形態学的異形成に基づく診断確度区分と 形態診断アトラス · 2018-01-10 ·...
TRANSCRIPT
不応性貧血(骨髄異形成症候群)の形態学的異形成に基づく診断確度区分と
形形態態診診断断アアトトララスス
不応性貧血(骨髄異形成症候群)の形態学的異形成に基づく診断確度区分と
形形態態診診断断アアトトララスス
編集 朝朝長長 万万左左男男(長崎大学原爆後障害医療研究施設) 松松田田 晃晃 (埼玉医科大学国際医療センター)
不応性貧血 骨髄異形成症候群 の形態学的診断基準作成のためのワーキンググループ
厚厚生生労労働働科科学学研研究究費費補補助助金金難難治治性性疾疾患患克克服服研研究究事事業業 特特発発性性造造血血障障害害にに関関すするる調調査査研研究究((平平成成1199年年度度))
主主任任研研究究者者 小小澤澤 敬敬也也
1
2
myelodysplastic syndromes,
MDS MDS
MDS
MDS
anemia of chronic disorders ACD
aplastic anemia, AA 8
MDS 2005 Mufti
idiopathic cytopenias of uncertain
significance, ICUS MDS
2006
MDS A
ACD
MDS MDS
B
MDS International
Working Group on MDS Morphology, IWG-MDS 2005
IWG-MDS
IWG-MDS
IWG-MDS IWG-MDS
A B 10
World Health Organization WHO
MDS 10%
WHO
Valent MDS minimal diagnostic criteria ICUS
criteria WHO MDS
10% 15%
3
A B
A MDS
10% A MDS
10% B
MDS
criteria
8 MDS MDS
I I
II III A
IV A B
V Grade of dysplasia
VI Division of cytogenetic findings
VII Grade of dysplasia Division of cytogenetic findings
Grade of diagnostic accuracy MDS ‘Definite’ ‘Probable’
‘Possible’ 3 ‘ICUS’ WHO
VIII I MDS
‘Possible’/‘ICUS’
‘ICUS’
ICUS ICUS MDS
French-American-British FAB5
refractory anemia,
RA MDS
6
AA
paroxysmal nocturnal hemoglobinuria, PNH
AA MDS
7
AA MDS
AA
A B 10
AA MDS
AA MDS
MDS (‘Definite’ ‘Probable’
4
‘Possible’)
PNH
818
94-2
5
I MDS
Valent MDS minimal criteria prerequisite criteria4)
A E Table 1
A. 1
1 3 3
6 ICUS
B. 20 WHO acute myeloid leukemia with
recurrent cytogenetic abnormalities
500
IWG-MDS agranular blasts FAB type 1 blasts granular blasts
FAB type 2 blasts Goasguen type 3 blasts10)
refractory anemia with excess blasts, RAEB acute
myeloid leukemia, AML 10
MDS RAEB-2 AML t (8;21) WHO acute myeloid
leukemia with recurrent cytogenetic abnormalities
20 WHO
AML 11q23 abnormalities
VI
C. 1 109/L
chronic myelomonocytic leukemia, CMML
FAB WHO
IWG-MDS
D.
MDS
MDS
ACD
6
E.
