CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Hematologic Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Leukemia, Myeloid ; pathology ; Lymphoma, Extranodal NK-T-Cell ; pathology ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Skin Neoplasms ; drug therapy ; genetics ; metabolism ; pathology ; surgery

Lineage switch in acute leukemia is an uncommon event at relapse, and therefore rarely reported in the literature. Here, we have described the clinical laboratory features of four cases in which the cell lineage switched from acute lymphoblastic leukemia (ALL) to acute myeloid leukemia (AML). One patient was initially diagnosed with B-ALL, switched to T-ALL at the first relapse, and eventually, AML at the second relapse. A lineage switch represented either relapse of the original clone with heterogeneity at the morphologic level or emergence of a new leukemic clone. Further sequential phenotypic and cytogenetic studies may yield valuable insights into the mechanisms of leukemic recurrence, with possible implications for treatment selection.
Acute Disease ; Bone Marrow/pathology ; Cell Lineage ; Child ; Chromosome Aberrations ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunophenotyping ; Infant ; Leukemia, Myeloid, Acute/*diagnosis/drug therapy/pathology ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/*diagnosis/drug therapy/pathology ; Recurrence ; Salvage Therapy ; Transplantation, Homologous

Breast metastases in cases leukemia are very rare and occur primarily in patients with acute myeloid leukemia. We report the involvement of breast metastases in a 30-year-old woman with acute T cell lymphoblastic leukemia. The patient's mammograms revealed an extremely dense pattern with ill-defined, denser mass-like lesions in both breasts. A bilateral breast ultrasonographic evaluation revealed lobular-shaped and partly ill-defined hypoechoic masses with a multi-septated nodular (mottled) appearance.
Adult ; Breast Neoplasms/drug therapy/radiography/*secondary/ultrasonography ; Diagnosis, Differential ; Female ; Humans ; Mammography ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/*pathology ; Ultrasonography, Mammary

OBJECTIVETo assess the prognostic value of both morphological persistent disease on day 19, on complete remission (CR) and minimal residual disease (MRD) in the bone marrow (BM) after multiagent remission induction therapy.

METHODSFrom January 1998 to May 2003, 193 patients with newly diagnosed ALL were enrolled on protocol of ALL-XH-99. BM blast counts on day 19 and on CR after induction therapy were examined. BM MRD at the end of induction therapy was detected by MP-FCM.

RESULTS(1) The probability of 5-year event-free survival (pEFS) was significantly worse for patients with > or = 0.050 BM lymphoblasts on day 19 than that with < 0.050 BM lymphoblasts [(42.59 +/- 14.28)% vs (74.24 +/- 6.67)%, P < 0.001]. (2) The 5-year pEFS was significantly worse for patients with a low percentage of lymphoblasts (< 0.050) in BM on CR as compared to those with no morphological persistent lymphoblasts [(63.47 +/- 9.23)% vs (76.41 +/- 6.09)%, P < 0.05]. (3) No significant difference was found in BM lymphoblasts between patients with MRD (> or = 10(-4) of nucleated bone marrow cells) and those without MRD (< 10(-4)) at the end of induction therapy (P > 0.05). The 22-month pEFS was significantly worse for patients with MRD as compared with those without MRD on CR [(23.81 +/- 20.26)% vs (94.44 +/- 5.40)%, P = 0.001].

CONCLUSIONSBM lymphoblast > or = 0.050 on day 19 after induction therapy is an independent prognostic factor for childhood ALL; low percentage of lymphoblasts and minimal residual disease in BM on remission also do it. Patients with > or = 0.050 lymphoblast in BM on day 19 or with MRD > or = 10(-4) at the end of induction therapy should receive altered and more intensive chemotherapy.

Adolescent ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bone Marrow ; drug effects ; pathology ; Bone Marrow Examination ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Neoplasm, Residual ; diagnosis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; blood ; drug therapy ; pathology ; Prognosis ; Remission Induction ; Survival Analysis

Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; CD4 Antigens ; metabolism ; CD56 Antigen ; metabolism ; Cyclophosphamide ; therapeutic use ; Dendritic Cells ; pathology ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Humans ; Leukemia, Myeloid ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Plasmacytoma ; drug therapy ; metabolism ; pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Prednisone ; therapeutic use ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; Vincristine ; therapeutic use

