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Rev Esp Med Nucl Imagen Mol 2019 Apr 6. Epub 2019 Apr 6.

Servicio de Medicina Nuclear, Hospital Universitario Virgen del Rocío, Sevilla, España.

Blood Res 2019 Mar 21;54(1):63-73. Epub 2019 Mar 21.

Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, Korea.

Background: Acute leukemia (AL), not clearly assigned to myeloid, B-lymphoid, or T-lymphoid lineage, is classified as either biphenotypic acute leukemia (BAL) based on the European Group for Immunological Classification of Leukemias (EGIL) or acute leukemia of ambiguous lineage (ALAL) encompassing acute undifferentiated leukemia (AUL) and mixed-phenotype acute leukemia (MPAL) based on the World Health Organization (WHO) criteria.

Methods: Medical records of children newly diagnosed with BAL or ALAL, based on the EGIL or the 2008/2016 WHO criteria, respectively, admitted at Chonnam National University Hospital in 2001-2017 were retrospectively reviewed.

Results: Twelve (3. Read More

Haematologica 2018 Dec 4. Epub 2018 Dec 4.

Cancer Science Institute of Singapore.

Acute leukemia of ambiguous lineage (ALAL) is a rare group of blood cancers that cannot be clearly classified into either myeloid or lymphoid lineage through traditional immunophenotyping (2016 World Health Organization classification). In this study, we performed exome and transcriptome sequencing of 15 diagnosis/relapse samples to identify mutations of this disease. Remarkably, genes involved in DNA repair pathway were frequently mutated and occurred in 80% of the samples. Read More

Leuk Res 2018 08 18;71:6-12. Epub 2018 Jun 18.

Hospital de Pediatria prof. Dr. Juan P. Garrahan, Combate de los Pozos 1881, Buenos Aires, Argentina.

Several conventions have been established in order to define and characterize Mixed Phenotype Acute Leukemia (MPAL). However, megakaryocytic markers have not been included in the definition of MPAL neither in the European Group for the Immunological Characterization of Leukemias (EGIL) proposal nor in any of the WHO Classification of Tumors issues. We report four pediatric acute leukemia (AL) cases (prevalence: 0. Read More

Blood 2018 Jul 2;132(3):264-276. Epub 2018 May 2.

Department of Pediatric Hematology/Oncology, Charles University, Second Faculty of Medicine, Hospital Motol, Prague, Czech Republic.

Despite attempts to improve the definitions of ambiguous lineage leukemia (ALAL) during the last 2 decades, general therapy recommendations are missing. Herein, we report a large cohort of children with ALAL and propose a treatment strategy. A retrospective multinational study (International Berlin-Frankfurt-Münster Study of Leukemias of Ambiguous Lineage [iBFM-AMBI2012]) of 233 cases of pediatric ALAL patients is presented. Read More

Arch Med Res 2018 Jan 19;49(1):44-50. Epub 2018 Apr 19.

Departamento de Hematología, Unidad Médica de Alta Especialidad, Hospital de Especialidades, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Ciudad de México, México; Departamento de Morfología, Escuela Nacional de Ciencias Biológicas, Instituto Politécnico Nacional, Ciudad de México, México. Electronic address:

Background: Acute lymphoblastic leukemia is an aggressive malignant disease with high mortality rates in adults.

Aim Of The Study: The expression levels of CD95, active caspase-3, and Bcl-2 were determined in 111 patients with de novo acute lymphoblastic leukemia (ALL) and correlated with overall survival (OS) and disease-free survival (DFS).

Methods: The immunophenotyped ok leukemia and the expression of CD95, active caspase-3, and Bcl-2, were determined by flow cytometry. Read More

Indian J Pathol Microbiol 2018 Jan-Mar;61(1):58-65

Department of Pathology, Tata Memorial Hospital; Department of Pathology, Hematopathology Laboratory, Tata Memorial Hospital, Mumbai, Maharashtra, India.

Background: 2008 World Health Organization (WHO) classification of hematolymphoid neoplasms (HLN) has classified them based on morphology, results of various ancillary techniques, and clinical features. There are no studies looking at the applicability of WHO classification.

