Intensive Therapy of Myelodysplastic Syndromes and Secondary Leukemias: Preliminary Findings of the French Experience

134 patients with a prior diagnosis of a malignant solid tumor presented between 1 Jan 95 and 31 Dec 04 to the leukemia service at PMH for management of a secondary hemopoietic malignancy or aplasia (AML n=99, MDS n=22, ALL n=11, AA n=2). The cohort included 65 males (median age 70 years, ranging from 18–94 years) and 69 females (median age 60 years, ranging from 25–87 years). The tumor types showed the expected gender based variation. Based on age, performance status and patient preference 81 individuals received remission induction therapy, while 53 patients were managed with supportive care. 49 of the patients undergoing induction therapy met the age criteria (70 years) for a blood and marrow transplant (BMT) and survived at least 70 days post initiation of induction therapy. 25 of the 49 patients underwent allogeneic BMT from related (n=20) or unrelated donors (n=5). The median survival of all 134 patients amounted to 314 days with an overall survival (OS) at 2 and 3 years of 25% and 20%. The respective values for patients undergoing induction therapy and supportive care were 413 days,36%,28% and 192 days, 9%,7% (p= 0.0002). Survival was strongly influenced by age (p= 0.0017), while gender (p=0.1436) and FAB subtype (p=0.219) did not contribute significantly to outcome. Subset analysis of the 25 BMT recipients showed a median survival of 922 days, with a 51% and 39.5 % OS at 2 and 3 years. The respective data for the remaining 24 not transplanted patients were not significantly different (p= 0.7508) with 522 days, 42% and 35%. Both subgroups however differed significantly in their causes of death favoring a lower relapse rate for transplant recipients and lower treatment related mortality (TRM) for non-transplanted patients (p=0.0012). In conclusion, this single center study confirms the relatively poor outcome for patients with secondary leukemias but is consistent with the view that patients able to undergo intensive therapy including transplantation may derive a significant survival benefit. The low relapse rate observed for BMT patients compared to patients not transplanted may translate into a survival benefit provided TRM can be reduced

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Impact of Age and Comorbidity in Myelodysplastic Syndromes

Journal of the National Comprehensive Cancer Network ◽

10.6004/jnccn.2008.0070 ◽

2008 ◽

Vol 6 (9) ◽

pp. 927-934 ◽

Cited By ~ 31

Author(s):

Reinhard Stauder ◽

Thomas Nösslinger ◽

Michael Pfeilstöcker ◽

Wolfgang R. Sperr ◽

Friedrich Wimazal ◽

...

Keyword(s):

Risk Assessment ◽

Elderly Patients ◽

Myelodysplastic Syndromes ◽

Intensive Therapy ◽

Functional Decline ◽

Surrogate Marker ◽

Scoring Systems ◽

The Elderly ◽

Western World ◽

Comorbidity Scores

Myelodysplastic syndromes (MDS) represent a heterogeneous group of myeloid neoplasms that are preferentially diagnosed in the elderly. With the increase in older patients with MDS in the Western world and the availability of more therapeutic options, new strategies and algorithms for optimal management and treatment of these patients must be developed. Although age is recognized as an important adverse variable affecting survival, most scoring systems have not included age in score risk calculations. Comorbidity is of particular importance and a frequent covariable in elderly patients with MDS. However, although comorbidity scores have been established and used for risk assessment in younger high-risk patients scheduled to undergo intensive therapy, these scores are only just being applied to elderly patients, with relevant results. Advanced age should not exclude a patient with MDS from appropriate treatment, and age alone should not be considered a surrogate marker for functional decline or comorbidities. This article discusses the need to improve scoring systems, individualized risk-assessment, and treatment algorithms for elderly patients with MDS by including age and comorbidities.

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Intensive therapy for high-risk myelodysplastic syndromes and the biological significance of karyotype abnormalities

Leukemia Research ◽

10.1016/s0145-2126(98)00037-x ◽

1998 ◽

Vol 22 ◽

pp. S23-S26 ◽

Cited By ~ 5

Author(s):

W Hiddemann ◽

G Jahns-Streubel ◽

W Verbeek ◽

B Wörmann ◽

D Haase ◽

...

Keyword(s):

High Risk ◽

Myelodysplastic Syndromes ◽

Intensive Therapy ◽

Biological Significance

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Additional Prognostic Value of Bone Marrow Histology in Patients Subclassified According to the International Prognostic Scoring System for Myelodysplastic Syndromes

Journal of Clinical Oncology ◽

10.1200/jco.2003.04.182 ◽

2003 ◽

Vol 21 (2) ◽

pp. 273-282 ◽

Cited By ~ 51

Author(s):

E. Verburgh ◽

R. Achten ◽

B. Maes ◽

A. Hagemeijer ◽

M. Boogaerts ◽

...

