Morphology Review- ISH Slides -

제 2 차 혈액학 종합학술대회 발표2000 년 5 월 25 일 -5 월 26 일

COEX

한림의대 조 현 찬

Clonal Bone Marrow Disorders

Metastatic Cancers

Lymphoma

FAB Classification Standard morphologic classification system Features considered

Morphology : Cell size, Amount of cytoplasmNuclear size and shape, Cytoplasmic membrane Cytoplasmic basophilia,

Cytoplasmic vacuolations, Other features Cytochemistry Immunophenotype cytogenetic & molecular genetic features Other features

M3

M5

M7

M6M1

M2

M0

M4

WHO Classification of Hematologic Malignancies

Myeloid Neoplasm Lymphoid Neoplasm Mast Cell Disorders Histiocytic Neoplasm

Diagnistic criteria Morphology Immunophenotype Genetic features Clinical syndromes

AMLs with recurrent cytogenetic translocations AML with t(8;21), AML1/ETO

Acute promyelocytic leukemia with t(15;17) AML with abnormal BM eosinophils with inv(16) AML with 11q23 (MLL) abnormalities AML with multilineage dysplasia AML & MDS, therapy-related AML not otherwise categorized AML minimally differentiated AML without maturation AML with maturation Acute myelomonocytic leukemia Acute monocytic leukemia Acute erythroid leukemia Acute megakaryocytic leukemia Acute basophilic leukemia Acute panmyelosis with myelofibrosis

AML, WHO Classification

(# 01) 69-year-old male Bone marrow film Poorly or minimally differentiated AML (AML-M0)

(#02) 5-year-old male, BM Acute myeloid leukemia (AML-M5a)

(#03) 67-year-old woman presented with pancytopeniaAcute megakaryoblastic leukemia

(#13) 9-year-old male with pancytopenia, BMMetastatic rhabdomyosarcoma

Morphology : The tumor cells vary in size; many have irregular cytoplasmic pseudopods. Occasional cells have cytoplasmic vacuoles.

Immunophenotype : CD41 (GpIIb/IIIa) (+) Tentative diagnosis : acute megakaryocytic

leukemia was established. Ultrastructural exam : Rhabdomyosarcoma

(#05) Newborn with Down syndromeTransient abnormal myelopoiesis(TAM) in Down syndrome

PB ; WBC 88 x 109/L, Hb 14.2 g/dl and platelets 12 x 109/l. Blast 50-60%

No phenotyping or chromosome studies. WBC count gradually returned to normal

over 10 weeks. There has been no recurrence and the child is well at nine years of age.

The blasts may express megakaryocytic and/or erythroid markers; promegakaryocytes and micromegakaryocytes may be numerous.

The CBC findings are not helpful in distinguishing the transient disorder from those processes which persist.

• Refractory anemia (RA) • RA with ring sideroblasts (RARS) • RA with excess of blasts (RAEB) • RAEB in transformation (RAEB/T) • CMML

Other Subtypes MDS with myelofibrosis Hypoplastic MDS Pure sideroblastic anemia

MDS, FAB Classification

MDS, WHO Classification Refractory anemia

with ringed sideroblastswithout ringed sideroblasts

Refractory cytopenia(MDS) with multilineage dysplasia

Refractory anemia with excess blasts 5q- syndrome MDS, unclassifiable

(#04) 37-year-old female house painter with a history of bruisingRAEB-T associated with an isolated 5q-

Congenital dyserythropoietic anemia, type III (#07) 27-year-old male, Hb 9.7 g/dl , BM

Chronic Myeloproliferative Disorders

Chronic myeloid leukemia

Atypical CMLJuvenile CMLChronic neutrophilic leukemia

Polycythemia vera Myeloid metaplasia with myelofibrosis Essential thrombocythemia

MPD, WHO Classification Chronic myelogenous leukemia, Ph(+) (t(9;22)

(q34;q11), BCR/ABL) Chronic neutrophilic leukemia Chronic eosinophilic leukemia/hypereosinophilic

syndrome Chronic idiopathic myelofibrosis Polycythemia vera Essential thrombocythemia Myeloproliferative disease, unclassifiable

Myelodysplastic/myeloproliferative disorders (MD/MPD) CMML, aCML, JMML

(#08) Ph(+) chronic myeloid leukemia

(#09) 45-year-old male PB film with Ph(+) CMLMixed lineage (B-lymphoid and myeloid) blast crisis of CML

Lymphoid neoplasm,WHO Classification

B-Cell neoplasm Precursor B-cell : ALL Mature(peripheral) B-cell : CLL, HCL, PCM, MCL , Burkitt’s Lymphoma

T-cell and NK-cell neoplasm Precursor T-cell Mature(peripheral) T-cell

Hodgkin’s lymphoma

(#10) 47-year-old male with an abdominal mass, PBRichter's transformation of B-cell CLL

(#11) 47-year-old male with easy bruisability and lethargy, BMPlasma cell leukemia

70/F, Sternal pain for 6 years (SNUH)CBC : 8.5 - 3.8 - 163kTotal protein/Albumin : 6.2/3.3, PEP : no M-spike

Plasma cell myeloma with heavy granules

Plasma cell leukemia, PB Multiple myeloma, BM

(#12) 66-year-old male, PB Mantle-cell lymphoma in leukemic phase

Morphology : medium size lymphoid population having a nucleus with dense but not clumped chromatin, occasional nuclear clefts and a small nucleolus.

Immunophenotype : clonal B-cell population with strong expression of SIg and membrane CD22(+), CD5(+), CD23(-)

Cytogenetic analysis : t(11:14)(q13;q32) Spleen histology : diffuse involvement by

medium size lymphocytes with a cleaved nucleus

Hairy cell leukemia

Middle-aged patients over 50 Weakness and lethargy Splenomegaly (80% )

Hairy cells : Fine & irregular pseudopods, immature nuclear features

BM : often unsuccessful

(#14) 52-year-old male, PB Large-cell lymphoma evolving into leukemia

Morphology : Large (> 3 times of RBC), reticular chromatin, deeply basophilic

cytoplasm and 1-3 nucleoli.

Immunophenotype : Clonal B-cell population kappa+, lambda-, CD19+, FMC7+, and negative with CD5, CD23 & CD2.

(#15) 28-year-old male, BMALL-L3, Burkitt’s lymphoma/Burkitt cell leukemia

(#16) 38-year-old black Caribbean Peripheral blood Adult T-cell leukemia lymphoma (ATLL).

Morphology : small to medium size lymphocytes with a highly irregular nucleus with inconspicuous nucleolus and multiple indentation

Immunophenotype : mature T-cell (TdT+, CD1a-, CD2+, CD3+, CD5+, CD7-) with a CD4+, CD8-, CD25+ phenotype.

Antibodies to the human T-cell leukemia/lymphoma virus (HTLV-1)

Molecular analysis : a clonal integration of the proviral HTLV-1 in the cell's DNA

(#17) 2-year-old boy with mediastinal mass

Increasedhematogones

50-55% lymphoid cells with a very high N/C ratio; condensed chromatin, indistinct nucleoli, Some cleft nuclei

CBC : Within normal limits Cell markers : non-B, non-T phenotype Mediastinal mass : plasma cell granuloma. Uneventful recovery from surgery. No follow-up BM study