Aims: The aim of the study was to calculate proportions of all HLN subtypes seen during 1-year period based on 2008 WHO classification of HLN and study applicability and also shortcomings of practices in a tertiary care center in India. Read More

Ann Hematol 2018 Jun 15;97(6):945-953. Epub 2018 Mar 15.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 402, Houston, TX, 77030, USA.

Mixed phenotype acute leukemia (MPAL) is an uncommon diagnosis, representing only about 2-5% of acute leukemia cases. The blast cells of MPAL express multilineage immunophenotypic markers and may have a shared B/T/myeloid phenotype. Due to historical ambiguity in the diagnosis of MPAL, the genetics and clinical features of this disease remain poorly characterized. Read More

Mod Pathol 2018 07 14;31(7):1141-1154. Epub 2018 Feb 14.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

The Philadelphia chromosome resulting from t(9;22)(q34;q11.2) or its variants is a defining event in chronic myeloid leukemia. It is also observed in several types of de novo acute leukemia, commonly in B lymphoblastic leukemia, and rarely in acute myeloid leukemia, acute leukemia of ambiguous lineage, and T lymphoblastic leukemia. Read More

Indian J Hematol Blood Transfus 2018 Jan 21;34(1):178-180. Epub 2017 Mar 21.

Department of Laboratory Medicine, Rajiv Gandhi Cancer Institute and Research Center, Rohini, Delhi India.

Leuk Lymphoma 2018 05 7;59(5):1231-1238. Epub 2017 Sep 7.

a Department of Pathology , Johns Hopkins University School of Medicine , Baltimore , MD , USA.

Mixed-phenotype acute leukemia (MPAL) is a progenitor type of leukemia with ambiguous expression of lineage markers. The diagnosis of MPAL is based on flow cytometric analysis of immunophenotype, which commonly identifies myeloid lineage markers as well as B- or T- lymphoid lineage markers on leukemic blasts. Due to the rare occurrence of this disease, few studies have delineated the molecular bases of MPAL. Read More

Case Rep Oncol 2017 May-Aug;10(2):588-595. Epub 2017 Jul 7.

Department of Central Military Laboratory and Blood Bank, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

Natural killer (NK) cell lymphoblastic leukaemia/lymphoma is a rare haemopoietic tumour currently defined in the 2008 WHO classification under the category of acute leukaemias of ambiguous lineage. A diagnosis of this type of leukaemia is considered in cases expressing CD56 along with immature T-cell-associated markers such as CD2 and CD7 with absence of B-cell and myeloid markers; in addition, blastic plasmacytoid dendritic cell leukaemia should be excluded. Prior to 2008, these precursor NK cell lymphoblastic leukaemias/lymphomas were categorized as myeloid/NK cell acute leukaemia with a phenotype identical to acute myeloid leukaemia with minimal differentiation. Read More

Arch Pathol Lab Med 2017 Oct 22;141(10):1342-1393. Epub 2017 Feb 22.

Context: - A complete diagnosis of acute leukemia requires knowledge of clinical information combined with morphologic evaluation, immunophenotyping and karyotype analysis, and often, molecular genetic testing. Although many aspects of the workup for acute leukemia are well accepted, few guidelines have addressed the different aspects of the diagnostic evaluation of samples from patients suspected to have acute leukemia.

Objective: - To develop a guideline for treating physicians and pathologists involved in the diagnostic and prognostic evaluation of new acute leukemia samples, including acute lymphoblastic leukemia, acute myeloid leukemia, and acute leukemias of ambiguous lineage. Read More

Case Rep Pathol 2016 26;2016:8937940. Epub 2016 Dec 26.

Department of Pathology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Mixed phenotype acute leukemia (MPAL) is a rare neoplasm which accounts for 2-5% of all leukemias and it is classified under heading of acute leukemia of ambiguous lineage in 2008 WHO classification. This patient was a 61-year-old man who presented with malaise and weakness. In physical examination there was cervical and axillary lymphadenopathy. Read More

Clin Lymphoma Myeloma Leuk 2017 02 23;17(2):100-107. Epub 2016 Nov 23.