Keyword(s):

Bone Marrow ◽

Myelodysplastic Syndromes ◽

Scoring System ◽

Lower Risk ◽

Prognostic Value ◽

Predictive Power ◽

Intensive Therapy ◽

International Prognostic Scoring System ◽

Prognostic System ◽

Bone Marrow Histology

Purpose: The most recent and powerful prognostic instrument established for myelodysplastic syndromes (MDS) is the International Prognostic Scoring System (IPSS), which is primarily based on medullary blast cell count, number of cytopenias, and cytogenetics. Although this prognostic system has substantial predictive power in MDS, further refinement is necessary, especially as far as lower-risk patients are concerned. Histologic parameters, which have long proved to be associated with outcome, are promising candidates to improve the prognostic accuracy of the IPSS. Therefore, we assessed the additional predictive power of the presence of abnormally localized immature precursors (ALIPs) and CD34 immunoreactivity in bone marrow (BM) biopsies of MDS patients. Patients and Methods: Cytogenetic, morphologic, and clinical data of 184 MDS patients, all from a single institution, were collected, with special emphasis on the determinants of the IPSS score. BM biopsies of 173 patients were analyzed for the presence of ALIP, and CD34 immunoreactivity was assessable in 119 patients. Forty-nine patients received intensive therapy. Results: The presence of ALIP and CD34 immunoreactivity significantly improved the prognostic value of the IPSS, with respect to overall as well as leukemia-free survival, in particular within the lower-risk categories. In contrast to the IPSS, both histologic parameters also were predictive of outcome within the group of intensively treated MDS patients. Conclusion: Our data confirm the importance of histopathologic evaluation in MDS and indicate that determining the presence of ALIP and an increase in CD34 immunostaining in addition to the IPSS score could lead to an improved prognostic subcategorization of MDS patients.

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A Paraneoplastic Syndrome Characterized by Extremity Swelling with Associated Inflammatory Infiltrate Heralds Aggressive Transformation of Myelodysplastic Syndromes/Myeloproliferative Neoplasms to Acute Myeloid Leukemia: A Case Series

Case Reports in Hematology ◽

10.1155/2012/582950 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

James K. Mangan ◽

Selina M. Luger

Keyword(s):

Acute Myeloid Leukemia ◽

Myelodysplastic Syndromes ◽

Myeloid Leukemia ◽

Myeloproliferative Neoplasms ◽

Intensive Therapy ◽

Myeloproliferative Neoplasm ◽

Case Series ◽

Disease Status ◽

Inflammatory Syndrome ◽

Acute Myeloid

There has been a long history of reports describing a variety of paraneoplastic phenomena associated with myelodysplastic syndromes, particularly those with autoimmune manifestations. We report here a series of patients with an antecedent myelodysplastic syndrome (MDS) or myeloproliferative neoplasm (MPN) that underwent aggressive transformation to acute myeloid leukemia (AML). In each case, the transformation to AML was preceded by an inflammatory syndrome characterized by unilateral extremity swelling and an associated inflammatory skin infiltrate, as well as other signs of inflammation, including profound hyperferritinemia without evidence of a hemophagocytic syndrome. We suggest that such an inflammatory syndrome may herald aggressive transformation of MDS/MPN to AML. Patients with known MDS/MPN who present with these features may benefit from early bone marrow examination to assess disease status. Early intervention with corticosteroids in select patients may result in improvement or resolution of the symptoms and permit intensive therapy for AML to be delivered.

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Intensive Stuttering Therapy With Telepractice Follow-up: A Case Study

Perspectives on Fluency and Fluency Disorders ◽

10.1044/ffd21.1.11 ◽

2011 ◽

Vol 21 (1) ◽

pp. 11-21 ◽

Cited By ~ 2

Author(s):

Farzan Irani ◽

Rodney Gabel

Keyword(s):

Case Report ◽

Therapeutic Intervention ◽

Intensive Therapy ◽

Positive Outcome ◽

Eclectic Approach

This case report describes the positive outcome of a therapeutic intervention that integrated an intensive, residential component with follow-up telepractice for a 21 year old male who stutters. This therapy utilized an eclectic approach to intensive therapy in conjunction with a 12-month follow-up via video telepractice. The results indicated that the client benefited from the program as demonstrated by a reduction in percent stuttered syllables, a reduction in stuttering severity, and a change in attitudes and feelings related to stuttering and speaking.

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