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Heme/Lymph > Leukemia Clinical Cases > Flashcards

Leukemia Clinical Cases Flashcards

1
Q

You see the HE below…

what is the typical age of the patient presenting with this?

Is this Immunophenotypically normal, polyclonal B cells

OR
Immunophenotypically abnormal, monocloncal B cells

A

Mononucleosis; more common in teens and young adults

Immunophenotypically normal, polyclonal B cells

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2
Q

What symptoms are typical of mononucleosis?

A

Splenomegaly d/t toxic T cells reacting to B cells,

hepatomegaly

fatigue and fever

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3
Q

Your pathologist attending points out the gingersnap apperance of the cells in the smear. What is the disease and what patient population is this typically seen in ?

A

Chronic Lymphocytic leukemia

seen in elderly

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4
Q

Your patient has been diagnosed with CLL, what is the pathology?

immunophenotypically normal, polyclonal B cells

OR

Immunophenotypically abnormal, monoclonal B cells?

A

Immunophenotypically abnormal, monoclonal B cells

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5
Q

The following are all examples of:

– Infectious mononucleosis
– IM-like syndromes
(common)
• CMV, Adeno, Acute HIV,Toxo
– Other viruses
• Hepatitis, chickenpox
– Tuberculosis
– Transient stresslymphocytosis (common)
– Whooping cough
– Polyclonal B-ce

A

Benign non-clonal lymphocytosis

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6
Q

The following are all examples of:

– Chronic lymphocyticleukemia (CLL)
– Leukemic lymphoma
– Sezary syndrome
– Hairy cell leukemia
– Adult T-cell leukemia
– T-cell large granular
lymphocytic leukemia

A

Neoplastic/clonal lymphocytosis

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7
Q

What clonality assesment methodology do we use for B cells?

A

• Light chain restriction on cell surface(kappa v lambda) by flow cytometry or
immunohistochemistry
• IgH variable gene PCR
• Immunophenotypic aberrancy (ie. CD5 expression on B cells)

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8
Q

What clonality assesment methodology do we use for T cells?

A
  • T cell receptor gene PCR
  • Immunophenotypic aberrancy
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9
Q

What percentages of Lymphoblast leukemias are repreasented by B cells and T cells

A
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10
Q

A 29 yo male teacher has increasing SOB while jogging and fatigue for past 3 weeks.

Afebrile, no change in weight/loss of appetite/muscle aches/sweats/bruising

PE he looks healthy, no rashes

Labs: 11.3/mL WBC

HgB = 6.3 and hematocrit = 19

MVC = 100 and plats = 115,000

has abnormal WBC smear w/ over 60% of cells like this.

What do the cells represent and what other tests should we get to Dx patient?

A

immature lymphoblasts–shoudn’t have that many in blood.. over 20% is concerning

Do flow cytometry to get more info! we cannot at this point give definitive Dx nor say what they will mature into

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11
Q

In Acute leukemia:

  • _____blasts in the blood or bone marrow
  • May involve extramedullary sites too
  • Initially classified into two categories:
A

≥20%

– Myeloid (AML) vs. Lymphoid (ALL)

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12
Q

Acute leukemia is diagnosed based on morphology and immunophenotype

we see

CD13, 14, 15, 33, 117 and Auer rods in what type of Acute Leukemia?

A

In Myeloid leukemia

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13
Q

These markers are present in what type of acute leukemia?

CD3, CD19, CD20

A

Acute lymphoid leukemia

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14
Q

Which picture below is a mature and which is the immature cell?

A

Blasts or immature cells are on the right

Mature cells are on the left

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15
Q

Flow cytometry reveals 60% population of large cells are positive for CD10, CD19 and CD34 and NEGATIVE for CD3 and CD20

Where in the body where we also see these cells?