Division of Hematology/Oncology, University of Arkansas for Medical Sciences and Central Arkansas Veterans Healthcare System, Little Rock, AR.

Background: Acute leukemia of ambiguous lineage (ALAL) is a rare leukemia with sparse data availability about the survival and management strategies in elderly patients.

Methods: We used the Surveillance Epidemiology and End Results (SEER)-Medicare database to describe the overall survival (OS) and treatment pattern of elderly patients (age > 65 years) with ALAL. OS analysis was done using the Kaplan-Meier method, and its determinants were analyzed using the Cox proportional hazard regression method with a significant P < . Read More

Genes Dev 2016 11 2;30(22):2486-2499. Epub 2016 Dec 2.

Department of Clinical and Experimental Medicine, Experimental Hematopoiesis Unit, Faculty of Health Sciences, Linköping University, 58183 Linkoping, Sweden.

Even though leukemia is considered to be confined to one specific hematopoietic cell type, cases of acute leukemia of ambiguous lineage and patients relapsing in phenotypically altered disease suggest that a malignant state may be transferred between lineages. Because B-cell leukemia is associated with mutations in transcription factors of importance for stable preservation of lineage identity, we here investigated the potential lineage plasticity of leukemic cells. We report that primary pro-B leukemia cells from mice carrying heterozygous mutations in either or both the Pax5 and Ebf1 genes, commonly mutated in human leukemia, can be converted into T lineage leukemia cells. Read More

Int J Hematol Oncol Stem Cell Res 2016 Jul;10(3):138-46

Professor, Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, India.

Background: Acute leukemias are characterized by neoplastic proliferation of hematopoietic stem cells and accumulation of blasts and immature cells in bone marrow. We applied a selective panel of immunohistochemical markers on bone marrow trephine tissue sections and observed their utility in diagnosis and typing of acute leukemia.

Materials And Methods: The study was done at PSG Institute of Medical Sciences and Research from 1st January, 2008 to 30th June, 2012. Read More

J Pediatr Oncol 2015;3(1):24-28. Epub 2015 Mar 25.

Division of Oncology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

We describe the case of a 16 year-old female with mixed phenotype acute leukemia B/myeloid, NOS (formerly biphenotypic leukemia) with masked hypodiploidy and somatic and deletions. She achieved morphologic remission with lymphoid-directed multi-agent chemotherapy, but experienced an early medullary relapse 11 months from initial diagnosis. Her case details the unusual finding of hypodiploidy in a patient with ambiguous lineage leukemia and highlights the complexity of therapy selection for these high-risk patients. Read More

Am J Clin Pathol 2015 Sep;144(3):361-76

Immunology Laboratory, University Hospital of Nancy-Brabois, Nancy, France.

Objectives: This session of the Society for Hematopathology/European Association for Haematopathology Workshop focused on acute leukemias of ambiguous origin.

Methods: We provide an overview of mixed-phenotype acute leukemia (MPAL) as recognized in the current World Health Organization classification and summarize diagnostic criteria for major categories of MPAL: B/myeloid, T/myeloid, B/T, and B/T/myeloid.

Results: Most MPAL cases submitted were B/myeloid and T/myeloid MPAL, the most frequent types, but three cases of B/T MPAL were also submitted, and examples of all categories are illustrated. Read More

Ann Hematol 2015 Oct 11;94(10):1761-3. Epub 2015 Jul 11.

Department of Pathology and Laboratory Medicine, Division of Hematopathology, Eastern Ontario Regional Laboratory Association, The Ottawa Hospital, Ottawa, Canada,

Pediatr Dev Pathol 2015 Jan-Feb;18(1):76-9. Epub 2014 Dec 1.

1 Department of Pathology, Children's Medical Center, Parkland Health and Hospital System, and UT Southwestern Medical Center, Dallas, Texas, USA.

