Презентация на тему Myelodysplastic Syndromes

Презентация на тему Презентация на тему Myelodysplastic Syndromes, предмет презентации: Медицина. Этот материал содержит 44 слайдов. Красочные слайды и илюстрации помогут Вам заинтересовать свою аудиторию. Для просмотра воспользуйтесь проигрывателем, если материал оказался полезным для Вас - поделитесь им с друзьями с помощью социальных кнопок и добавьте наш сайт презентаций ThePresentation.ru в закладки!

Слайды и текст этой презентации

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MYELODYSPLASTIC SYNDROMESDr. Fineman
Текст слайда:

MYELODYSPLASTIC SYNDROMES

Dr. Fineman


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MDS• Clonal stem cell disorders• Maturation defects• Ineffective hematopoiesis• Blood cytopenias• Risk of transformation to AML
Текст слайда:

MDS

• Clonal stem cell disorders
• Maturation defects
• Ineffective hematopoiesis
• Blood cytopenias
• Risk of transformation to AML


Слайд 3
FEATURES USED TO DEFINE MDS • Blood cytopenias• Ineffective hematopoiesis• Dyserythropoiesis• Dysgranulopoiesis• Dysmegakaryopoiesis• Increased myeloblasts
Текст слайда:

FEATURES USED TO DEFINE MDS

• Blood cytopenias
• Ineffective hematopoiesis
• Dyserythropoiesis
• Dysgranulopoiesis
• Dysmegakaryopoiesis
• Increased myeloblasts


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FAB CLASSIFICATION OF MDS • Refractory Anemia• Refractory Anemia with Ringed
Текст слайда:

FAB CLASSIFICATION OF MDS

• Refractory Anemia
• Refractory Anemia with Ringed Sideroblasts
• Refractory Anemia with Excess Blasts (RAEB)
• Refractory Anemia with Excess Blasts in
Transformation (RAEB-T)
• Chronic Myelomonocytic Leukemia (CMML)


Слайд 8
WHO CLASSIFICATION OF MDS• Refractory anemia• Refractory anemia with ringed sideroblasts•
Текст слайда:

WHO CLASSIFICATION OF MDS

• Refractory anemia
• Refractory anemia with ringed sideroblasts
• Refractory cytopenia with multilineage dysplasia (RCMD) with or without ringed sideroblasts
• Refractory anemia with excess blasts (RAEB)
• RAEB type 1 (5%-9% blasts)
• RAEB type 2 (10-19% blasts)
• MDS, unclassifiable
• MDS associated with isolated del (5q) chromosome abnormality (“5q- syndrome”)


Слайд 9
MAJOR CHANGES IN WHO CLASSIFICATION OF MDS • Blast count
Текст слайда:

MAJOR CHANGES IN WHO CLASSIFICATION OF MDS

• Blast count for a diagnosis of AML is reduced from 30% to 20%
• RAEB-T is eliminated
• Refractory cytopenia with multilineage dysplasia (RCMD) is added
• 5q- syndrome is added
• CMML is incorporated into a bridging MDS-MPD classification


Слайд 10
REFRACTORY ANEMIA (RA)• Only erythroid lineage involved• Normocytic or macrocytic anemia•
Текст слайда:

REFRACTORY ANEMIA (RA)

• Only erythroid lineage involved
• Normocytic or macrocytic anemia
• Reticulocytopenia
• Usually normal or ↑RBC precursors
• Variable dyserythropoiesis
• <5% blasts in BM, none in blood


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REFRACTORY CYTOPENIA WITH MULTILINEAGE DYSPLASIA (RCMD) • Usually bicytopenia or pancytopenia• Multilineage dysplasia•
Текст слайда:

REFRACTORY CYTOPENIA WITH MULTILINEAGE DYSPLASIA (RCMD)

• Usually bicytopenia or pancytopenia
• Multilineage dysplasia
• <5% blasts in blood or BM
• May have ringed sideroblasts
• Worse prognosis than for RA and RARS and a higher incidence of cytogenetic abnormalities


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MDS WITH ISOLATED del(5q) • Refractory macrocytic anemia• Thrombocytosis• Hypolobulated megakaryocytes• Stable clinical course
Текст слайда:

MDS WITH ISOLATED del(5q)

• Refractory macrocytic anemia
• Thrombocytosis
• Hypolobulated megakaryocytes
• Stable clinical course


