Malignant hematopathology (detailed) 12/20

Question 1

Precursor B and T cell neoplasms

Answer 1

ALL: most common is B cell, more common younger than 15, peak at 4 y/o and M>F

T cell types are associated with a mediastinal mass, teenagers

Pre-B=TdT+,CD19+,CD10+

Pre-T=CD1,2,5,7+, CD3,4,8-

Peripheral T=CD3,4,8+

Question 2

ALL

Answer 2

majority have numerical or structural changes

abrupt or stormy onset

bone pain or tenderness

CNS manifestation

generalized lymphadenopathy, hepatosplenomegaly

Question 3

Worse prognosis for ALL

Answer 3

less than 2 y/o or older age

blasts>100,000

unfavorable genetics

Ph chromosome (9,22)

Question 4

Peripheral B cell neoplasms

Answer 4

CLL or SLL

CD19,20+ but also CD5,23+(even though these are T-cell markers)

mostly older pts>50

often asymptomatic

prolymphocytic transformation and Richter’s syndrome

Question 5

Follicular lymphoma

Answer 5

most common NHL in US

middle age, M=F

CD19,20,10,SIg,BCl6+

t(14,18)–>18=BCl2

indolent course and incurable

can transform into DLBCL

Question 6

DLBCL

Answer 6

heterogenous group of tumors

20% of NHL,5% of childhood lymphoma,60-70% of aggressive lymphomas

old male pts

CD19,20+, variable CD10,BCL6 and SIg

c-myc mutations, some show t(14,18)

immunodeficiency-associated and body cavity large cell lymphomas

Can present as enlarging mass with poor prognosis

Question 7

DLBCL in liver

Answer 7

is characterized by fish-flesh appearance

Question 8

Burkitt lymphoma

Answer 8

t(8,14), c-myc translocation

CD19,20,10,BCL6+

endemic seen in mandible and abdominal viscera, sporadic seen as abdominal mass of ileocecum and peritoneum

responds to therapy and children have better prognosis

Starry sky appearance

Question 9

Mantle Cell lymphoma

Answer 9

t(11,14), 11=cyclin D1

M>F, 5th-6th decades

CD19,20,5+ but CD23-

painless lymphadenopathy or splenomegaly and can involve GI

poor prognosis

transformation uncommon

Question 10

Marginal zone lymphoma

Answer 10

MALToma

often in tissues with autoimmune disease=H pylori gastritis, hashimoto thyroiditis

remains localized for prolonged periods and may regress with eradication of inciting agent

Question 11

anaplastic large cell lymphoma (peripheral T cell lymphoma)

Answer 11

rearrangement of ALK gene on chromosome 2P23

children and young adults

frequently involve soft tissues

good prognosis and curable

older adults lack ALK and have poor prognosis

Slide can be stained with ALK stain

Question 12

peripheral T and NK cell lymphoma

Answer 12

lack TdT but CD2,5,3,4,8+

generalized lymphadenopathy, eosinophilia,fever, pruritis and weight loss

aggressive and has poor prognosis

See cerebriform morphology of cells on slides

Question 13

mycosis fungoides/sezary syndrome

Answer 13

affect CD4+

predilection for skin

generalized exfoliative erythema of skin

leukemia with sezary cells

transformation to large cell is a terminal event

Question 14

classification of HL

Answer 14

Nodular sclerosis

mixed cellularity

lymphocyte rich

lymphocyte depletion

lymphocyte predominance

Question 15

ANN ARBOR Staging of HL

Answer 15

Stage I=involvement of a single node group or extranodal site

StageII=involvement of 2 or more node groups on the same side of the diaphragm

StageIII=involvement of nodes on both sides of diaphragm and may include spleen

StageIV=multiple foci of involvement of extra lymphatic organs

A=no systemic symptoms

B=systemic symptoms, fever, night sweats, weight loss over 10% body weight

Question 16

RS cells stain

Answer 16

CD15,30+ but CD45- and other T cell markers

Question 17

mixed cellularity type of HL

Answer 17

strongly associated with EBV

more systemic effects and advanced stage than nodular sclerosis but good prognosis

Question 18

lymphocyte depleted type of HL

Answer 18

DDx is large cell lymphoma and therefore phenotyping is important

older pts, HIV+ and EBV associated

less favorable prognosis

Question 19

lymphocyte rich type of HL

Answer 19

EBV in 40% of cases

CD15,30+ but CD45,20-

good to excellent prognosis

Question 20

lymphocyte predominant type of HL

Answer 20

popcorn cells

CD20+ and BCL6+

males,young age, cervical or axillary adenopathy

excellent prognosis

Question 21

RS cells

Answer 21

Question 22

Differences between HL and NHL

Answer 22

HL=orderly spread, Waldeyer’s ring rare, rarely extra nodal, stage is most important

NHL=non-contiguous, Waldeyer’s ring common, commonly extra nodal, type is most important

Question 23

Myeloproliferative disease

Answer 23

RBC–>polycythemia vera

WBC–>CML

Megakaryocytes–>essential thrombocytosis (>600,000), primary myelofibrosis

Question 24

CML etiology

Answer 24

25-60 y/o

translocation involving BCR gene on chromosome 9 and ABL gene on chromosome22 (ph chromosome)

fusion protein tyrosine kinase–>cell division and inhibition of apoptosis

Question 25

CML clinical

Answer 25

multiple myeloid lineages, B lymphoid and even T cells express the fusion protein pluripotent stem cell is target insidious onset, mild anemia, fatigue, easy bruising and splenomegaly blast crisis:70% myeloblasts and remainder lymphoblasts

Question 26

primary meylofibrosis

Answer 26

rapid development of marrow fibrosis extensive extramedullary hematopoiesis extensive collagen deposition by non-neoplastic fibroblasts \>60 y/o, progressive anemia and splenomegaly See teardrop cells on blood smear

Question 27

myelodysplastic syndrome

Answer 27

clonal stem cell disorder maturation defects and ineffective hematopoiesis increased risk of transformation to AML ideopathic or primary may be therapy related

Question 28

MDS etiology

Answer 28

accelerated apoptosis ineffective hematopoiesis hypercellular marrow peripheral cytopenia

Question 29

MDS classification: FAB

Answer 29

refractory anemia refractory anemia with ringed sideroblasts refractory anemia with excess blasts chronic myelomonocytic leukemia

Question 30

MDS final stage

Answer 30

is AML see Auer rods

Question 31

acute Leukemia epi

Answer 31

leading cause of cancer death in kids under 15 and 7th most common form of cancer death overall

Question 32

Morphologic features of ALL

Answer 32

L1=small cells predominate but may vary, with some cells up to twice diameter of small lymphocytes/ nuclei are generally round and regular with occasional clefts/ nucleoli absent, cytoplasm scanty and cell population is homogeneous L2=cells are heterogeneous in size/ nuclei show clefts and nucleoli are present L3=population of medium sized cells/ nuclei round to oval with multiple basophilic nucleoli/ cytoplasm abundant and deeply basophilic with cytoplasmic lipid vacuoles

Question 33

karyotypic changes in ALL

Answer 33

in general, hyperdiploidy has a good prognosis where as all translocations are associated with poor prognosis

Question 34

revised classification of AML

Answer 34

**M0(minimally differentiated AML)**=blasts lack definitive markers of myeloblasts (MPO-) but express myeloid lineage Ag and resemble myeloblasts ultrastructurally **M1(AML without differentiation)=**very immature but at least\>3% are MPO+,few granules or auer rods and little maturation beyond the myeloblast stage **M2(AML with maturation)=**full range of maturation through granulocytes, Auer rods present mostly.presence of t(8,21) defines a prognostically favorable subgroup **M3(acute promyelocytic leukemia)**=hypergranular promyelocytes with many auer rods per cell, younger pts, DIC, t(15,17) **M4(acute myelomonocytic leukemia)=**myeloid element resembles M2, monocytic cells positive for nonspecific esterase, presence of chromosome 16 abnormalities defines a subset with eosinophils in marrow and excellent prognosis **M5(acute monocytic leukemia)=**monoblasts with MPO- and esterase+, promonocytes predominate,older pts,gingival hyperplasia **M6(acute erythroleukemia)=**abnormal erythroblasts,advanced age **M7(acute megakaryocytic leukemia)=**megakaryoblasts predominate,react with platelet-specific Ab against GPIIb/IIIa or vWF,myelofibrosis in most cases