Malignant hematopathology (detailed) 12/20 Flashcards

1
Q

Precursor B and T cell neoplasms

A

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+

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2
Q

ALL

A

majority have numerical or structural changes

abrupt or stormy onset

bone pain or tenderness

CNS manifestation

generalized lymphadenopathy, hepatosplenomegaly

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3
Q

Worse prognosis for ALL

A

less than 2 y/o or older age

blasts>100,000

unfavorable genetics

Ph chromosome (9,22)

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4
Q

Peripheral B cell neoplasms

A

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

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5
Q

Follicular lymphoma

A

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

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6
Q

DLBCL

A

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

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7
Q

DLBCL in liver

A

is characterized by fish-flesh appearance

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8
Q

Burkitt lymphoma

A

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

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9
Q

Mantle Cell lymphoma

A

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

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10
Q

Marginal zone lymphoma

A

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

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11
Q

anaplastic large cell lymphoma (peripheral T cell lymphoma)

A

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

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12
Q

peripheral T and NK cell lymphoma

A

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

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13
Q

mycosis fungoides/sezary syndrome

A

affect CD4+

predilection for skin

generalized exfoliative erythema of skin

leukemia with sezary cells

transformation to large cell is a terminal event

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14
Q

classification of HL

A

Nodular sclerosis

mixed cellularity

lymphocyte rich

lymphocyte depletion

lymphocyte predominance

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15
Q

ANN ARBOR Staging of HL

A

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

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16
Q

RS cells stain

A

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

17
Q

mixed cellularity type of HL

A

strongly associated with EBV

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

18
Q

lymphocyte depleted type of HL

A

DDx is large cell lymphoma and therefore phenotyping is important

older pts, HIV+ and EBV associated

less favorable prognosis

19
Q

lymphocyte rich type of HL

A

EBV in 40% of cases

CD15,30+ but CD45,20-

good to excellent prognosis

20
Q

lymphocyte predominant type of HL

A

popcorn cells

CD20+ and BCL6+

males,young age, cervical or axillary adenopathy

excellent prognosis

21
Q

RS cells

A
22
Q

Differences between HL and NHL

A

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

23
Q

Myeloproliferative disease

A

RBC–>polycythemia vera

WBC–>CML

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

24
Q

CML etiology

A

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

25
Q

CML clinical

A

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

26
Q

primary meylofibrosis

A

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

27
Q

myelodysplastic syndrome

A

clonal stem cell disorder

maturation defects and ineffective hematopoiesis

increased risk of transformation to AML

ideopathic or primary

may be therapy related

28
Q

MDS etiology

A

accelerated apoptosis

ineffective hematopoiesis

hypercellular marrow

peripheral cytopenia

29
Q

MDS classification: FAB

A

refractory anemia

refractory anemia with ringed sideroblasts

refractory anemia with excess blasts

chronic myelomonocytic leukemia

30
Q

MDS final stage

A

is AML

see Auer rods

31
Q

acute Leukemia epi

A

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

32
Q

Morphologic features of ALL

A

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

33
Q

karyotypic changes in ALL

A

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

34
Q

revised classification of AML

A

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