Adolescent ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; administration & dosage ; Cyclophosphamide ; administration & dosage ; Diagnosis, Differential ; Doxorubicin ; administration & dosage ; Female ; Hodgkin Disease ; pathology ; Humans ; Lung Neoplasms ; pathology ; Lymphoma, B-Cell ; drug therapy ; pathology ; surgery ; Male ; Mediastinal Neoplasms ; drug therapy ; pathology ; surgery ; Mediastinum ; surgery ; Neoplasm Invasiveness ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; pathology ; Prednisone ; administration & dosage ; Thymoma ; pathology ; Thymus Neoplasms ; pathology ; Vincristine ; administration & dosage

AML relapsing as ALL has rarely been reported. We describe the case of a 62-yr-old man who was diagnosed with erythroleukemia with a complex karyotype and achieved complete hematologic and cytogenetic remission after induction chemotherapy. However, 4 months after the initial diagnosis, he showed relapse with blasts showing a different morphology and immunophenotype and was diagnosed with precursor B-cell ALL. The relapsing precursor B-cell ALL presented with the same leukemic clones as the primary erythroleukemia. Cytogenetic analysis of his bone marrow (BM) at the time of the primary erythroleukemia showed complex karyotypic abnormalities, including monosomy 5 and monosomy 7. At relapse, his BM showed reemergence of these leukemic clones of complex karyotypic abnormalities with clonal switch. To our knowledge, this is the first case of a lineage switch from erythroleukemia to ALL.
Acute Disease ; Antimetabolites, Antineoplastic/therapeutic use ; Bone Marrow Cells/pathology ; Cell Lineage ; Cell Transformation, Neoplastic ; Chromosome Deletion ; Chromosomes, Human, Pair 5 ; Chromosomes, Human, Pair 7 ; Cytarabine/therapeutic use ; Drug Therapy, Combination ; Humans ; Immunophenotyping ; Karyotyping ; Leukemia, Erythroblastic, Acute/*diagnosis/drug therapy ; Male ; Middle Aged ; Monosomy ; Naphthacenes/therapeutic use ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/*diagnosis/pathology ; Recurrence

OBJECTIVETo study the clinicopathologic features and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm.

METHODSThe clinical, morphology and immunophenotypic features were analyzed in 3 cases of blastic plasmacytoid dendritic cell neoplasm, with review of literature.

RESULTSThe pathologic changes of these tumors accorded with that of blastic plasmacytoid dendritic cell neoplasm, and they also had new characteristics, including lineage other than T, B, myeloid and NK cells, and immunophenotypes of CD56(+) CD4(-) CD123(+) TdT(+) CD43(+) CD68(+) , CD56(+) CD4(+) CD123(-) TdT(+) CD43(+) CD68(-) and CD56(+) CD4(+) CD123(-/+) TdT(-) CD43(+) CD68(+) in the 3 cases, respectively. Bone marrow involvement was found 5 years later in case 1, and was then stable after chemotherapy; case 2 and case 3 were died 5 and 2 months after diagnosis, respectively.

CONCLUSIONBlastic plasmacytoid dendritic cell neoplasm is a heterogeneous group of lymphoproliferative disorders, with different clinical, morphologic and immunophenotypic features.

Adolescent ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bleomycin ; therapeutic use ; CD56 Antigen ; metabolism ; Cyclophosphamide ; therapeutic use ; Dendritic Cells ; metabolism ; pathology ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Hematologic Neoplasms ; drug therapy ; metabolism ; pathology ; Humans ; Interleukin-3 Receptor alpha Subunit ; metabolism ; Leukemia, Myeloid ; metabolism ; pathology ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; Male ; Middle Aged ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Prednisone ; therapeutic use ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; Treatment Outcome ; Vincristine ; therapeutic use

OBJECTIVETo study the clinicopathologic features of granulocytic sarcoma.

METHODSThe clinical and pathologic findings of 38 cases of granulocytic sarcoma were retrospectively analyzed. Immunohistochemical study was performed and the literature was reviewed.

RESULTSThe age of patients ranged from 2 to 77 years (mean = 43.3 years). The male-to-female ratio was 1.5:1. Major clinical presentations included superficial lymph node enlargement and painful soft tissue mass. Follow-up data were available in 18 patients; and 14 of them died of tumor-related diseases. The average duration of survival of the patients was 16.9 months. Histologically, the tumor cells were relatively uniform in appearance and small to medium in size. The cytoplasm was scanty and pale in color. The nuclei were round or focally irregular, with fine chromatin and inconspicuous nucleoli. Mitosis figures were readily identified. Scattered immature eosinophilic myelocytes were seen. Immunohistochemical study showed that the tumor cells in all cases expressed MPO and CD43. Most cases were also positive for CD68, lysozyme, CD99 and TdT. The staining for CD3, CD20, CD79a, pan-cytokeratin and PLAP were negative.

