Childhood monosomy 7 revisited

Evans, Jane; Czepulkowski, Barbara; Gibbons, Barbara; Swansbury, G. J.; Chessells, Judith M.

doi:10.1111/j.1365-2141.1988.tb07600.x

Cited by 51 publications

(18 citation statements)

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“…[20][21][22][23] The monosomy 7 syndrome has many similarities with JMML and the distinction between the two nosological entities has not been clear-cut. In a previous study we did not find any major clinical differences between JMML in children with and without −7.…”

Section: Introductionmentioning

confidence: 99%

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

et al. 1999

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We reviewed the clinical features, treatment, and outcome of 100 children with myelodysplastic syndrome (MDS), juvenile myelomonocytic leukemia (JMML), and acute myeloid leukemia (AML) associated with complete monosomy 7 (−7) or deletion of the long arm of chromosome 7 (7q−). Patients with therapyinduced disease were excluded. The morphologic diagnoses according to modified FAB criteria were: MDS in 72 (refractory anemia (RA) in 11, RA with excess of blasts (RAEB) in eight, RAEB in transformation (RAEB-T) in 10, JMML in 43), and AML in 28. The median age at presentation was 2.8 years (range 2 months to 15 years), being lowest in JMML (1.1 year). Loss of chromosome 7 as the sole cytogenetic abnormality was observed in 75% of those with MDS compared with 32% of those with AML. Predisposing conditions (including familial MDS/AML) were found in 20%. Three-year survival was 82% in RA, 63% in RAEB, 45% in JMML, 34% in AML, and 8% in RAEB-T. Children with −7 alone had a superior survival than those with other cytogenetic abnormalities: this was solely due to a better survival in MDS (3-year survival 56 vs 24%). The reverse was found in AML (3-year survival 13% in −7 alone vs 44% in other cytogenetic groups). Stable disease for several years was documented in more than half the patients with RA or RAEB. Patients with RA, RAEB or JMML treated with bone marrow transplantation (BMT) without prior chemotherapy had a 3-year survival of 73%. The morphologic diagnosis was the strongest prognostic factor. Only patients with a diagnosis of JMML fitted what has previously been referred to as the monosomy 7 syndrome. Our data give no support to the concept of monosomy 7 as a distinct syndrome.

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Section: Introductionmentioning

confidence: 99%

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

et al. 1999

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“…18 In children, monosomy 7 has an equally poor outcome and is the most common cytogenetic abnormality in preleukemia, usually occurring as an isolated finding. [1][2][3][4][5][6][7] Two of the patients described in the current study (Patients 3 and 4) were treated with alkylating agents as part of their chemotherapy regimens. Patient 5 had de novo MDS and received chemotherapy after he was diagnosed with infantile monosomy 7.…”

Section: Discussionmentioning

confidence: 99%

“…L oss of chromosome 7 (monosomy 7) or deletions of its long arm [del(7q)] are recurring, nonrandom chromosomal abnormalities frequently found in children and adults with malignant myeloid disorders. [1][2][3][4][5][6][7] Monosomy 7/del(7q) is by far the most common cytogenetic abnormality observed in children with both adult type myelodysplastic syndromes (MDS) as well as juvenile myelomonocytic leukemia (JMMC). In young children, monosomy 7/del(7q) frequently is associated with leukocytosis, hepatosplenomegaly, a high risk of transformation to myeloid leukemia, poor response to chemotherapy, and death within 2 years of diagnosis from infections and bleeding.…”

Section: Discussionmentioning

confidence: 99%

“…In young children, monosomy 7/del(7q) frequently is associated with leukocytosis, hepatosplenomegaly, a high risk of transformation to myeloid leukemia, poor response to chemotherapy, and death within 2 years of diagnosis from infections and bleeding. [1][2][3][4][5][6][7] Monosomy 7 syndrome is more common in males and 90% of patients are diagnosed before age 5 years. 2 Monosomy 7/del(7q) also is associated with prior chemotherapy with alkylating agents and is the most common cytogenetic abnormality detected in the bone marrow of children and adults with treatment-related myelodysplastic syndromes (t-MDS).…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4][5][6][7] Monosomy 7 syndrome is more common in males and 90% of patients are diagnosed before age 5 years. 2 Monosomy 7/del(7q) also is associated with prior chemotherapy with alkylating agents and is the most common cytogenetic abnormality detected in the bone marrow of children and adults with treatment-related myelodysplastic syndromes (t-MDS). 8,9 Although the overall prognosis for patients who develop myeloid disorders associated with monosomy 7/del(7q) is poor, to our knowledge three case reports describing spontaneous hematologic remission have been published in the English literature.…”

Section: Discussionmentioning

confidence: 99%

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Myelodysplastic Syndromes

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SynopsisThe myelodysplastic syndromes are a diverse group of clonal stem cell disorders characterized by ineffective hematopoiesis, peripheral cytopenias, and an increased propensity to evolve to acute myeloid leukemia. The molecular pathogenesis of these disorders is poorly understood, but recurring chromosomal abnormalities occur in ~50% of cases, and are the focus of much investigation. The availability of newer molecular techniques has allowed the identification of additional genetic aberrations, including mutations and epigenetic changes of prognostic and potential therapeutic importance. This review will focus on the key role of cytogenetic analysis in MDS in the context of the diagnosis, prognosis, and pathogenesis of these disorders.Keywords myelodysplastic syndromes; cytogenetics; molecular genetics; diagnosis; prognosis; classification IntroductionThe myelodysplastic syndromes (MDS) include a large spectrum of clonal hematopoietic stem cell disorders that are characterized by peripheral cytopenia(s), morphologic dysplasia, ineffective hematopoiesis, and a variable propensity to transform to acute myeloid leukemia (AML). 1,2 The cytopenia can be limited to a single cell line resulting in anemia, This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author Manuscript thrombocytopenia, or neutropenia, and can be chronic and somewhat indolent, or profound involving all three lineages with life-threatening consequences. The morphologic dysplasia associated with the ineffective hematopoiesis may be subtle and difficult to recognize but, in some cases, it can be impressive and evident in both the bone marrow and peripheral blood. The variable increase in blasts relates, in part, to the risk for transformation to AML, although progression is not solely dependent on the blast percentage. MDS is a disease of older adults with a median age at diagnosis of ~70 years 3 . Other risk factors for the development of MDS include tobacco use and exposure to solvents, such as benzene and agricultural chemicals. In ~10-15% of cases, the disease arises as a late complication of cytotoxic therapy (radiotherapy and/or chemotherapy) for a prior disorder, and is referred to as a therapy-related myeloid neoplasm (t-MN). MDS is frequently associated with clonal cytogenetic abnormalities, of significant prognostic 4,5 and emerging therapeutic importance. An in-depth analysis of some of these is providing significant insights into underlying molecular alterations, which may hold clues to unraveling the pathogenesis of these disorders. This review will focus on the ...

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Childhood monosomy 7 revisited

Cited by 51 publications

References 18 publications

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

Myelodysplastic syndrome, juvenile myelomonocytic leukemia, and acute myeloid leukemia associated with complete or partial monosomy 7

Transient monosomy 7

Myelodysplastic Syndromes

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