Stem cell transplant in the treatment of childhood biphenotypic acute leukemia

Park, Jeong A; Ghim, Thad T.; Bae, Keun Wook; Im, Ho Joon; Jang, Seong Soo; Park, Chan Jeong; Sook, Hyun; Seo, Jong Jin

doi:10.1002/pbc.22105

Cited by 28 publications

(55 citation statements)

References 38 publications

(40 reference statements)

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“…Two other studies that specifically evaluated pediatric patients used different immunophenotypic diagnostic criteria: Park et al used the EGIL system, stringent than those proposed by the EGIL. 9 While our overall incidence rate is in agreement with the rates in previously published series which included both adult and pediatric patients 6,7 and with the rate in the pediatric series in which the EGIL diagnostic system was used, 10 when we utilized the more stringent, new WHO diagnostic criteria we found an incidence similar to that reported by Rubnitz et al 9 The phenotype distribution in our patients was no different from previously published distributions, with the majority of patients having a B-lymphoid/myeloid phenotype and the T-lymphoid/myeloid phenotype being the next common phenotype. Only two of our patients had a B-lymphoid/T-lymphoid phenotype.…”

Section: Discussionsupporting

confidence: 80%

“…This incidence of MLL gene rearrangement in our cohort appears somewhat higher than previously reported, 6,7,10 but is consistent with the incidence found in studies confined to the pediatric age group, 9,10 and may in fact be a reflection of the younger overall populations in these studies.…”

Section: Discussioncontrasting

confidence: 53%

“…Two recently published studies, focusing only on pediatric patients, reported incidences of 2% and 4.4%. 9,10 The diagnostic criteria used in the two studies were, however, different.…”

Section: Introductionmentioning

confidence: 99%

“…The need for hematopoietic stem cell transplantation (HSCT) in first remission also remains contentious. 7,[9][10][11][12][13][14] The Leukemia Team at our institution elected to treat these patients using a strategy based on the St. Jude Total XIII-B high risk protocol. 15 While this protocol was originally developed for the treatment of patients with high-risk acute lymphocytic leukemia (ALL), it incorporates several agents which are effective in the treatment of myeloid leukemias.…”

Section: Introductionmentioning

confidence: 99%

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Clinical characteristics and outcome of children with biphenotypic acute leukemia

Al-Seraihy¹,

Owaidah²,

Ayas³

et al. 2009

Haematologica

102

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BackgroundKnowledge concerning the clinical and biological presentation, as well as the outcome of treatment, of biphenotypic acute leukemia in children is limited. Design and MethodsThis 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 (3.7%) of 633 patients with acute leukemia were classified as having biphenotypic acute leukemia. The diagnostic work-up and results were reviewed specifically for this study in the light of the newly published WHO criteria for the diagnosis of leukemia of ambiguous lineage. Based on these criteria, 11 (1.7%) patients were categorized according to the new nomenclature as having mixed phenotype acute leukemia. The majority of the patients (58.3%) had a B-lymphoid/myeloid phenotype, followed by the T-lymphoid/myeloid phenotype. The most frequent chromosomal abnormality involved the 14q32 locus. Patients received therapy based on a treatment regimen for acute lymphocytic leukemia regimen, which included myeloid-effective agents. ResultsAt a median follow up of 4 years (range, 6 month -7 years) the overall survival rate was 75.7% and the event-free survival rate was 73.5%. The survival of those patients who underwent hematopoietic stem cell transplantation in first complete remission was not different from that of the patients who were treated with chemotherapy alone (overall survival: 70.1% versus 81.1%, respectively, p=0.39; event-free survival: 70.1% versus 76.2%, respectively, p=0.75). The outcome of the 11 patients who were retrospectively classified as having mixed phenotype acute leukemia according to the new WHO criteria was excellent, with no relapses or deaths occurring among these patients. ConclusionsAn acute lymphocytic leukemia type of induction therapy, using agents that are active against lymphoid and myeloid leukemias, appears to be more effective in achieving and maintaining complete remissions regardless of whether the patients are classified according to EGIL criteria or the new WHO criteria. Hematopoietic stem cell transplantation may not be necessary for all patients in first complete remission.Key words: biphenotypic acute leukemia, diagnosis, therapy, immunophenotyping, stem cell transplant.Citation: Al-Seraihy AS, Owaidah TM, Ayas M, El-Solh H, Al-Mahr M, Al-Ahmari A, and Belgaumi AF. Clinical characteristics and outcome of children with biphenotypic acute leukemia. Haematologica 2009; 94:1682-1690. doi:10.3324/haematol.2009

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Section: Discussionsupporting

confidence: 80%

Section: Discussioncontrasting

confidence: 53%

“…Two recently published studies, focusing only on pediatric patients, reported incidences of 2% and 4.4%. 9,10 The diagnostic criteria used in the two studies were, however, different.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical characteristics and outcome of children with biphenotypic acute leukemia

Al-Seraihy¹,

Owaidah²,

Ayas³

et al. 2009

Haematologica

102

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“…Его распро-страненность среди всех ОЛ не превышает 2-5% [7][8][9]. Тем не менее необходимость выделения данного варианта обосновыва-ется прежде всего неудовлетворительными результатами лечения как цитостатическими препаратами, так и после выполнения трансплантации аллогенных гемопоэтических стволовых клеток [2,[10][11][12][13].…”

