Humans ; Leukemia, Biphenotypic, Acute ; Leukocytosis

Biphenotypic acute leukemia (BAL) is a subtype of acute leukemia that expresses two different immunophenotypic lineages, most commonly myeloid and either B- or T-lymphoid lineages. This entity has been defined by a scoring system proposed by the European Group for the Immunological Characterization of Leukemias (EGIL). The prognosis of BAL is regarded as being worse than either acute lymphoid or myeloid leukemia that does not show lineage ambiguity. However, a treatment strategy for BAL has not yet been established. We experienced a case of BAL with the t(8;21) translocation, a favorable cytogenetic rearrangement in acute myeloid leukemia (AML). The patient was successfully treated with cytarabine and anthracycline for induction and consolidation. The quantitative value of the AML1-ETO gene decreased after achieving complete hematologic remission. Thus, the AML1-ETO gene rearrangement in BAL may be associated with an acceptable response to the treatment strategy for AML.
Cytarabine ; Cytogenetics ; Gene Rearrangement ; Humans ; Leukemia ; Leukemia, Biphenotypic, Acute ; Leukemia, Myeloid ; Leukemia, Myeloid, Acute ; Prognosis

OBJECTIVETo highlight the current understanding of mixed phenotype acute leukemia (MPAL).

DATA SOURCESWe collected the relevant articles in PubMed (from 1985 to present), using the terms "mixed phenotype acute leukemia", "hybrid acute leukemia", "biphenotypic acute leukemia", and "mixed lineage leukemia". We also collected the relevant studies in WanFang Data base (from 2000 to present), using the terms "mixed phenotype acute leukemia" and "hybrid acute leukemia".

STUDY SELECTIONWe included all relevant studies concerning mixed phenotype acute leukemia in English and Chinese version, with no limitation of research design. The duplicated articles are excluded.

RESULTSMPAL is a rare subgroup of acute leukemia which expresses the myeloid and lymphoid markers simultaneously. The clinical manifestations of MPAL are similar to other acute leukemias. The World Health Organization classification and the European Group for Immunological classification of Leukaemias 1998 criteria are most widely used. MPAL does not have a standard therapy regimen. Its treatment depends mostly on the patient's unique immunophenotypic and cytogenetic features, and also the experience of individual physician. The lack of effective treatment contributes to an undesirable prognosis.

CONCLUSIONOur understanding about MPAL is still limited. The diagnostic criteria have not been unified. The treatment of MPAL remains to be investigated. The prognostic factor is largely unclear yet. A better diagnostic criteria and targeted therapeutics will improve the therapy effect and a subsequently better prognosis.

Humans ; Leukemia, Biphenotypic, Acute ; diagnosis ; metabolism ; MicroRNAs ; metabolism

Pyoderma gangrenosum is a neutrophilic dermatosis that may be associated acute leukemia. We report a case of pyoderma gangrenosum associated refractory acute biphenotypic leukemia wherein the malignant cells show a phenotype specific for myelogenous and lymphocytic leukemia. Histopathologic examination revealed moderate perivascular infiltrate consisting mainly of neutrophils and lymphocytes without involvement of leukemic cells in the skin lesions. Treatment with systemic steroid was followed by a characteristically rapid healing of the skin lesions.
Leukemia ; Leukemia, Biphenotypic, Acute* ; Leukemia, Lymphoid ; Lymphocytes ; Neutrophils ; Phenotype ; Pyoderma Gangrenosum* ; Pyoderma* ; Skin ; Skin Diseases

A case of acute biphenotypic leukemia with mixed blast morphology and combined myeloid and T-lymphoid features is reported. The leukemic cells consisted of small to medium sized hand mirror shaped blasts and large blasts with cytoplasmic granules and some cytoplasmic inclusion bodies. The blast cells were found to be immature T-lymphoid cells(CD2+ and CD7+) that also expressed the myeloid antigens such as CD13 and CD33. In the review of the literatures, additional cases of acute mixed leukemia-hand mirror variant show strong expression of adhesion moleules such as CD2, CD7, and CD11b. Cytogenetic studies revealed a trisomy 4 previously described in acute undifferentiated myeloblastic leukemia with hand-mirror cells. This case represents a morphologically and phenotypically distinct subtype of acute biphenotypic leukemia with hand mirror morphologic features. Adult acute leukemia cases with hand mirror morphology should be considered the possibility of mixed lineage leukemia and processed further studies.
Adult* ; Cytogenetics ; Cytoplasmic Granules ; Granulocyte Precursor Cells ; Hand ; Humans ; Inclusion Bodies ; Leukemia ; Leukemia, Biphenotypic, Acute ; Trisomy

No abstract available.
Leukemia, Biphenotypic, Acute ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Paraproteinemias ; Thrombocythemia, Essential

OBJECTIVETo explore the relationship between EGIL 1998 and WHO2008 criteria for the diagnosis of mixed phenotype acute leukemia (MPAL).

METHODSThe same group patients were diagnosed for MPAL by EGIL 1998 and WHO2008 criteria, respectively. The coincidence rate of diagnosis and therapeutic response of two diagnosis standards were compared.

