Lymphocytic leukemias and related conditions

bone marrow

where do lymphocyte stem cells originate from?

T or B lymphocytes

what do lymphocyte stem cells develop into?

• lymph nodes

• spleen

• other lymphoid tissues

where do T or B lymphocytes migrate after development?

malignant proliferation of lymphoblasts

what is ALL (acute lymphoblastic leukemia)?

ALL (acute lymphoblastic leukemia)

what is the most common malignancy of children?

limited maturation and function 

what do abnormal blasts have?

curable

what is ALL the first to be called?

it has the best survival rate of any of the acute leukemias

why is ALL considered “curable”?

cell morphology

what is the classification of ALL primarily based on?

3

how many categories of cell morphology does ALL have?

immunophenotype and cytogenetics

what is the WHO classification of ALL based on?

7

how many categories does the WHO classification of ALL have?

L1

what is the most common FAB classification of ALL?

• small, uniform lymphoblasts

  • scant cytoplasm

  • inconspicuous nucleoli

• pediatric age group—best prognosis

• B cell lineage—may be t(12;21)

what is L1 of ALL?

over 15 years old

when is L2 classification of ALL often found?

• large, pleomorphic lymphoblasts

  • more abundant cytoplasm

  • less condensed nuclei

  • more conspicuous nucleoli

  • cells often have indented nucleus

• B or T cel lineage

what is L2 of ALL?

Burkitt’s type leukemia

what is L3 of ALL also known as?

cells are vacuolated with abundant, deeply basophilic cytoplasm

what characterizes Burkitt’s type leukemia?

• represents the leukemic phase of Burkitt lymphoma

  • leukemic B cells

  • BCL-2 (B-cell lymphoma protein) is absent so cells rapidly proliferate

• worse prognosis

what is L3 of ALL?

the B-cell lymphoma protein

what is BCL-2?

1. cytochemical staining using PASS

2. cytoplasmic, nuclear, or surface immunoglobulins

3. common ALL antigen (CALLA)

what are the 3 types of markers used for immunologic and cytochemical ALL classification?

glycogen

what does periodic acid schiff detect?

ALL from AML

what does periodic acid schiff differentiate?

flow cytometry

what is used to detect cytoplasmic, nuclear, or surface immunoglobulins?

CD34

what does both B and T cells express?

• CD10

• CD19

• CD20

• CD21

• CD22

• CD24

• C79a

what are the B cell markers?

• CD2

• CD3

• CD4

• CD5

• CD7

• CD8

what are the T cell markers?

early B lymphocyte precursors

where is the common ALL antigen found?

CD10 surface marker

what is CALLA the same as?

terminal deoxynucleotidyl transferase

what is the nuclear enzyme?

cell nuclei

where is DNA polymerase found?

• T cell ALL

• B lymphoblasts—not mature B cell ALL

which cells have DNA polymerase?

ALL from malignant lymphomas

what does Tdt distinguish?

no

is TdT beneficial in classifying ALL?

• often presents with infections, anemia, and thrombocytopenia

• petechiae and bruising first symptom

• more likely to go to CNS than any other leukemia

what are the clinical presentations of ALL?

• WBC can be low, normal, or high

• anemia is often severe

• decreased platelet count, often severe

• bone marrow is hypercellular with lymphoblasts

what are the laboratory findings of ALL?

normal

what is the M:E ratio of ALL?

cytochemical stains

what is used as a differential diagnosis of ALL?

esterase, Sudan Black, myeloperoxidase

what cytochemical stains is AML positive for?

PAS and TdT

what cytochemical stains is ALL positive for?

• chemotherapy

• support with blood products 

• transplant for those who relapse during the first 6-12 months

what is the treatment of ALL?

WBC upon diagnosis and age of patient

what is the prognosis of ALL related to?

long

how long are remissions of ALL?

yes

do most patients achieve remission with ALL?

an lymphoproliferative disorder

what is chronic lymphocytic leukemia?

chronic lymphocytic leukemia

what is the most common of all leukemias?

older adults

who does chronic lymphocytic leukemia primarily affect?

men

are men or women frequently diagnosed with chronic lymphocytic leukemia?

best prognosis for adults

what does chronic lymphocytic leukemia have?

• lymphocytosis in both peripheral blood and bone marrow

• lymphocytes are mature, well-differentiated

• smudge cells present

• neutropenia, NC/NC anemia, thrombocytopenia

• often discovered by accident

• chronic fatigue

• lymphadenopathy

• splenomegaly

• altered humoral immunity

what are the clinical presentations of chronic lymphocytic leukemia?

disintegrated lymphoid cells

what do smudge cells represent?

suppression of all classes of immunoglobulins and impaired immunologic activity

how is humoral immunity altered in chronic lymphocytic leukemia?

• increased susceptibility to infections

• autoimmune disease may develop

• flow cytometry

what are the clinical findings of chronic lymphocytic leukemia?

• idiopathic thrombocytopenic purpura

• autoimmune hemolytic anemia

what autoimmune diseases can develop with chronic lymphocytic leukemia?

• high WBC

• mild anemia and mild thrombocytopenia

• hypercellular bone marrow

• NC/NC anemia

• positive direct antiglobulin test

• may have extra chromosome 12

• flow cytometry will determine if B or T cell

• no cytochemical stains required

what are the laboratory findings of chronic lymphocytic leukemia?

15,000-100,000/µL

what can the WBC count be in chronic lymphocytic leukemia?

3:1

what is the M:E ratio with chronic lymphocytic leukemia?

B cells, mature lymphocytes, smudge cells

what is found in the peripheral blood with chronic lymphocytic leukemia?

if patient is having problems or if WBC count is very elevated

when is chemotherapy used to treat chronic lymphocytic leukemia?

50%

what is the median 5 year survival of chronic lymphocytic leukemia?

succumb to some other illness

what do most patients with chronic lymphocytic leukemia often do?

a subacute lymphocytic leukemia with the predominant cell being the pro-lymphocyte

what is prolymphocytic leukemia?

>55%

what percent of circulating lymphoid cells have morphology of prolymphocyte during prolymphocytic leukemia?

very high

what is the WBC count of prolymphocytic leukemia?

larger with a less clumped nucleus

how do prolymphocytes look?

a prominent nucleolus

what do prolymphocytes have?

leukemic reticuloendotheliosis

what is hairy cell leukemia also referred as?

fine, hair-like, irregular cytoplasmic projections

what do lymphocytes have with hairy cell leukemia?

diagnostic

what are hairy cells?

pancytopenia

what is common in hairy cell leukemia?

tartrate-resistant acid phosphatase (TRAP)

what are cells with hairy cell leukemia?

leukocytes

where is acid phosphatase found?

isoenzyme 5

what do hairy cells contain?

tartrate inhibition

what is the acid phosphatase activity of hairy cells resistant to?

strongly TRAP positive

what is hairy cell leukemia said to be?

splenomegaly

what is present in hairy cell leukemia?

dry tap

what is common with hairy cell leukemia bone marrow aspirate?

lymph nodes and other lymphoid organs

what does small lymphocytic lymphoma primarily involve?

chronic lymphocytic leukemia

what is small lymphocytic lymphoma similar to?