Слайд 17
REFRACTORY ANEMIA WITH EXCESS BLASTS (RAEB)• Usually pancytopenia or bicytopenia• Multilineage
Текст слайда:

REFRACTORY ANEMIA WITH EXCESS BLASTS (RAEB)

• Usually pancytopenia or bicytopenia
• Multilineage dysplasia
• 5% to 19% blasts in blood or BM
• Type 1 - 5-9% blasts
• Type 2 - 10-19% blasts
• May have ringed sideroblasts
• Absence of specific AML cytogenetic abnormalities


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REFRACTORY ANEMIA WITH EXCESS BLASTS (RAEB)
Текст слайда:

REFRACTORY ANEMIA WITH EXCESS BLASTS (RAEB)


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MYELODYSPLASTIC SYNDROME UNCLASSIFIABLE (MDS-U) • Cytopenias• No or rare blasts in blood• Unilineage dysplasia•
Текст слайда:

MYELODYSPLASTIC SYNDROME UNCLASSIFIABLE (MDS-U)

• Cytopenias
• No or rare blasts in blood
• Unilineage dysplasia
• <5% marrow blasts
• No Auer rods


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MYELODYSPLASTIC SYNDROME WITH INCREASED MARROW FIBROSIS • Pancytopenia• Trilineage
Текст слайда:

MYELODYSPLASTIC SYNDROME WITH INCREASED MARROW FIBROSIS

• Pancytopenia
• Trilineage dysplasia
• Bone marrow fibrosis
• ↑and dysplastic megakaryocytes
• No splenomegaly
• ? poor survival


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HYPOCELLULAR MDS • Cellularity of marrow
Текст слайда:

HYPOCELLULAR MDS

• Cellularity of marrow <30%; (<20% in patients over 60 yrs)
• ~ 10% of cases
• More common in therapy related MDS
and in children with MDS
• RA, RCMD, and RAEB most common


Слайд 23
PROGNOSTIC INDICATORS IN MDS• Age• Gender• Bone marrow blast %• # of cytopenias• Cytogenetics
Текст слайда:

PROGNOSTIC INDICATORS IN MDS


• Age
• Gender
• Bone marrow blast %
• # of cytopenias
• Cytogenetics


Слайд 24
Cytogenetics in prognosis of MDS
Текст слайда:

Cytogenetics in prognosis of MDS


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DIFFERENTIAL DIAGNOSIS OF MDS • Non-neoplastic causes of myelodysplasia• Megaloblastic changes•
Текст слайда:

DIFFERENTIAL DIAGNOSIS OF MDS

• Non-neoplastic causes of myelodysplasia
• Megaloblastic changes
• Toxic agents, i.e., heavy metals, acute alcohol intoxication
• Drug effects
• Congenital dyserythropoietic anemia
• Chronic infectious disease
• Acquired immunodeficiency syndrome (AIDS)

• Neoplastic Diseases
• Chronic myeloproliferative disease
• Acute myeloid leukemia


Слайд 26
PROBLEM AREAS IN THE DISTINCTION OF MDS AND AML •
Текст слайда:

PROBLEM AREAS IN THE DISTINCTION OF MDS AND AML

• Borderline blast counts
• Cases with >50% erythroid precursors
• Cases with criteria of MDS with a cytogenetic abnormalities specific for AML


(Rosati S, Anastasi J, Vardiman J. Sem Hematol 23:


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DISTINCTION OF MDS FROM CHRONIC MYELOPROLIFERATIVE DISORDERSMDSCMPDCytopeniasIneffective hematopoiesisDysplasiaNo splenomegalyNormal or elevated blood countsEffective hematopoiesisNo dysplasiaSplenomegaly
Текст слайда:

DISTINCTION OF MDS FROM CHRONIC MYELOPROLIFERATIVE DISORDERS

MDS

CMPD

Cytopenias

Ineffective hematopoiesis
Dysplasia
No splenomegaly

Normal or elevated blood counts
Effective hematopoiesis
No dysplasia
Splenomegaly


Слайд 28
WHO CLASSIFICATION OF MYELODYSPLASTIC/ MYELOPROLIFERATIVE DISEASE • Chronic Myelomonocytic Leukemia (CMML)•
Текст слайда:

WHO CLASSIFICATION OF MYELODYSPLASTIC/ MYELOPROLIFERATIVE DISEASE

• Chronic Myelomonocytic Leukemia (CMML)
• Atypical Chronic Myeloid Leukemia (aCML)
• Juvenile Myelomonocytic Leukemia (JMML)
• Myelodysplastic/Myeloprolilferative Disease,
Unclassifiable


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CHRONIC MYELOMONOCYTIC LEUKEMIA• Bridges MDS and MPD• Persistant monocytosis >1.0 X
Текст слайда:

CHRONIC MYELOMONOCYTIC LEUKEMIA


• Bridges MDS and MPD
• Persistant monocytosis >1.0 X 109/L
• No Ph1 chromosome or BCR/ABL
• <20% blasts
• Dysplasia or clonal chromosome abnormality or persistent monocytosis for >3 months old and other causes excluded
• Splenomegaly in 30 to 50% of cases


Слайд 30
ATYPICAL CHRONIC MYELOID LEUKEMIA (aCML) ↑WBC with mature and maturing granulocytes
Текст слайда:

ATYPICAL CHRONIC MYELOID LEUKEMIA (aCML)


↑WBC with mature and maturing granulocytes
Low percent of monocytes
Includes rare cases of BCR-ABL negative leukemia comprised of mature and maturing granulocytes
High leukocyte count MDS or chronic myeloproliferative syndrome with myelodysplastic features


Слайд 31
JUVENILE MYELOMONOCYTIC LEUKEMIA (JMML)• Synonymous with JCML• 60% of patients >2
Текст слайда:

JUVENILE MYELOMONOCYTIC LEUKEMIA (JMML)


• Synonymous with JCML
• 60% of patients >2 year old
• Ph chromosome and BCR-ABL negative
• Hepatosplenomegaly, lymphadenopathy
• Skin involvement - café-au-lait spots - NF
• Usually prominent monocytic component
• ↑ Leukocytes, ↓ platelets
• Elevated hemoglobin F
• Usually normal cytogenetics


Слайд 32
MONOSOMY 7 SYNDROME• Anemia and leukocytosis• Thrombocytopenia in 50%• Monocytosis• Defective
Текст слайда:

MONOSOMY 7 SYNDROME

• Anemia and leukocytosis
• Thrombocytopenia in 50%
• Monocytosis
• Defective neutrophil function with recurrent infections
• Hypercellular BM with ↑ reticulin
• Dysplasia
• Hepatosplenomegaly
• Neurofibromatosis


Слайд 33
OTHER MORPHOLOGIC FINDINGS IN MDS • Changes in marrow cellularityusually hypercellular• Increased iron stores• Myelofibrosis
Текст слайда:

OTHER MORPHOLOGIC FINDINGS IN MDS

• Changes in marrow cellularity
usually hypercellular
• Increased iron stores
• Myelofibrosis


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MDS/MPD, UNCLASSIFIABLE • Features of MDS but with thrombocytosis (>600 X
Текст слайда:

MDS/MPD, UNCLASSIFIABLE

• Features of MDS but with thrombocytosis (>600 X 109/L) or leukocytosis (>13.0 X 109/L)
• (and) No prior history of MDS or MPD
• (and) No cytogenetic abnormality associated with specific myeloid disorder
• (or) Mixed MDS and MPD features and cannot be assigned to any other category


Слайд 35
TreatmentSupportive care – blood products, infections etc.Growth factors – G-CSF, erythropoetinHypomethylating
Текст слайда:

Treatment

Supportive care – blood products, infections etc.
Growth factors – G-CSF, erythropoetin
Hypomethylating agents – azacitidin, dacogen
Revlimid – 5q-
Allogeneic stem cell transplantation


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Myeloid Clonal Hemopathies: Evolutions
Текст слайда:

Myeloid Clonal Hemopathies: Evolutions


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Survival Based on WPSS  Malcovati et al, ASH 05, #788L
Текст слайда:

Survival Based on WPSS Malcovati et al, ASH 05, #788

L et al. Blood. 2005;106:232a [abstract 788]


Слайд 43
WPSS for MDS: Clinical Outcomes Malcovati et al, ASH 12/05 #788a
Текст слайда:

WPSS for MDS: Clinical Outcomes Malcovati et al, ASH 12/05 #788a


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THANK  YOU
Текст слайда:





THANK YOU


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