We describe a case of acute leukemia of ambiguous lineage with a novel cytogenetic abnormality. A 1-year-old boy presented with abnormal complete blood count findings, and was found to have blasts and mild dysgranulopoiesis. The blasts showed immunophenotypic evidence of myeloid and T-lineage differentiation. Read More

Sci Rep 2014 Nov 6;4:6942. Epub 2014 Nov 6.

Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.

Conventional myeloablative conditioning (MAC) regimens often cause severe regimen-related toxicity (RRT). Furthermore, many patients suffer from poor quality of life in accordance with the increase in long-term survivors. We therefore devised a reduced-toxicity myeloablative conditioning (RTMAC) regimen consisting of 8-Gy total body irradiation (TBI), fludarabine (FLU) and cyclophosphamide (CY) for pediatric hematological malignancies. Read More

Leuk Res Rep 2014 2;3(2):33-5. Epub 2014 May 2.

Department of Internal Medicine, Texas Tech University, Paul L. Foster School of Medicine, 4801 Alberta Avenue, El Paso, TX 79905, USA.

We describe a patient with acute leukemia of ambiguous lineage who had trisomy 4 as the sole cytogenetic abnormality. Clinical, pathological, immunophenotypic and molecular features are presented and compared with the previous 4 published cases. Over expression of c-kit, which is localized to chromosome 4, was documented on the leukemic blasts. Read More

Indian J Hematol Blood Transfus 2013 Jun 3;29(2):119-22. Epub 2012 May 3.

Department of Haematology, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi, 110060 India.

We report an unusual case of bi-lineal mixed-phenotype acute leukemia (T/Myeloid, NOS) with complex cytogenetic abnormalities in a 2-year-old boy. Despite attaining complete remission with therapy, he succumbed to status epilepticus following febrile illness. Flow cytometry represents the current standard of care for the diagnosis of this malignancy and the approach adopted in our case is discussed. Read More

Mod Pathol 2014 May 1;27(5):651-6. Epub 2013 Nov 1.

Special Hematology Laboratory, Division of Hematopathology, University of Minnesota Medical Center, Fairview, Minneapolis, MN, USA.

Four patients presented with acute leukemia of ambiguous or myeloid lineage in association with Langerhans cell histiocytosis and provide evidence suggesting a common origin of the two neoplasms. One patient had a non-constitutional trisomy 21 in both the leukemic blasts and the Langerhans cells indicative of a clonal relationship. A second case expressed CD2, CD13, and CD117 on both the Langerhans cells and the blasts suggesting a possible clonal relationship. Read More

Ann Lab Med 2013 Sep 8;33(5):371-4. Epub 2013 Aug 8.

Department of Laboratory Medicine, Chung-Ang University College of Medicine, Seoul, Korea.

Br J Haematol 2013 Oct 25;163(1):24-39. Epub 2013 Jul 25.

Laboratory of Health Physics & Enviromental Health, Department of Cytogenetics, National Centre for Scientific Research (NCSR) "Demokritos", Aghia Paraskevi, Athens, Greece.

Acute leukaemia of ambiguous lineage (ALAL) is a rare complex entity with heterogeneous clinical, immunophenotypic, cytogenetic and molecular genetic features and adverse outcome. According to World Health Organization 2008 classification, ALAL encompasses those leukaemias that show no clear evidence of differentiation along a single lineage. The rarity of ALAL and the lack of uniform diagnostic criteria have made it difficult to establish its cytogenetic features, although cytogenetic analysis reveals clonal chromosomal abnormalities in 59-91% of patients. Read More

Clin Cancer Res 2013 Apr 26;19(8):2187-96. Epub 2013 Feb 26.

Department of Hematology, VU University Medical Center, Cancer Center Amsterdam, Amsterdam, The Netherlands.

Purpose: Classification of acute leukemia is based on the commitment of leukemic cells to the myeloid or the lymphoid lineage. However, a small percentage of acute leukemia cases lack straightforward immunophenotypical lineage commitment. These leukemias of ambiguous lineage represent a heterogeneous category of acute leukemia that cannot be classified as either acute myeloid leukemia (AML) or acute lymphoid leukemia (ALL). Read More

Ann Hematol 2013 Jun 15;92(6):747-58. Epub 2013 Feb 15.