CONCLUSIONSGranulocytic sarcoma is a known histologic mimicker of non-Hodgkin lymphoma, Ewing sarcoma/PNET and embryonal rhabdomyosarcoma. Detailed morphologic examination, when coupled with immunohistochemical study, is useful in arriving at a correct diagnosis.

Adolescent ; Adult ; Aged ; Burkitt Lymphoma ; metabolism ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Leukosialin ; metabolism ; Lymph Nodes ; pathology ; Male ; Middle Aged ; Muscle Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Ovarian Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Peroxidase ; metabolism ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Retrospective Studies ; Sarcoma, Ewing ; metabolism ; pathology ; Sarcoma, Myeloid ; drug therapy ; metabolism ; pathology ; surgery ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Survival Rate ; Young Adult

OBJECTIVETo estimate the significance of the adjustment of acute lymphoblastic leukemia (ALL) risk group by monitoring minimal residual disease(MRD).

METHODTotally 285 children ALL patients who were diagnosed and systematically treated according to CCLG-2008 in Institute of Hematology and Blood Diseases Hospital, CAMS and PUMC, from April 2008 to August 2011 were prospectively selected. Among these cases, 62.8% (n = 179) were boys and 37.2% (n = 106) were girls and the median age was 5.3(0.5-14.0). The patients who were at high-risk group initially were excluded. The grouping of cases: the patients were divided into two groups according to the dates of initial diagnosis. Group I had 126 patients who were initially diagnosed between April 2008 and December 2009 in whom therapeutic regimen was not adjusted by reassignment of risk group by MRD. Group II had 159 patients who were initially diagnosed between January 2010 and August 2011 whose therapeutic regimen was adjusted by reassignment of risk group by MRD at specific time (33rd day of induction chemotherapy and 12 weeks after the beginning of chemotherapy). MP-FCM Coulter FC-500 was used in the detection of MRD.

RESULTAmong these 285 patients, 94.0% (n = 268) were diagnosed as B-lineage acute lymphoblastic leukemia and 6.0% (n = 17) were T-lineage acute lymphoblastic leukemia. In group I, 61.9% (n = 78) patients belonged to low-risk group, 38.1% (n = 48) median-risk; in group II, before the adjustment, the rates of the low-risk group and median-risk group were 68.6% (n = 109) and 31.4% (n = 50) , respectively, while after the adjustment they were altered to 53.5% (n = 85) and 39.6% (n = 63) , furthermore 6.9% (n = 11) patients went into the high-risk group. Both groups were followed up for 2.5 years after their diagnoses, the disease of 7.4% (n = 21) patients relapsed, and the rates of two groups were 12.7% (n = 16) and 3.1% (n = 5) respectively, P = 0.009. The rate of serious infection (such as sepsis, pulmonary infection) of all these patients was 32.3% (92/285) , there was no significant difference between the two groups [28.6% (36/126) vs.35.2% (56/159) , P = 0.392]. The mortality of all these patients was 6.7% (19/285) , and that of group I was higher than that of group II [10.3% (13/126) vs. 3.8% (6/159) , P = 0.044]. The 2.5 years overall survival (OS), event-free survival (EFS) and disease-free survival (DFS) of group I were all lower than those of group II in Kaplan-Meier survivorship analysis (all P < 0.05). The two groups were followed up for 2.5 years after their diagnoses, after elimination of the confounding influence of sex, age, FAB subtype, WBC count, ratio of blast cells in bone marrow at diagnosed, chromosome karyotype and fusion gene, reassignment of risk group by MRD was used to calculate the OS, EFS and DFS of ALL patients (all P < 0.05). After the adjustment the risk group was more significant in the assessment of prognosis.

CONCLUSIONThe reassignment of risk group in low and median risk groups children with acute lymphoblastic leukemia by MRD did not increase the rate of serious infection but could reduce the relapse rate and mortality, and was beneficial to increase the patients' OS, EFS and DFS.

Adolescent ; Antineoplastic Agents ; administration & dosage ; Antineoplastic Combined Chemotherapy Protocols ; administration & dosage ; therapeutic use ; Bone Marrow ; pathology ; Child ; Child, Preschool ; Disease-Free Survival ; Female ; Flow Cytometry ; Humans ; Infant ; Male ; Neoplasm, Residual ; diagnosis ; drug therapy ; pathology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; drug therapy ; pathology ; Prognosis ; Prospective Studies ; Recurrence ; Remission Induction ; Survival Rate ; Treatment Outcome