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

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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

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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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