AA MDS
1.5cm Jamshidi
60 30% 60 20%
11, 12)
MRI AA
MDS
II
A B Table 2
A
MDS 4
hypo-segmented mature neutrophils pseudo Pelger-Huet anomaly, Pelger
2 fine thin
degranulation of neutrophils a- or hypogranular neutrophils, Hypo-Gr
80%
micromegakaryocytes mMgk
2
ringed sideroblasts RS
1/3 5
IWG-MDS RS
B
MDS ACD A
10 MDS PAS
PAS WHO
A B
7
III A
ringed sideroblasts
hypo-segmented mature neutrophils pseudo Pelger-Huet anomaly, Pelger
100
degranulation of neutrophils a- or hypogranular neutrophils, Hypo-Gr
100
Hypo-Gr
Hypo-Gr
micromegakaryocytes mMgk
25 25
25
mMgk 3 25 10
mMgk 10
ringed sideroblasts RS
100 RS
IV A B
100 100 25 A
B 25
25
3 25 10
10 100
RS 5 100
B 10 15 5
10
8
V
Grade of dysplasia ‘High’
‘Intermediate’ ‘Low’ ‘Minimal’ Table 3
High 1 2
1. A 10% Pelger 10% Hypo-Gr 10%
10% mMgk 10%
2. A 15% RS 15%
RS 15% ‘High’ MDS
Intermediate
A B 2 3 10
Low
A B 1 10
Minimal:
A B 1 3 1 9
VI
Division of cytogenetic findings ‘Abnormal’ ‘Normal’
‘Unknown’
Abnormal
MDS
MDS Valent MDS minimal criteria
MDS-related decisive criteria4)
Haase13)
5q- -7/7q-
+8 20q- complex others t(8;21)(q22;q22) t(15;17)(q22;q12)
inv(16)(p13;q22) t(16;16)(p13;q22) WHO AML with recurrent
cytogenetic abnormalities
WHO t(8;21)(q22;q22) t(15;17)(q22;q12) inv(16)(p13;q22)
t(16;16)(p13;q22) 20% AML
11q23 abnormalities
9
Normal
Unknown
VII
Grade of dysplasia Division of cytogenetic
findings MDS diagnostic accuracy ‘Definite’ ‘Probable’ ‘Possible’
3 ‘ICUS’ Table 4
MDS Definite
3
(1) 5 19%
‘High’ ‘Intermediate’ ‘Low’
‘Abnormal’ ‘Normal’ ‘Unknown’
(2) 0 4
‘High’ ‘Intermediate’ ‘Low’
‘Abnormal’
(3) Grade of dysplasia: ‘High’
0 19% ‘Abnormal’ ‘Normal’
‘Unknown’
MDS Probable
0 4 ‘Intermediate’
‘Normal’ ‘Unknown’
MDS Possible
0 4% ‘Low’ ‘Normal’
‘Unknown’ AA
MDS
ICUS
Valent ICUS criteria4)
10
6 1 3
0 4 ‘Normal’ ‘Unknown’
‘Minimal’
‘ICUS’ 6 MDS
MDS ‘Possible’ 6
VIII WHO
WHO ‘Possible’
6
11
AA MDS
2 Jamshidi
MRI
1.5cm MRI
MRI
MRI
MRI
/ 60 30% 60
20% 60 30 59% 60 20 59%
60%
AA:
A B 10
A B 10
A B
5%
MDS
12
MDS:
MDS ‘Definite’ ‘Probable’
‘Possible’
10 A B
MDS
‘Possible’
13
Table 1.
����
�A.�������1��������� �:
� ������� <11 g/dL
� ���� <1,500/�L
���� <100,000/�L
�B.���������� 20���
� ��
� Acute myeloid leukemia with recurrent cytogenetic abnormalities��������� ��
�C.������� 9/L��
�D.�� �����������������������
�E. ����������
������������� ��������������������
�A-E����
* t(8;21)(q22;q22); (AML1/ETO), t(15;17)(q22;q12); (PML/RAR�),
inv(16)(p13;q22) ��� t(16;16)(p13;q22); (CBF�/MYH11) ���
�� �11q23 (MLL) abnormalities����!���
Table 2.
����� A
�Granulocytic series
hypo-segmented mature neutrophils (Pelger)
degranulation (a- or hypogranular neutrophils: Hypo-Gr)
�Megakaryocytic series
micromegakaryocytes (mMgk)
�Erythroid series
ringed sideroblasts (RS)
����� B
�Granulocytic series
small size
hypersegmentation
pseudo Chediak-Higashi granule
�Megakaryocytic series
non-lobulated nuclei
multiple, widely-separated nuclei
�Erythroid series
nucleus budding
internuclear bridging
karyorrhexis
multinuclearity
megaloblastoid change
cytoplasm vacuolization
14
Table 3.
High
High 1 2
1. Pelger 10% Hypo-Gr 10% mMgk 10%
2. RS 15%
Intermediate
2 3 A B 10%
Low
1 A B 10%
Minimal
1 3 A B = 1 9%
Pelger : hypo-segmented mature neutrophils
Hypo-Gr :degranulation (a- or hypogranular neutrophils)
mMgk : micromegakaryocytes RS: ringed sideroblasts
Table 4.
(%)
MDS Definite 5 19 High, INT, Low Any
0 4 High, INT, Low Abnormal
0 4 High Any
MDS Probable 0 4 INT Normal or Unknown
MDS Possible 0 4 Low Normal or Unknown
ICUS 0 4 Minimal or None Normal or Unknown
INT Intermediate ICUS idiopathic cytopenia of uncertain significance
15
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