hematologic malignancies treated at the Catholic Blood and Marrow Transplantation Center between January 2004 and June 2013. We identified 7 cases exhibiting t(9;22) and inv(16) coexistence. In addition, we analyzed mutations in the IKZF1, NPM1, FLT3, N-RAS, K-RAS, c-KIT, and TP53 genes. RESULTS: Four cases of chronic myelogenous leukemia (CML; 1 chronic phase, 2 accelerated phase, and 1 blast phase) and 3 cases of acute myeloid leukemia (AML; 1 de novo and 2 therapy-related) were identified. The percentages of circulating blasts and bone marrow eosinophils were higher in AML cases than in CML cases (53% vs. 5% and 30% vs. 5.5%, respectively). The proportions of each chromosomal abnormality were used along with follow-up karyotyping results to identify secondary changes. In BCR/ABL, a p210 fusion transcript was associated with CML, whereas a p190 fusion transcript was associated with AML. One patient with AML harbored 2 mutations: c-KIT D816V and TP53 E11Q. All patients except 1 with CML blast phase sustained clinical remission after treatment, which included an imatinib mesylate regimen. CONCLUSION: This study shows that observations of bone marrow morphology, initial and follow-up cytogenetic studies, and karyotyping of BCR/ABL1 and CBFB/MYH11 provide valuable information for characterizing hematologic malignancies exhibiting t(9;22) and inv(16) coexistence.
Blast Crisis ; Bone Marrow ; Chromosome Aberrations* ; Cytogenetics ; Eosinophils ; Follow-Up Studies ; Genes, p53 ; Hematologic Neoplasms* ; Humans ; Karyotyping ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Mesylates ; Imatinib Mesylate

The aim of this study was to investigate the expression status of the helicase antigen (HAGE) transcript and its clinical significance in patients with acute myeloid leukemia (AML) and chronic myeloid leukemia (CML). The expression of HAGE cDNA in bone marrow mononuclear cells from AML and CML patients was detected by using real-time quantitative PCR. The results indicated that overexpression of HAGE transcript (117.12% - 9842.70%, median 434.96%) was detected in 14.8% (11/74) AML patients. AML patients with HAGE cDNA expression were significantly older than those HAGE-negative patients (median 67 and 45 years, respectively, p = 0.001). HAGE cDNA expression was more frequently present among the patients with acute monoblastic leukemia (M(4) and M(5), 7 of 20, 35.0%), compared to the patients with acute non-monoblastic leukemia (M(1), M(2), M(3) and M(6), 4 of 54, 7.4%) (p = 0.007). 28.6% (8/28) cases with normal karyotypes showed HAGE cDNA overexpression, significantly higher than 7.5% (3 of 40) in those with chromosomal abnormalities (p = 0.041). Overexpression of HAGE transcript was found in 9 (34.6%) CML cases and more frequently observed at accelerated phase and blast crisis (4/4, 100%) than that at chronic phase (5/22, 22.7%) (p = 0.008). It is concluded that HAGE cDNA expression is relevant to specific subtypes of AML and to the progression of CML.
Blast Crisis ; Bone Marrow Cells ; metabolism ; DEAD-box RNA Helicases ; genetics ; metabolism ; DNA, Complementary ; Gene Expression ; Humans ; Karyotype ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; genetics ; metabolism ; Leukemia, Myeloid, Acute ; genetics ; metabolism ; Neoplasm Proteins ; genetics ; metabolism ; RNA, Messenger ; genetics

OBJECTIVETo evaluate the therapeutic effect of imatinib on chronic myeloid leukemia (CML) in different phases and analyze the factors that may affect the effects.

METHODSEighty-five patients with CML in chronic phase, 24 in accelerated phase and 19 in blastic phase patients were treated with imitinib. The hematologic response, cytogenetic response, molecular response, overall survival (OS), progression-free survival (PFS) and adverse events were analyzed in these groups.

