Lymphoblastic transformation of myelodysplastic syndrome

Lima, Carmen Sílvia Passos; Souza, Cármino Antonio De; Cardinalli, Izilda Aparecida; Lorand-Metze, Irene

doi:10.1590/s1516-31801997000400009

Cited by 11 publications

(4 citation statements)

References 30 publications

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“…Similar to other studies, none of the patients in this series transformed to acute lymphoblastic leukemia. 45,46 The recently published IPSS for MDS has been shown to be an improved method for evaluating prognosis of patients with MDS. The percentage of our patients who fell into the int-2 or high risk IPSS groups (61%) was significantly greater than the percentage of patients Ͻ60 years in the equivalent groups in the IPSS study (28%), and also significantly greater than all patients (29%) in the IPSS study, which again may represent selection bias for referral of high-risk patients to a tertiary bone marrow transplantation center.…”

Section: Discussionmentioning

confidence: 99%

Primary myelodysplasia occurring in adults under 50 years old: a clinicopathologic study of 52 patients

et al. 2002

Leukemia

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Although myelodysplastic syndromes (MDSs) are generally thought to be diseases of elderly patients, younger patients also have rarely been diagnosed with MDS. This is a report of the clinical, morphologic and cytogenetic features of 52 cases of primary MDS occurring in adults under the age of 50 years. Cases secondary to chemotherapy or radiotherapy were excluded. There were 31 males and 21 females. The median age at presentation was 39 years (range, 18 to 49 years). The interval between onset of symptoms and diagnosis was brief (median, 4 weeks; range, 1-32 weeks). Of the 49 patients for whom information about duration of symptoms was available, 13 (27%) were asymptomatic. Forty-two (81%) of the patients were classified using FAB criteria for blood and bone marrow morphology: refractory anemia (RA), 11; refractory anemia with ringed sideroblasts (RARS), four; refractory anemia with excess blasts (RAEB), 12; chronic myelomonocytic leukemia (CMML), three; refractory anemia with excess blasts in transformation (RAEB-T), 12 patients. Ten patients could not be categorized. Abnormalities involving chromosome 7 was the most frequent cytogenetic abnormality (31%). Partial chromosomal deletion and chromosome gain were also common abnormalities (22% and 9%, respectively). Translocations accounted for only 9% of the main cytogenetic abnormalities encountered in this patient population. For the 49 patients for whom information regarding AML transformation was available, 23 (47%) progressed to acute myeloid leukemia, with an overall median time to progression of 2 months (range 3 weeks to 3 years). In each category except for RARS, approximately half of the patients progressed, with a slightly less median time to progression in RAEB-T than for the other subtypes of MDS. Thirteen patients underwent bone marrow transplantation at the time of presentation of their disease. Leukemia (2002) 16, 623-631.

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Section: Discussionmentioning

confidence: 99%

Primary myelodysplasia occurring in adults under 50 years old: a clinicopathologic study of 52 patients

et al. 2002

Leukemia

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“…With newer diagnostic methods these children might have been diagnosed as having AML instead (possible AML‐M0). Alternatively, the flawed early stem cell in SDS may be predisposed to malignant lymphoblastic transformation, as is sometimes seen in adults with MDS 59. The juvenile myelomonocytic leukemia diagnosed in an 8‐year‐old SDS patient reported by Caselitz and colleagues (1979) 26 might now have been diagnosed as AML.…”

Section: Clinical Featuresmentioning

confidence: 99%

Shwachman‐Diamond syndrome

Dror

2005

Pediatric Blood & Cancer

124

156

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Shwachman-Diamond syndrome (SDS) is an inherited marrow failure disorder with varying cytopenia, pancreatic dysfunction, and metaphyseal dysostosis. SDS is also characterized by a risk of myelodysplasia and leukemia in up to one third of the patients. Over the last 5 years, major advances have been made in understanding the bone marrow phenotype. The gene associated with the disease, SBDS, has recently been identified. Herein we provide an update on the clinical features, the hematopoietic defects, and the genetics of the disease as they are currently understood. We also review the diagnostic and therapeutic approaches to the hematological complications in the syndrome.

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“…This shows that the origin of this disorder is at the same level as a common progenitor cell, which gives rise to both hematopoietic as well as lymphoid origin cells. [3][4][5][6][7][8][9] The APA is a hematological illness, and the molecular mechanism behind this abnormal immune system and insuf-ciencies in hematopoietic cells is genetic, namely mutations in repairing genes of the telomeres and the dysregulated pathways of T-cell stimulation. 10 Some case studies have reported this type of conversion of APA to ALL.…”

Section: Introductionmentioning

confidence: 99%