Glossary of 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
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)
- 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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