Erkrankungsklassifikationen

Erstellt von: Infocenter (Projekt 2) , am: 27.11.2006, letzte Änderung: 05.09.2011

FAB-Klassifikation, WHO-Klassifikation

Inhaltsübersicht

WHO-Klassifikation: Myeloproliferative neoplams
WHO-Klassifikation: Myeloplastic/myeloproliferative neoplasms
WHO-Klassifikation: Myelodysplastic syndroms
WHO-Klassifikation: Acute myeloid leukaemia (AML) and related precursor neoplasms
WHO-Klassifikation: Precursor lymphoid neoplasms
FAB-Klassifikation der AML

Zusammengestellt vom Informationszentrum, Stand: 9/2010

WHO-Klassifikation: Myeloproliferative neoplams


MYELOPROLIFERATIVE NEOPLASMS
Chronic myelogenous leukaemia, BCR-ABL1 positive	9875/3
Chronic neutrophilic leukaemia	9963/3
Polycythaemia vera	9950/3
Primary myelofibrosis	9961/3
Essential thrombocythaemia	9962/3
Chronic eosinophilic leukaemia, NOS	9964/3
Mastocytosis
- Cutaneous mastocytosis	9740/1
- Systemic mastocytosis	9741/3
- Mast cell leukaemia	9742/3
- Mast cell sarcoma	9740/3
- Extracutaneous mastocytoma	9740/1
Myeloproliferative neoplasm, unclassifiable	9975/3

WHO-Klassifikation: Myeloplastic/myeloproliferative neoplasms


MYELODYSPLASTIC/MYELOPROLIFERATIVE NEOPLASMS
Chronic myelomonocytic leukaemia	9945/3
Atypical chronic myeloid leukaemia, BCR-ABL1 negative	9876/3
Juvenile myelomonocytic leukaemia	9946/3
Myelodysplastic/myeloproliferative neoplasm, unclassifiable	9975/3
Refractory anaemia with ring sideroblasts associated with marked thrombocytosis*	9982/3*

* provisorisch histologische Subgruppe

WHO-Klassifikation: Myelodysplastic syndroms


MYELODYSPLASTIC SYNDROMES
Refractory cytopenia with unilineage dysplasia
- Refractory anaemia	9980/3
- Refractory neutropenia	9991/3
- Refractory thrombocytopenia	9992/3
Refractory anaemia with ring sideroblasts	9982/3
Refractory cytopenia with multilineage dysplasia	9985/3
Refractory anaemia with excess blasts	9983/3
Myelodysplastic syndrome associated with isolated del(5q)	9986/3
Myelodysplastic syndrome, unclassifiable	9989/3
Childhood myelodysplastic syndrome
- Refractory cytopenia of childhood*	9985/3*

* provisorisch histologische Subgruppe

WHO-Klassifikation: Acute myeloid leukaemia (AML) and related precursor neoplasms


AML with recurrent genetic abnormalities
AML with t(8;21)(q22;q22); RUNX1-RUNX1T1	9896/3
AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11	9871/3
Acute promyelocytic leukaemia with t(15;17)(q22;q12); PML-RARA	9866/3
AML with t(9;11)(p22;q23); MLLT3-MLL	9897/3
AML with t(6;9)(p23;q34); DEK-NUP214	9865/3
AML with inv(3)(q21q26.2) or t(3;3)(q21 ;q26.2); RPN1-EVI1	9869/3
AML (megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1	9911/3
AML with mutated NPM1*	9861/3*
AML with mutated CEBPA*	9861/3*

* provisorisch histologische Subgruppe


AML with myelodysplasia-related changes	9895/3


Therapy-related myeloid neoplasms	9920/3


Acute myeloid leukaemia, NOS	9861/3
AML with minimal differentiation	9872/3
AML without maturation	9873/3
AML with maturation	9874/3
Acute myelomonocytic leukaemia	9867/3
Acute monoblastic and monocytic leukaemia	9891/3
Acute erythroid leukaemia multilineage dysplasia	9840/3
Acute megakaryoblastic leukaemia	9910/3
Acute basophilic leukaemia	9870/3
Acute panmyelosis with myelofibrosis	9931/3


Myeloid sarcoma	9930/3


Myeloid proliferations related to Down syndrome
Transient abnormal myelopoiesis	9898/1
Myeloid leukaemia associated with Down syndrome	9898/3


Blastic plasmacytoid dendritic cell neoplasm	9727/3

WHO-Klassifikation: Precursor lymphoid neoplasms


B lymphoblastic leukaemia/lymphoma
B lymphoblastic leukaemia/lymphoma, NOS	9811/3
B lymphoblastic leukaemia/lymphoma with recurrent genetic abnormalities
- B lymphoblastic leukaemia/lymphoma with t(9;22)(q34;q11.2); BCR-ABL1	9812/3
- B lymphoblastic leukaemia/lymphoma with t(v;11q23); MLL rearranged	9813/3
- B lymphoblastic leukaemia/lymphoma with t(12;21)(p13;q22); TEL-AML1 (ETV6-RUNX1)	9814/3
- B lymphoblastic leukaemia/lymphoma with hyperdiploidy	9815/3
- B lymphoblastic leukaemia/lymphoma with hypodiploidy (hypodiploid ALL)	9816/3
- B lymphoblastic leukaemia/lymphoma with t(5;14)(q31;q32); IL3-IGH	9817/3
- B lymphoblastic leukaemia/lymphoma with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1)	9818/3


T lymphoblastic leukaemia/lymphoma	9837/3

Quelle: Swerdlow S.H., Campo E. Harris N.L., Jaffe E.S., Pileri S.A., Stein H., Thiele J., Vardiman J.W. (Eds.):
WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues,
IARC: Lyon 2008

FAB-Klassifikation der AML

FAB	Subtyp	Morphologie	Auer- stäbe	MPO	UE	Zyto- genetik*	Häufigkeit
M 0	AML ohne Ausreifung	Myeloblasten ohne Granula	-	-**	-		< 5%
M 1	AML mit minimaler Ausreifung	Myeloblasten +/- Granula	+/-	+	-	t(9;22)	15 - 20%
M 2	AML mit Ausreifung	Myeloblasten mit Granula, einzelne Myelozyten	+	+	-	t(8;21)	25 - 30%
M 3	Akute Promyelozytenleukämie	Promyelozyten, deutlich granuliert	++	+	-	t(15;17)	5 - 10%
M 4	Akute myelomonozytäre Leukämie	Myeloblasten und Promyelozyten > 20%	+/-	+	+	Inv/del(16) bei M4eo	20 - 30 %
M 5a	Akute Monoblastenleukämie ohne Ausreifung	Große Monoblasten	-	-	+	t/del(11)	5%
M 5b	Akute Monoblastenleukämie mit Ausreifung	Monoblasten, Promonozyten und Monozyten; Monozytose im peripheren Blut	-	-	+	t(8;16)	5 - 10%
M 6	Akute Erythroleukämie	Megaloblastäre Erythropoese > 50%, Myeloblasten > 30%	+	+	+/-		5%
M 7	Akute Megakaryoblasten-leukämie	Megakaryoblasten	-	-	+/-		5%
nur häufigste Abberationen *nur elektronenoptisch bzw. immunologisch nachweisbar +nachweisbar, ++stark nachweisbar, -nicht nachweisbar, +/-z.T. nachweisbar (modifiziert nach Bennett, 1985

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