Department of Hematology, Oncology and Tumor Immunology, Charité University Hospital Berlin, Hindenburgdamm 30, 12203, Campus Benjamin Franklin, Berlin, Germany.

Acute leukemias of ambiguous lineage represent a heterogeneous group of rare, poorly characterized leukemias with adverse outcome. No larger studies have yet performed a combined approach of molecular and clinical characterization of acute undifferentiated leukemia (AUL) and biphenotypic acute leukemia (BAL) in adults. Here we describe 16 adults with AUL and 26 with BAL and performed mutational as well as expression studies of genes with prognostic impact in acute leukemia (BAALC, ERG, MN1, WT1, and IGFBP7). Read More

Cytometry B Clin Cytom 2013 Mar 16;84(2):114-8. Epub 2013 Jan 16.

Department of Hematology, Cancer Centre Amsterdam, VU University Medical Center, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands.

Background: According to WHO 2008 guidelines, an important role is designated for cytoplasmic myeloperoxidase (cMPO) as measured by flow cytometry for classifying acute leukemia of myeloid or ambiguous origin (AML or MPAL). However, no threshold with respect to expression level and percentage positive cells is provided. Since the expression of solely cMPO can change the diagnosis from acute lymphoid leukemia into MPAL in the current WHO 2008, a consensus is needed for the cut-off for cMPO. Read More

Ann Hematol 2013 May 30;92(5):679-87. Epub 2012 Dec 30.

Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou Dadao North Street no. 1838, Guangzhou, Guangdong, China.

Knowledge concerning the clinical and biological characteristics of acute leukemia of ambiguous lineage (ALAL) is limited so that there has been a lack of uniformity in treatment. In this report, we retrospectively investigated the effect of intensified conditioning on adult ALAL undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). A total of 59 patients with ALAL (male in 37 cases and female in 22 cases) were consecutively enrolled in the data analyses. Read More

Cytometry B Clin Cytom 2012 May 19;82(3):186-91. Epub 2012 Mar 19.

Department of Pathology and Laboratory Medicine, Roswell Park Cancer institute, Buffalo, New York 14263, USA.

Semin Diagn Pathol 2012 Feb;29(1):12-8

Laboratoire d'Immunologie du CHU and Nancy Université, Vandoeuvre lès Nancy, France.

The 2008 edition of the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues recognizes a special category called "leukemias of ambiguous lineage." The vast majority of these rare leukemias are classified as mixed phenotype acute leukemia (MPAL), although acute undifferentiated leukemias and natural killer lymphoblastic leukemias are also included. The major immunophenotypic markers used by the WHO 2008 to determine the lineage for these proliferations are myeloperoxidase, CD19, and cytoplasmic CD3. Read More

Hematol Oncol Clin North Am 2011 Dec;25(6):1235-53

Adult Leukemia Program, Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Suite D1B30 (Mayer 1B21), Boston, MA 02215, USA.

From the standpoint of the hematopathologist, attempts to dissect the immunophenotype and other lineage-defining characteristics of the puzzling group of acute leukemias of ambiguous origin have prompted considerable discussion and debate. For clinicians, however, such definitions, although academically interesting, as yet give relatively little insight into the most appropriate therapy, and patients with MPAL continue to do poorly compared with more typical AML or ALL cases. The most recent WHO 2008 MPAL definitions are provocative, but represent a major change from the previous EGIL BAL classification, and the clinical relevance of this change has yet to be established. Read More

Zhonghua Yi Xue Za Zhi 2011 May;91(20):1379-83

Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.

Objective: To evaluate the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the conditioning of different intensities for acute leukemias of ambiguous lineage (ALAL).

Methods: A total of 38 ALAL patients were treated with two conditioning of different intensities in our hospital from March 2002 to August 2010. The standard conditioning included TBI + Cy or Bu + Cy, intensified conditioning included Fludarabine + Ara-C + TBI + Cy. Read More

Exp Oncol 2010 Sep;32(3):195-9

RE Kavetsky Institute of Experimental Pathology, Oncology and Radiobiology of NAS of Ukraine, Vasylkivska str 45, Kyiv 03022, Ukraine.

