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chronic lymphocytic leukemias


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What are the 8 Chronic Lymphoproliferative disorders?
1. Chronc Lymph Leukemia CLL-B
2. Chronc Lymph Leukemia CLL-T
3. Prolymphocytic Leukemia
4. Hairy Cell Leukemia
5. Sezary Syndrome
6. Hodgkin's lymphoma
7. Plasma cell myeloma
8. Waldenstrom's macroglobulinemia
What is the ratio of CLL cases that are T cell vs. B cell?
5% T cell to 95% B cell
In what patient population is CLL type B usually seen
CLL type T?
B: Old people - some older than 50 yrs old, most older than 60.
T: middle-aged people, 40's.
What is the M:F ratio for
2 to 1
For CLL-B lab data, what is the
1. WBC count
2. abnormal counts (2)
3. How do abnormal cells look?
4. What is commonly seen?
5. What develops later?
1. WBC is ELEVATED; avg 90,000
2. Absolute Lymphocytosis, relative neutropenia.
3. Uniform mature-like lymphs with soccer ball nuclei
4. Commonly see smudge cells.
5. Anemia, thrombocytopenia, neutropenia.
What do cytogenetics and molecular studies show in CLL-B?
-Abnormalities of Ch' 11 and 14

-Increased bcl-2 and bcl-1
what does bcl-2/1 do?
increases cell life because it inhibits APOPTOSIS.
Besides bcl-1/2, what cellmarker is found in some CLL-B cases?
CD5 -> this is a tcell marker - weird! but it confers a better prognosis.
What is the prognosis for this leukemia?
Depends on the Rai/Binet classification of what stage it's at.
What are the bone marrow findings in CLL-B?
NOT hyperplastic; will be infiltrated over TIME, but way later.
What are 4 main complications of CLL-B?
1. Anemia
2. Hypogammaglobinemia
3. Richter's Syndrome
4. Prolymphocytic transformation
What 3 pathologies contribute to the anemia in CLL-B?
1. BM infiltration of lymphs don't leave room for RBCs.
2. Sequestration hemolyses RBCs.
3. AIHA can develop
what is Hypogammaglobulinemia?
Not enough immunoglobulins on cells; thus patient susceptible to viral/bacterial infections.
What is Richter's syndrome?
Enlarged lymph nodes due to immature cells in them.
What does it tell us about the patient when they go into Prolymph transformation?
Their survival rate is SEVERELY decreased.
How do you identify prolymph transformation?
-Lymphs become more immature;
-Nucleolus is prominent CYCLOPS
-nucleus opens up
Regarding gammaglobulins what is
Bcell = hypogammaglobulinemia

Tcell = hypERgammaglobulinemia
why the hypergammaglob..?
Because Tcells stimulate Bcells; Bcells produce antibody..
What type of lymph morphology is seen in CLL-T?
Large, GRANULAR lymphs
-Cytoplasm is expanded and granules are easy to see.
What 3 sites can CLL-T disseminate?
1. Skin (cutaneous)
2. Bone marrow (infiltration)
3. CNS
Is prolymphocytic predomin. B or Tcell associated?
B cell - 95B:5T
Who gets Prolymphcytic leukemia?
Old men; older than 60 yrs.

Men:female is 6:1
What physical symptom sets PLL apart?
Marked splenomegaly.
What does the bone marrow and WBC count look like in PLL?
BM: infiltrated
WBC: elevated, >50% prolymphs
What is the patient's prognosis in PLL?
not good - 6 mo. survival rate post-diagnosis.
What is another name for Hairy cell leukemia?
Chronic Leukemic Reticuloendotheliosis.
What is Hairy Cell Leukemia?
A proliferation of abnormal cells in the RES and blood.
What population gets HCL?
Men over 50

M:F ratio is 4:1
What is a common physical symptom in HCL?
Marked Splenomegaly
What 2 CLLs show mkd splenomegaly so far?
1. Prolymphocytic
2. Hairy cell
What are 2 PB features that distinguish Hairy cell?
1. Mod-severe pancytopenia
2. Hairy cells!
What are 2 BM features that distinguish Hairy cell?
-Dry tap from incr. reticulin
-Bald hairy cells
What does TRAP stand for?
Tartrate Resistant Acid Phosphatase
What is a TRAP stain?
Stain that uses Tartrate first to destroy all normal cells; Hairy cells are resistant so will still stain w/ Acid phosph.
What is the prognosis/treatment for hairy cell leukemia?
Prognosis is good - can treat with drugs now.
No complications; drugs are very effective.
What are Mycosis Fungoides and Sezary Syndrome?
T-cell cutaneous lymphomas with a CD4 phenotype.
What is the difference between Mycosis fungoides and Sezary?
Mycosis - only in the skin
Sezary - in the blood.
What type of disease is mycosis fungoides?
Cutaneous, like exzema.
What 3 stages of symptoms are seen in mycosis fungoides?
1. Nonspcf red rash stage
2. Plague/scabby stage
3. Cutaneous tumor
What type of disease is Sezary syndrome?
a systemic disease.
What 3 stages of symptoms are seen in sezary syndrome?
1. Nonspcf red rash stage
2. Abnormal skin, lymph node, and peripheral blood cells.
What do sezary cells look like?
They have a convoluted/folded nucleus.
What feature about the cells in cutaneous lymphomas is very distinguishing?
PEARL NECKLACES - vacuoles are PAS positive.
How do you tell the difference between Reactive and Abnormal Lymphocytes?
Reactive: polyclonal and have varied cell morphology; both cytoplasm/nucleus are expanded.
Abnormal: All cells look alike - abnormal is clonal. High N:C

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