A

Cerebrospinal fluid, Lymph nodes, Bone marrow and splenic sinusoids

This is B-ALL and we see very dispersed invovement

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16
Q

In a patient will B-ALL would we see:

expanded myeloid lineage

majority of cells showing MPO expression

Cells with cytogenic abnormalities

A

Cells with cytogenic abnormalities

–this is seen in all ALLs

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17
Q

You see these resuts on a cytogenetic analysis from patient with B-ALL. What is true of these test results?

A

Hyperdiploidy

This is FAVORABLE for patient!!!

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18
Q

Patient presents with large mediastinal mass, if this is neoplastic, what type of tumor cells would we expect to see?

A

CD3+

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19
Q

AML is seen in adults or children

has what cell features?

If we see myleodysplasia, what do we call it?

A

Adults > Children
• Myeloblasts ± Auer rods
– May have granulocytic, erythroid, megakaryocytic, monocytic differentiation
• Myelodysplasia = pre-leukemia

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20
Q

ALL is seen in children or adults?

Does or does not have pre-leukemia state?

HOw is it classified?

A

Children

no pre-leukemia state

85% B cells

15% T cells

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21
Q

What is the most common cytogenetic abnormality in child with B cell ALL?

Is this a good or bad prognosis?

A

t(12;21)

good prognosis

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22
Q

What is the cyogenetic results is the worst prognosis in child with B cell ALL?

A

t(9;22)

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23
Q

T-ALLs are seen in what type of patient and how does it present?

A

young male adolescent

mediastinal mass

worse ALL prognosis

24
Q

What are the key prognostic factors in B-ALL?

A
  • Age
  • Cytogenetics
  • White blood cell count
  • CSF involvement
  • Marrow involvement following therapy
25
Q

What are the 6 bad signs for B-ALL?

A

– <2y/o, >10y/o
– t(9;22), MLL gene
– Hypodiploidy (<45chromosomes)
– Elevated WBC count (>100k/uL)
– CSF involvement
– Marrow involvement at day 15 post chemo

26
Q

What are the three Good prognostic indicators for B-ALL?

A

– 2-10y/o
– Hyperdiploidy (>50 chromosomes)
– t(12;21)

27
Q

T-ALL

•______ masses in adolescents
– “thymic lymphomas”
• May appear morphologically_____ to B-ALL
– Flow cytometry:

A

Mediastinal

identical

CD3(+), CD1a(+)

28
Q

What are the 4 stages in ALL treatement?

A
  • INDUCTION
  • INTENSIFICATION
  • CNS PROPHYLAXIS
  • MAINTENANCE
29
Q

What is Blinatumoamb and what is it used for?

A

bispecific T-cell engaging (BiTE®)
antibody construct that directs cytotoxic T-cells to
CD19 expressing B-cells.

(Chimeric antigen receptors for the adoptive T cell
therapy of hematologic malignancies)

30
Q

What is the first line TX for adult patients with Ph+ chromosome ALL

A

Dasatinib; tyrosine kinase inhibitor~ take pill every day

31
Q

65 yo female has increased fatigue for 6mo and LUQ, decreased appetite.

Healthy appearing, but you know several firm, mobile lymph nodes about 1.5 cm in posterior triangles.

Tip of spleen is palpable

WBC = 60,000

90% differential lymphocytes, 8% neutrophils, 2% eosinophils

HgB = 12 and plats = 170,000

which of following is most likley true:

Pierpheral blood lymphocytes are most likely T cells

Peripheral blood lymphocytes have same immunophernotype as those in the lymph nodes and spleen

EVB serology indicates recent infection

A

Peripheral blood lymphocytes have same immunophernotype as those in the lymph nodes and spleen

32
Q

WBC = 60,000

90% differential lymphocytes, 8% neutrophils, 2% eosinophils

What is the absolute lymphocytes count in this case?

A

54,000

33
Q

If you see a peripheral smear with gingersnap cells, what is the Dx?

What CD markers do you expect?

would this be kappa expressing, lamba expressing or both

A

CLL (may be asymptomatic)

CD5+, CD19+, CD23+ and cells express kappa light chain only is more common

34
Q

If a patient has CLL and the oncologist recommends a ‘watch and wait’ approach, what is true about this type?