The study included 1742 patients with acute myeloblastic leukaemias (AML) and acute lymphoblastic leukaemias (ALL), Kyiv city residents and patients from 20 regions of Ukraine. Bone marrow and blood smears were sent at diagnosis to Reference Center. The analysis was based on May-Grünvald-Giemza (MGG) stain and cytochemical reactions (MPO, acNSE, CAE, AP, PAS). Read More

Rinsho Ketsueki 2011 Jan;52(1):23-7

Department of Medicine, Yamaguchi Prefectural Medical Center.

A 67-year-old female was admitted with a diagnosis of acute leukemia. Immature blasts did not show cytoplasmic granules and were POX(-), ES(-), and PAS(+). Flow cytometry of leukemic cells demonstrated positivity for CD7, CD10, CD19, CD13, CD34, HLA-DR, and coexpression of CD7 and CD34, CD10 and HLA-DR, and CD19 and CD13. Read More

Rinsho Ketsueki 2010 May;51(5):339-44

Department of Hematology, Kanagawa Cancer Center.

A 47-year-old man was admitted to our hospital in June 2009 because of fatigue and blast cells in peripheral blood. Bone marrow examination showed that 67% leukemic cells were positive for myeloperoxidase (MPO) and negative for esterase stain. Flow cytometric analysis (FCM) revealed the expressions of CD2, cyCD3, CD5, TdT, CD13 on the blasts. Read More

Am J Hematol 2010 Jun;85(6):451-4

The majority of cases of acute leukemia belong to a specific lineage origin, either lymphoid or myeloid, and therefore are classified as acute lymphoblastic leukemia (ALL) or acute myelogenous leukemia (AML), based on morphologic features and cytochemical and immunophenotypic profile of the blast cells. A minority of acute leukemias however, show no clear evidence of differentiation along a single lineage. These are now classified under acute leukemias of ambiguous lineage by the most recent WHO classification and account for <4% of all cases of acute leukemia [1]. Read More

Leukemia 2010 Jul 20;24(7):1392-6. Epub 2010 May 20.

Br J Haematol 2010 Apr 18;149(1):84-92. Epub 2010 Jan 18.

Department of Paediatric Haematology/Oncology, Hannover Medical School, Germany.

Acute leukaemias of ambiguous lineage (ALAL) represent a rare type of leukaemia, expressing both myeloid and lymphoid markers. This study retrospectively analyzed data from 92 children (biphenotypic n = 78, bilineal n = 6, lineage switch n = 8) with ALAL registered in the Berlin-Frankfürt-Münster (BFM) acute myeloid leukaemia (AML) and acute lymphoblastic leukaemia (ALL) studies between 1998 and 2006 (2.4% of all cases with acute leukaemia). Read More

Nihon Rinsho 2009 Oct;67(10):1853-62

Hematoimmunology, School of Health Science, Faculty of Medicine, University of the Ryukyus.

Myeloid leukemia in this series corresponds to the myeloid neoplasms of the 4th WHO classification of pathology and genetics of tumor of haematopoietic and lymphoid tissue. The myeloid neoplasms are composed of six categories, which are 1) myeloproliferative neoplasms (MPN), a new category of 2) myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1, 3) myelodysplastic syndrome (MDS)/MPN, 4) MDS, 5) acute myeloid leukemia (AML) and related precursor neoplasms, and 6) acute leukemias of ambiguous lineage. In MPNs without chronic myelogenous leukemia, the genetic marker of JAK2 V617F is added to the diagnostic criteria for polycythemia vera, essential thrombocythemia and primary myelofibrosis. Read More

Leuk Res 2010 Apr 29;34(4):438-46. Epub 2009 Sep 29.

Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital G Gennimatas, Mesogion Avenue 154, Athens 11527, Greece.