Section: терапевтический архив 7 2015unclassified

The specific features of diagnosis of mixed-phenotype acute leukemia: A combination of B-cell antigen expressions according to the results of flow cytometry and morphological markers of myeloid differentiation in blast cells: A clinical case

et al. 2015

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Согласно классификации ВОЗ, выделяют 3 вида острого лейкоза (ОЛ) [1]: острый миелоидный лейкоз (ОМЛ) и родствен-ные неоплазии из миелоидных предшественников; острый лим-фобластный лейкоз/лимфома (ОЛЛ); наконец, ОЛ, для обозна-чения которого предложен термин «ambiguous» (неоднозначный, двусмысленный), который может быть интерпретирован как «не-определенный».Данный термин представляется вполне оправданным, если принять во внимание, что при этом варианте ОЛ невозможно корректно верифицировать ни ОМЛ, ни ОЛЛ. Речь идет об остром недифференцированном лейкозе и ОЛ со смешанным фенотипом (mixed phenotype acute leukemia -MPAL) -ОЛ-СФ. В первом случае отсутствуют маркеры линейной принадлежно-сти бластных клеток (БК), во втором одновременно выявляется экспрессия миелоидных и/или лимфоидных (В-и/или Т-клеточных) маркеров (не путать с аберрантной коэкспрессией лимфоидных или миелоидных маркеров на бластных клетках ОМЛ или ОЛЛ!). АннотацияДиагностика редкого варианта острого лейкоза, бластные клетки которого экспрессируют миелоидные и/или лимфоидные маркеры, осуществляется преимущественно по данным проточной цитометрии. Приведен клинический случай острого лейкоза со смешанным фенотипом, при котором методом проточной цитометрии выявлена экспрессия В-лимфоидных антигенов, в то время как в морфологических препаратах костного мозга обнаружены признаки, типичные для бластных клеток миелоидной направленности. Сделано заключение о необходимости комплексного обследования больных с впер-вые выявленным острым лейкозом и совокупным анализом результатов проточной цитометрии с данными морфологиче-ского и цитохимического исследований. Ключевые слова: острый лейкоз, смешанный фенотип.This rare type of acute leukemia, blast cells of which express myeloid and/or lymphoid markers, is mainly diagnosed using flow cytometric findings. The paper describes a clinical case of mixed-phenotype acute leukemia, in which B-cell lymphoid antigen expressions were revealed by a flow cytometric technique, while bone marrow morphological specimens showed the signs of myeloid differentiation specific to blast cells. It is concluded that there is a need for a comprehensive examination of patients with new-onset acute leukemia and for an aggregate analysis of flow cytometric results with morphological and cytochemical findings.

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Prognostic impact of Philadelphia chromosome in mixed phenotype acute leukemia (MPAL): A cancer registry analysis on real‐world outcome

2020

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Mixed phenotype acute leukemia (MPAL) is thought to have poor outcome, and presence of the Philadelphia chromosome (Ph+) has been considered to be an adverse prognostic marker. However, most of these reports were in the pre‐tyrosine kinase inhibitors (TKIs) era. Recent limited reports indicate improved outcomes for MPAL with the addition of TKIs. We examined the outcomes of 241 cases of MPAL according to the 2008 WHO classification from the Surveillance, Epidemiology, and End Results registry. The MLL+ patients had a median age of 6 years while other subtypes occurred mostly in adults and had comparable age. On multivariate analyses and after adjustment for age, year of diagnosis and chemotherapy status, Ph+ MPAL patients had reduced risk of death in comparison to Ph(–) MPAL patients (hazard ratio [HR] = 0.28, P = .002). So, MLL+ MPAL had the worst outcome with a 10‐fold increased risk of death in comparison to Ph+ MPAL patients (HR = 10.2, P < .001). Importantly, the outcome of Ph+ MPAL was comparable to Ph+ acute lymphoblastic leukemia in a 1:1 matched case‐control analysis. In conclusion, this is the largest registry study which examines the outcomes of MPAL subtypes. We confirm that MPAL is a heterogenous disease. Note, Ph+ MPAL nowadays has a better OS in comparison to other subtypes and is comparable to Ph+ ALL patients. This is most likely secondary to changes in practice and more utilization of TKIs. On the other hand, MLL rearrangement is associated with infantile MPAL and has a dismal prognosis.

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Stem cell transplant in the treatment of childhood biphenotypic acute leukemia

Cited by 28 publications

References 38 publications

Clinical characteristics and outcome of children with biphenotypic acute leukemia

Clinical characteristics and outcome of children with biphenotypic acute leukemia

The specific features of diagnosis of mixed-phenotype acute leukemia: A combination of B-cell antigen expressions according to the results of flow cytometry and morphological markers of myeloid differentiation in blast cells: A clinical case

Prognostic impact of Philadelphia chromosome in mixed phenotype acute leukemia (MPAL): A cancer registry analysis on real‐world outcome

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