A

this is INDOLENT

35
Q

Pt was dx with CLL 9 months ago with a ‘watch and wait’ approach suggested. She now has progressive anemia (hgb = 8) and lymphocyosis (140k/uL) with night sweats and fever. Why is this going on?

A

Bone Marrow replaced with neoplastic cells

36
Q

If you have patient with 17p deletion that has CLL, what is the significance?

A

very bad prognostic indicator!

17p deletion is assoicated with loss of p53

37
Q

Why do we see splenomegaly in hairy cell leukemia

A

dt red pulp inflitration

38
Q

Hairy cell leukemia:

what population of people?

______cells

associated with fibrosis in marrow called:

A

40-60 yo males

TRAP+ cells

dry tap

39
Q

What would our flow dx be of Hairy cell leukemia?

A

bright CD11c and CD22 expression

(indolent)

40
Q

Mantle Cell lymphoma and CLL/SLL both have what CD marker?

How are they different?

A

Both have CD5+

Mantle Cell lymphoma = CD23- and FMC7+

CLL/SLL = CD23+ and FMC7-

41
Q

Follicular lymphoma and marginal zone lymphoma are the same and different in CD markers how?

A

BOTH are CD5-

Follicular lymphoma = CD10+

MALT lymphoma = CD10-

42
Q

What are four bad prognostic indicators for CLL/SLL?

A

– High Rai stage
– Unmutated IGHV gene
– CD38/ZAP-70 expression
– Deletion 11q, 17p

43
Q

What are 3 Good prognostic indicators in CLL/SLL?

A

– Low Rai stage
– Mutated IGHV gene
– Deletion 13q

44
Q

What is the prognostic staging system for CLL?

A

Stage 0 — 150 months
Stage I — 101 months
Stage II — 71 months
Stages III/IV — 9 months

45
Q

What are the lab indication to tx in CLL?

A

Rapid lymphocyte doubling time (<6months)

if hemoglobin is below 10

if plats below 100k

46
Q

What are clincal indications for treatement in pt with CLL?

A

Autoimmune anemia or thrombocyopenia

Richter transformation (large cell transformation)

Enlarging symptomatic lymph nodes

B symptoms

47
Q

What drug therapy would we give to symptomatic patient with CLL?

A

Rituximab

and cyclophosphamide

*95% of patients will respond, this is not cure, just tx

48
Q

What are the bad cytogenetic prognosis for CLL?

A

17p

49
Q

What are the not so bad cytogenetic prognosis for CLL?

A

del (13q)

Mutated IgHV

(intermediate = trisomy 12)

50
Q

what signaling is overacting in CLL?

A

B cell signaling: the Pl3k adn BTK) signaling motif

***to cure, give pts Pl3 kinase inhibitor!!! see much improved results

51
Q

Sezary Syndrome triad

A

Triad: erythroderma, generalized
lymphadenopathy, circulating sezary cells

52
Q

• “Cerebriform nuclei” and CD4 + T cells

seen in:

A

Sezary Sydnrome

(think cerebriform = brains~~ what patients faces look like)

53
Q

What is our concern with Sezary and MF?

A

that it will transform into aggressive T cell tumor

54
Q

What disease do we see flower cells in?

A

Adult T-cell leukemia/lymphoma

HTLV-1 associated! most infection DO NOT devo ATLL

55
Q

CD4(+) Tcell neoplasm, seen in Japan/Caribean/Africa

HYPERcalcemia and aggressive

A

Adult Tcell leukemia/lymphoma

56
Q
  • Lymphoproliferative disorder of cytotoxic T cells
  • Indolent disorder
  • Usually presents with neutropenia, anemia and splenomegaly
  • Associated with autoimmune disorder (rheumatoid arthritis)
A

Large granular lyphocytic leukemia

57
Q

What characterstics are presented in large granular lymphocytic leukemia?

A

Neutropenia, and anemia and splenomegaly

Heme/Lymph (10 decks)

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