Familial chronic myelocytic leukaemia‐like syndrome probably of congenital origin

Lardi, Amer A; Taha, Omer M. A.; Al‐Jefri, Abdulla; Ahmed, Mirghani A

doi:10.1111/j.1651-2227.1994.tb13080.x

Cited by 3 publications

(2 citation statements)

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“…This suggests a new assumption that three origins of MPN exist: (i) sporadic, (ii) familial associated with a genetic heterogeneity or multifactorial inheritance (iii) an AD inheritance with variable penetrance. Still the possibility of an AR inheritance cannot be excluded, especially as three case studies with consanguinity have been excluded from this study (21, 41). Among families with AD trait, there were no significant differences in frequency of paternal or maternal pairs.…”

Section: Discussionmentioning

confidence: 91%

Inheritance of the chronic myeloproliferative neoplasms. A systematic review

et al. 2012

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This systematic review investigated the inheritance of the classical chronic myeloproliferative neoplasms (MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and chronic myelogenous leukemia (CML). Sixty-one articles were included and provided 135 families with a total of 341 participants distributed to various subtypes of MPN: 50% PV, 23% ET, 14% PMF, 10% CML and 3% non-MPN hematological disorder. Women developed the disease earlier than men (43.1 years vs 47.3 years; p = 0.074), while the general average age of onset was 46 years, notably younger than sporadic cases. The clinical phenotype of the families showed a homogenous (67%) and a heterogeneous (33%) pattern, with the majority being PV-PV pairs (36%) and PV-PMF pairs (17%), respectively. This observation suggests that the susceptibility gene (or genes) is not restricted to one subtype supporting the hypothesis of a mutation in an early multipotent stem cell. Furthermore, a major subgroup of families provided evidence of an autosomal dominant (AD) inheritance with reduced penetrance. This study suggests that the origin of MPNs may occur in at least three different settings: (i) a sporadic, (ii) genetic heterogeneity with polygenetic and environmental impact and (iii) a familial phenotype following an AD inheritance.

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Section: Discussionmentioning

confidence: 91%

Inheritance of the chronic myeloproliferative neoplasms. A systematic review

et al. 2012

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“…Accordingly, many multiple-case families with hematological neoplasms (HN) have been described and this has led to the suggestion of the genetic susceptibility to these disorders [1, 2, 3, 4]. We read the article by Shpilberg et al [5]with great interest.…”

Section: Introductionmentioning

confidence: 76%

Increased General Predisposition to Cancer but Not to Specific Neoplasms of the Hematopoietic System in Children with Hematological Neoplasms: A Hypothesis

Roganović

Badovinac²

2000

Acta Haematol

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Familial Leukemias

Wiernik

2015

Curr. Treat. Options in Oncol.

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Familial leukemia has been described for more than 50 years but only recently have modern genetic techniques allowed for the investigation of the genome. Genome-wide association studies have identified a number of genetic sites that appear to relate to susceptibility to leukemia in certain families and occasionally to susceptibility to a specific leukemia in general. Many questions remain, including susceptibility to what? An oncogenic virus? An environmental chemical? Mutation of another gene induced by a heritable mutation-promoting gene?.Clinically important facts have been learned. Chronic lymphocytic leukemia (CLL) is by far the most common familial leukemia. Patients with CLL have approximately a 10% chance of a first-degree relative developing CLL, and even a greater chance of one developing monoclonal B-cell lymphocytosis which may be an asymptomatic forme fruste of the neoplasm. Furthermore, there may be an increased incidence of breast cancer in familial CLL pedigrees which raises the question of a common etiology for neoplasms in general, or at least a previously unrecognized relationship among them.

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Familial chronic myelocytic leukaemia‐like syndrome probably of congenital origin

Cited by 3 publications

References 5 publications

Inheritance of the chronic myeloproliferative neoplasms. A systematic review

Inheritance of the chronic myeloproliferative neoplasms. A systematic review

Increased General Predisposition to Cancer but Not to Specific Neoplasms of the Hematopoietic System in Children with Hematological Neoplasms: A Hypothesis

Familial Leukemias

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