RESULTSA cohort of 1835 de novo acute leukemia (AL) patients admitted to our hospital from February 1996 to October 2010 were retrospectively analyzed by applying both EGIL1998 and WHO2008 classification criteria. Seventy four patients were diagnosed with MPAL according to EGIL 1998, accounting for 4.0% of all AL cases. The main subtype is M/B (54 cases, 73.0%). While 81 patients were diagnosed as MPAL, accounting for 4.4% based on WHO 2008 criteria. The most common type is also M/B (63 cases, 77.8%). Fifty nine (79.7%) cases met both criteria. In the subtypes of M/B, M/T, M/B/T and B/T, the coincidence rate is 85.2% (46/54), 56.3% (9/16), 0(0/2) and 50.0% (1/2), respectively. In the 1761 cases excluded as MPAL by EGIL1998, 22 cases can be diagnosed as MPAL by WHO 2008 classification. (2) Among the patients diagnosed as B/M MPAL by WHO2008, 13 were MPAL with t (9; 22) (q34; q11.2)/BCR-ABL1, 1 was MPAL with t (v; 11q23)/MLL-rearranged, 49 was B/myeloid not otherwise specified (NOS), 14 was T/myeloid NOS and 4 was MPAL NOS-rare types. (3) The overall final complete remission rate of the patients diagnosed by EGIL1998, 2008 WHO, met both criteria, EGIL1998 but excluded by 2008 WHO and by 2008 WHO but excluded by EGIL1998 was 69.0%, 73.5%, 73.5%, 44.4% and 73.7%, respectively, with no significant difference between any two groups based on χ(2) test (P > 0.05).

CONCLUSIONSEGIL 1998 and WHO2008 criteria have reciprocity in the diagnosis of MPAL.

Adolescent ; Adult ; Aged ; Female ; Humans ; Immunophenotyping ; Leukemia, Biphenotypic, Acute ; diagnosis ; pathology ; Male ; Middle Aged ; Young Adult

BACKGROUND: Granulocyte colony-stimulating factor (G-CSF) is commonly used to reduce leukopenic period during treatment of malignancy including acute leukemia. Leukemic blasts expressing granulocyte colony-stimulating factor receptor (G-CSFR) were reported and also may proliferate in response to therapeutic administration of G-CSF. However, it is not clear whether G-CSFR expression on leukemic blasts is related to clinical outcome such as leukocyte recovery or leukemia relapse. Current study evaluated expression of G-CSFR in acute leukemia and correlated with hematologic and clinical parameters. METHODS: Peripheral blood or bone marrow aspirate was evaluated from 20 patients with acute myelogenous leukemia (AML) and 10 with acute lymphoblastic leukemia (ALL), 2 with acute undifferentiated leukemia (AUL), 1 with acute biphenotypic leukemia (ABL), 1 with acute mixed-lineage leukemia (AMLL). G-CSFR expression was analyzed using flow cytometry and was correlated with immunophenotype and response for chemotherapy. RESULTS: More than 20% of blasts were positive for G-CSFR in 65% (13/20) of AML, 40% (4/10) of ALL, and all negative in ABL, AMLL, and AUL. Except that all 6 monocytic lineage leukemias (M4, M5) and all three cases of ALL with CD33 expression were positive, no consistent correlation was observed among G-CSFR expression pattern, type of acute leukemia, response to induction therapy and relapse (P>0.05). CONCLUSION: Current study revealed G-CSFR was expressed on not only myelogenous leukemic cells but also lymphoid ones. Although our data suggest G-CSFR expression does not affect therapeutic outcome, it remains to be determined whether G-CSF therapy is safe in G-CSFR-positive acute leukemia.
Bone Marrow ; Drug Therapy ; Flow Cytometry ; Granulocyte Colony-Stimulating Factor* ; Granulocytes* ; Humans ; Leukemia* ; Leukemia, Biphenotypic, Acute ; Leukemia, Myeloid, Acute ; Leukocytes ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence

Biphenotypic acute leukemia (BAL) is a subtype of leukemia of ambiguous lineage in the World Health Organization classification system. About one third of the cases have the Philadelphia chromosome, and some cases are associated with other structural abnormalities involving 11q23. BAL is known to have a poor prognosis in both children and adults. According to the previously reported BAL cases with positive BCR-ABL fusion gene, most of the BCR-ABL mRNA transcript type was e1a2. So, we describe here a 30-year-old adult BAL case with the karyotype 46,XY,t(9;22)(q34;q11.2) resulting in a very rare b3a2 type of BCR-ABL mRNA transcript.
Adult ; Child ; Classification ; Humans ; Karyotype ; Leukemia ; Leukemia, Biphenotypic, Acute* ; Philadelphia Chromosome ; Prognosis ; RNA, Messenger* ; World Health Organization

This study was aimed to explore the expression of CD34 in patients with biphenotypic acute leukemia (BAL) and its relation with the prognosis of BAL. The flow cytometry was used to detect leukemia-associated antigen. The used monoclonal antibodys (McAb) included CD10, CD19 and CD34 for B lymphocyte lineage, CD2, CD3 and CD5 for T lymohocyte lineage, MPO, CD13 and CD33 for myeloid lineage. The finally results were respectively analyzed. The results indicated that 9 out of 216 cases of leukemia was diagnosed as BAL (4.2%). Among 9 cases of BAL, 6 cases showed the common expression of myeloid and T lymohocyte lineages (66.7%), 3 cases showed the common expression of myeloid and B lymohocyte lineages (33.3%). 4 cases of BAL displayed CD34 positive expression (44.4%). As compared with acute myelogenous leukemia (AML) and acute lymphoblastic leukemia (ALL), the BAL patients showed higher CD34 positive expression (P < 0.05). It is concluded that the BAL patients show a poor prognosis, as compared with AML or ALL patients. The therapeutic effect of BAL may negatively correlate with the CD34 positive expression.
Aged ; Antigens, CD34 ; metabolism ; Flow Cytometry ; Humans ; Immunophenotyping ; Leukemia, Biphenotypic, Acute ; diagnosis ; metabolism ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis