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hema final > Hema > Flashcards

Hema Flashcards

1
Q

hairy cell leukemia

A

Hairy cell leukemia is- small B lymphocytes w/ abundant cytoplasm and fine (“hairy”) cytoplasmic projections.

origin- peripheral B cell of post–germinal center stage (memory B cell) fibers.

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2
Q

Neoplastic cells display an oval or indented nucleus, abundant cytoplasm, and fine, hairlike cytoplasmic projections

A

hairy cell

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3
Q

most specific markers for classic hairy cell leukemia

A

CD123 and annexin A1

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4
Q

Morphology of Hairy Cell Leukemia

A
  • found in bone marrow and the red pulp of the spleen
    -Low number of neoplastic B cells in peripheral blood.
    -lymph node involvement=rare
    -bone marrow infiltrates are interstitial=composed of small-med size lymphoid cells w/abundant cytoplasm
    biopsy of bone marrow=increased reticulin
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5
Q

Clinical Features of hairy cell

A

Rare lymphoproliferative disorder of middle-aged individuals (median age, 55 years).
Presenting signs include splenomegaly and pancytopenia.

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6
Q

Prognosis of hairy cell

A

Extreme tiredness, frequent infections
Weakness and unexplained weight loss

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7
Q

Immunophenotyping of hairy cell cases show

A

strong positivity for B cell markers (CD19, CD20, CD22), coupled with bright expression of CD11c, CD25, CD103, tartrate-resistant acid phosphatase (TRAP), DBA-44, CD123, and annexin A1

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8
Q

Burkitt Lymphoma

A

characterized by medium-sized, highly proliferating lymphoid cells with basophilic vacuolated cytoplasm

involves the central nervous system, bone marrow, and peripheral blood (Burkitt leukemia

EBV- present in a proportion of patients

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9
Q

Burkitt Lymphoma-WHO classification

A

endemic (occurring predominantly in Africa)- 4-7 yrs most commonly as jaw mass

Sporadic–young adults and children

immunodeficiency associated—predominantly in HIV + patients—- prognosis=not favorable

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10
Q

Burkitt Lymphoma- Morphology

A

“starry sky” pattern imparted by numerous tingiblebody macrophages

Lymphoma cells are medium size with round nuclei, finely distributed chromatin, and small nucleoli. The cytoplasm is deeply basophilic and highly vacuolated

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11
Q

what are the macrophages in Burkitt lymphoma responsible for

A

for phagocytosing apoptotic debris, a by-product of the extremely high proliferative activity.

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12
Q

what is hallmark of Burkitt lymphoma

A

high proliferation rate. 100% of Burkitt lymphoma cells are actively proliferating

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13
Q

Burkitt lymphoma–immunophentype

A

CD19, CD20, CD10, and BCL6 antigens are present
There is surface expression of monoclonal immunoglobulin light chains. BCL2 is absent

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14
Q

Burkitt lymphoma translocation

A

pathognomonic, MYC gene expression
translocation under the promoter of immunoglobulin heavy or light chain genes [t(8;14), t(2;8), or t(8;22)

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15
Q

CLL presents

A

mostly in peripheral blood and bone marrow

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16
Q

SLL primarily involves

A

lymph nodes and other lymphoid organs

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17
Q

CLL/SLL generally affects

A

older adults, however approximately 24% of patients are less than 55 years old.

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18
Q

Chronic Lymphocytic Leukemia (CLL)

A

Characterized by accumulation of small lymphoid cells in peripheral blood, bone marrow, & lymphoid organs.

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19
Q

Morphology of CLL

A

small lymphoid cells with a characteristically coarse chromatin (“soccer-ball” pattern)
smudge cells
Absent/inconspicuous nucleoli & scant cytoplasm in 55%
lymphoid cells with cleaved nuclei, or large lymphoid cells with an atypical appearance

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20
Q

CLL immunophenotype

A

CD19, CD20, and CD23, with aberrant expression of CD5.

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21
Q

CLL Diagnosis

A

CLL is diagnosed based on a sustained increase in the Monoclonal B lymphocytes with CLL immunophenotype which is =/> 5000/uL.

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22
Q

CLL Clinical Features

A

CLL/SLL generally affects older adults
First indication of disease is often an incidental finding of lymphocytosis on a routine CBC ordered for a different reason
heterogenous disease

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23
Q

Prognosis of CLL

A

patients w/ mutated IGVH= survive 24 yrs
unmutated IGVH is more aggressive and survive up to 8 yrs

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24
Q

the most frequent leukemia in adults

A

CLL

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25
Q

CLL Chromosomal abnormalities

A

del13q14.3, del11q22-23, trisomy 12, and del17p13

26
Q

5% of patients with CLL/SLL develop

A

a high-grade diffuse large B cell lymphoma (called Richter syndrome) with a survival of less than 1 year

27
Q

Hodgkin lymphoma

A

-can often be cured
-increase of Hodgkin lymphoma in EBV & HIV

28
Q

Hodgkin lymphoma can be divided into

A

Nodular lymphocyte-predominant Hodgkin lymphoma and Classical Hodgkin Lymphoma.

29
Q

Classical Hodgkin lymphoma comprises of

A

a heterogeneous group of lymphoid neoplasms derived from the germinal center

30
Q

Age Group of ALL

A

less than 13 years but can be present in any age group

31
Q

what is the Immunophenotype of Hodgkins

A

CD30+ in all cases
and CD15+ in approximately 80% of cases
Expression of B cell marker CD20 is weak to absent

32
Q

Age Group of AML

A

13-40 years but can be present at any age group

33
Q

Age Group of CML

A

40-60 years

34
Q

Age Group of CLL/SLL

A

less than 60 years

35
Q

Age Group of MDS

A

older the 65

36
Q

Age Group of Hodgkin

A

15-34 and older than 54

37
Q

Age Group of Burkitt

A

Endemic 4-7 yr in jawbone mass, sporadic variant- children & young adults most commonly as abdominal mass.

38
Q
A
39
Q

Age Group of Hairy cell leukemia

A

middle age around55

40
Q

Significance of Auer Rods

A

Auer rods are cytoplasmic inclusions that result from an abnormal fusion of the primary (azurophilic) granules. Their identification is very important because if found they confirm the presence of myeloblasts indicating the presence of a non-lymphocytic (myeloid) leukemia. They can also be seen in myeloid blast crisis in chronic granulocytic leukemia. Auer rods are never seen in lymphoblasts. This differentiation is important because the treatment of lymphoblastic and myeloblastic are different.

41
Q

DIC seen in?

A

APL(M3) T(15;17)(q22;q12) Acute promyelocytic leukemia
AML(M5a)(t(9;11)(p22pq23q11)) Acute monocytic leukemia

42
Q

Auer rods seen in?

A

AML M1(t 8,5 7) w/o maturation (up to50%)
AML M2 t(8;21)(q22q22)runx1/runx1q
APL M3 t(15:17)(q22:q12)PML(rara) in bundles

43
Q

Down syndrome seen in

A

Acute megakaryocytic leukemia (M7) Inv 3(q21q26) The mutation is in GATA1

44
Q

FAB T(8;21)(q22;q22) runx1/runx1 interchangeable with WHO?

A

AML w/Maturation (M2)

45
Q

FAB inv(16)(p13.1q22) or t(16:16)(p13.1:q22); CBF BMYH11 interchangeable with WHO?

A

M4: Acute myelomonocytic leukemia (AMML)

46
Q

FAB T(9:11)(q22:q22):MLLT3-MLL interchangeable with WHO?

A

Acute monocytic leukemia poorly differentiated (M5a)

46
Q

FAB T(15:17)(q22:q22)PML RARA interchangeable with WHO?

A

Acute promyelocytic leukemia(APL) (M3)

47
Q

infiltration to tissues seen in?

A

AMML M4 acute myelomonocytic leukemia tissue infiltration
AMML M4eo with eosinophilia tissue infiltration

M5b acute monocytic leukemia well-differentiated skin and gum infiltration

48
Q

FAB Critera’s

A

-Morphology, cytochemistry. immunophenotyping
MPD, MDS, Acute leukemias
-More than or equal to 30% blasts. Based on the morphologic examination along with cytochemical stains to distinguish lymphoblasts for myeloblasts
-1st system is still used by some but is being replaced by Who

48
Q

Who criteria’s

A

-Morphology cytochemistry, immunophenotyping, Cytogenetics, Clinical Features
-Acute Myeloid Leukemia, Acute leukemias of ambiguous, B-lymphoblastic leukemia/lymphoma and T-lymphoblastic leukemia/lymphoma
-more than or equal to 20% blast in the bone marrow is required for diagnosis of the majority of acute leukemias and testing must be performed to detect the presence or absence of genetic anomalies
-Widely used

49
Q

All immunophenotype

A

Early B ALL
CD34, CD19, Cytoplasmic CD22, TDT

Intermediate(common) B all
CD34,CD19 CD10 cytoplasmic CD22 TDT

Pre B aLL
CD34, CD19 cytoplasmic CD22 Cytoplasmic tdt variable

T ALL
CD2, CD3, CCD4,CD5,CD7,CD8 TDT

50
Q

AML prognosis

A

the cure rates have increased up to15% in pt older than 60 years and about 40% in pt below 60 years of age. The prognosis remains very poor in the elderly population.

51
Q

AML prognosis

A

All has the worst prognosis among alls seen more in adults. B lymphoblastic leukemia t(v11q23 MLL) is most common in infants with very poor prognosis. Depends on the age at the time of diagnosis, lymphoblast load, immunophenotype, and genetic abnormalities. Children, rather than infants or teens, do the best. Chromosomal translocation is the strongest predictor of adverse treatment outcomes for children and adults. Peripheral blood lymphoblast counts greater than 20 to 30X10^9L, hepatosplenomegaly, and lymphadenopathy(swollen lymph nodes) are the worst outcomes;

52
Q

MDS prognosis

A

overall survival depends upon multiple factors such as the severity of cytopenia(low amount of blood cells), % of blasts in Pbloor or BM, and karyotype. Supportive care is the predominant mode of treatment in the form of packed RC and PLT transfusions, GCSF and EPO medication, Induction chemotherapy, and allogeneic stem cell transplant.

53
Q

CLL/SLL prognosis

A

There is an aggressive disease with unmutated IGVH median survival of 8 years. and slow-growin mutated IGVH survival of 24r yrs. 5 % develop high-grade diffuse large B cell lymphoma survival of less than 1 year= Richter syndrome

54
Q

Burkitt’s lymphoma prognosis

A

munodeficiency-associated Burkitt lymphoma presents most often as nodal disease not favorable. High cure rates 60% to 90% chemotherapy aggressive treatment

55
Q

Hodgkins lymphoma prognosis

A

five years after diagnosis

56
Q

Hairy cell leukemia prognosis

A

it is a slow growing disorder chemo is a option when it get worst

57
Q

Both WHO MDS classification

A

2008
Refractory cytopenia with lineage dysplasia
Refractory anemia with ring sideroblasts
Refractory cytopenia with multilineage dysplasia
Refractory anemia with excess blasts
Myelodysplastic syndrome with isolated del(5q)
Myelodysplastic syndrome unclassifiable
Childhood myelodysplastic syndrome(provisional)

2016
MDS with single lineage dysplasia:granulocytic, erythroid or megakaryocytic
MDS with ring sideroblasts: the presence of 15% ring sideroblasts on the prussian blue-stained marrow smear
MDS with multilineage dysplasia: dysplasia in two or the three myeloid lineage
MDS with excess blasts(1&2)
MDS with isolated del(5q) chromosome
MDS unclassifiable

58
Q
A

hema final (1 decks)

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