RESULTSThe rates of complete hematologic response (CHR), complete cytogenetic response (CCyR) and complete molecular response (CMoR) of the patients in chronic phase were 100%, 82.4% and 21.2%, respectively, and the 5-year OS and PFS of these patients were 92.1% and 84.7%. All these rates were significantly higher than those in patients in accelerated and blastic phases (P<0.0001). The CCyR, CMoR, 5-year OS and PFS in the 42 newly diagnosed patients in chronic phase were 92.9%, 26.3%, 100% and 95.2%, respectively, all significantly higher than those in patients with interferon therapy failure (P<0.001). Severe leukocytopenia and thrombocytopenia occurred at greater frequencey in AP and BP patients than in chronic phase patients (P<0.0001). Non-hematologic toxicity was rarer and milder in patients in chronic phase. Multivariate analysis showed that interferon therapy prior to imitinib treatment and prolonged drug cessation were the independent factors that affected the achievement of cytogenetic response and PFS.

CONCLUSIONEarly imitinib therapy can be effective and safe, and should be used as the first line drug for CML.

Antineoplastic Agents ; therapeutic use ; Benzamides ; Female ; Humans ; Imatinib Mesylate ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; drug therapy ; Leukemia, Myeloid, Chronic-Phase ; drug therapy ; Male ; Piperazines ; therapeutic use ; Pyrimidines ; therapeutic use ; Treatment Outcome

Objective: To explore the influencing covariates of severe neutrophils and/or thrombocytopenia and their effect on treatment response and outcome in patients with chronic-phase chronic myeloid leukemia (CP-CML) receiving initial second-generation tyrosine kinase inhibitors (2G-TKI) . Methods: Data from consecutive patients aged ≥18 years with newly diagnosed CP-CML who received initial 2G-TKI at Peking University People's Hospital from September 2008 to November 2021 were interrogated. Binary logistic regression models and Fine-Gray and Cox regression models were applied. Results: Data from 267 patients who received initial 2G-TKI, including nilotinib (n=239, 89.5% ) and dasatinib (n=28, 10.5% ) , were interrogated. The median age was 36 (range, 18-73) years, and 156 (58.4% ) patients were male. At a median treatment period of 1.0 (0.1-3.0) month, 43 (16.1% ) patients developed grade ≥3 neutrophils and/or thrombocytopenia and recovered within 1.0 (0.1-24.6) month. Male (OR=2.9, 95% CI 1.2-6.8; P=0.018) , age of ≥36 years (OR=3.2, 95% CI 1.4-7.2, P=0.005) , a spleen below a costal margin of ≥7 cm (OR=2.8, 95% CI 1.2-6.6, P=0.020) , and a hemoglobin (HGB) level of <100 g/L (OR=2.9, 95% CI 1.3-6.8, P=0.012) at diagnosis were significantly associated with grade ≥ 3 neutrophils and/or thrombocytopenia. Based on their regression coefficients, male, age of ≥36 years, a spleen below a costal margin of ≥7 cm, and an HGB level of <100 g/L were given 1 point to form a predictive system. All patients were divided into three risk subgroups, and the incidence of severe cytopenia significantly differed among the three groups (P < 0.001) . Grade ≥3 neutrophils and/or thrombocytopenia for >2 weeks was significantly associated with lower cumulative incidences of complete cytogenetic response (CCyR, HR=0.5, 95% CI 0.3-0.7, P<0.001) and major molecular response (MMR, HR=0.4, 95% CI 0.3-0.8, P=0.004) and was not significantly associated with failure, progression, and survival. Conclusion: Male, advanced age, a large spleen, and a low HGB level were significantly associated with severe cytopenia. The four covariates were used to establish a prediction model, in which the incidence of severe cytopenia among different risk groups was significantly different. Severe cytopenia for >2 weeks was a negative factor for responses but not for outcomes.
Humans ; Male ; Adolescent ; Adult ; Female ; Protein Kinase Inhibitors/therapeutic use* ; Tyrosine Protein Kinase Inhibitors ; Treatment Outcome ; Retrospective Studies ; Dasatinib/therapeutic use* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy* ; Leukemia, Myeloid, Chronic-Phase/drug therapy* ; Thrombocytopenia