We present a cohort of 22 patients with type 2 dendritic cell (DC2) acute leukemia (or blastic plasmacytoid dendritic cell neoplasm-BPDCN, as it has been recently named), diagnosed in Greece over the past 12-year period, according to the main clinical and immunophenotypic features of this entity. Four additional cases are discussed, classified as leukemia of ambiguous lineage (LAL), because of the simultaneous detection of a CD56 negative DC2 population and of a second myeloid precursor cell population. The morphological features and cytogenetic findings of the typical BPDCN cases were similar to those previously described. Read More

Haematologica 2009 Dec 27;94(12):1682-90. Epub 2009 Aug 27.

Deprtment of Pediatric, Hematology-Oncology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

Background: Knowledge concerning the clinical and biological presentation, as well as the outcome of treatment, of biphenotypic acute leukemia in children is limited.

Design And Methods: This retrospective review analyzes the clinical features and outcome of children with biphenotypic acute leukemia diagnosed and treated over an 8-year period. According to the EGIL scoring system 24 (3. Read More

Intern Med 2009 17;48(16):1437-41. Epub 2009 Aug 17.

Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Kagawa University.

Biphenotypic acute leukemia (BAL) is a rare entity that comprises 0.5-3% of all acute leukemias and probably arises from multipotent progenitor cells. The optimal approach for BAL therapy is unknown. Read More

Ceylon Med J 2009 Jun;54(2):51-3

Department of Pathology, Faculty of Medicine, Colombo, Sri Lanka.

Acute leukaemia of ambiguous lineage (ALAL) is a rare form of leukaemia in which morphologic, cytochemical and immuno-phenotypic features of the proliferating blasts lack sufficient evidence to classify them as myeloid or lymphoid in origin or have characteristics of both myeloid and lymphoid cells. We report a 22-year-old man presenting with clinical features of an acute lymphoblastic leukaemia but blasts in his blood and bone marrow with morphological features of myeloblasts. His immuno-phenotyping by flowcytometry showed antigens specific for both myeloid and B-lymphoid lineages. Read More

Haematologica 2009 Jul;94(7):891-3

Acta Med Croatica 2008 Oct;62(4):387-90

Zavod za imunologiju i Referentni centar za imunodijagnostiku imunoloskih i hematoloskih bolesti Ministarstva zdravstva Republike Hrvatske, Klinicki bolnicki centar Zagreb, Zagreb, Hrvatska.

Human acute leukemias (AL) are classified as myeloid or lymphoid according to cytomorphology and the expression of leukocyte differentiation antigens/CD-markers. However, in the minority of cases leukemic cells express markers of more than one lineage, which has led to the introduction of a new subgroup of acute leukemias termed mixed or biphenotypic acute leukemias (BAL). In an effort to distinguish between BAL and those AL with aberrant expression of markers of other lineage, the European Group for the Immunological Characterization of Acute Leukemias (EGIL) has proposed a scoring system in which CD-markers are assigned a score of 0. Read More

Acta Med Croatica 2008 Oct;62(4):379-85

Zavod za citologiju, Klinicki zavod za patologiju i citologiju, Klinicki bolnicki centar Zagreb, Zagreb, Hrvatska.

Unlabelled: Biphenotypic acute leukemias (AL) with blasts expressing both myeloid and lymphoid antigens are grouped with undifferentiated AL and bilineal AL in the group of AL of ambiguous lineage. Not all AL with myeloid and lymphoid antigens (ALMy+Ly) are true biphenotypic AL. According to EGIL scoring system, true biphenotypic ALMy+Ly are those with a sum of antigens 2 or more points for both myeloid and lymphoid lineage or for B and T lineage. Read More

Int J Hematol 2008 Mar 7;87(2):144-151. Epub 2008 Feb 7.

Department of Hematology, Atomic Bomb Disease Institute, Nagasaki University School of Medicine, Nagasaki, Japan.

We reviewed and categorized 638 of 809 patients who were registered in the Japan Adult Leukemia Study Group acute myeloid leukemia (AML)-97 protocol using morphological means. Patients with the M3 subtype were excluded from the study group. According to the WHO classification, 171 patients (26. Read More