Term
| Describe the neoplastic cells of CLL? |
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Definition
| small mature lymphocytes that are immunologically defective |
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Term
| Describe the neoplastic cells of chronic leukemia? |
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Definition
| granulocytic cells involving all stages but with predominance of late and intermediate stages |
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Term
| Name two chronic b cell leukemias. |
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Definition
| chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) |
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Term
| Name two chronic T cell leukemia. |
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Definition
| mycosis fungoides and sezary syndrome; and adult T-cell leukemia/lymphoma |
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Term
| What is the most common chronic leukemia in the western world? |
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Definition
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Term
| What is the age of onset of CLL? |
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Definition
| middle and late adulthood (90% are >50 years of age) |
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Term
| What organs/tissues are usually involved in CLL? |
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Definition
| bone marrow, lymph nodes, spleen |
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Term
| What are the clinical findings of CLL? |
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Definition
| one-third of pts asymptomatic at diagnosis (discovered on routine CBC); nontender LAD, usually generalized; splenomegaly usually mild to moderate |
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Term
| What is seen on PBS of CLL? |
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Definition
| small, mature-appearing lymphocytes and smudge or basket cells |
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Term
| What would you see on BMA of CLL? |
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Definition
| small round lymphocytes that gradually replace normal marrow cells |
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Term
| Ddx of lymphoid proliferation in pts aged childhood to young adult= |
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Definition
| reactive lymphocytosis vs. ALL |
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Term
| Ddx of lymhpoid cell proliferation in a middle-age to elderly adult= |
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Definition
| CLL vs. viral infection (ALL less likely) |
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Term
| Dx of PBS with large "atypical lymphs" and small lymphs. |
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Definition
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Term
| What functional immune abnormalities occur in CLL? |
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Definition
| hypogammaglobulinemia in 50%; AIHA in 10-25% |
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Term
| What is the immunophenotypic profile of CLL? |
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Definition
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Term
| What cytogenetic abnormality of CLL has a favorable prognosis? |
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Definition
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Term
| What CLL cytogenetic abnormality is associated with a less favorable prognosis? |
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Definition
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Term
| What is the most common chromosomal abnormality of CLL? |
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Definition
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Term
| What staging system is used for CLL? |
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Definition
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Term
|
Definition
| low risk, lymphocytosis; >10 year median survival |
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Term
|
Definition
| intermediate (>8 yr med survival rate); lymphocytosis + LAD |
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Term
|
Definition
| intermediate (>6 year median survival) lymphocytosis + splenomegaly |
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Term
|
Definition
| high risk (2 yr median survival); lymphocytosis + anemia |
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Term
|
Definition
| high risk (2 yr median survival); lymphocytosis + thrombocytopenia |
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Term
| How does RAI staging system define lymphocytosis? |
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Definition
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Term
| List the transformations that can occur in CLL? |
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Definition
| prolymphocytic (10-20%) or richter's syndrome (3-10%) or ALL (EXTREMELY RARE) |
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Term
| What is prolymphocytic transformation? |
|
Definition
| more immature lymphocytes associated with worsening symptamatology |
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Term
| What is Richter's syndrome? |
|
Definition
| development of large cell lymphoma involving lymph nodes or extranodal tissue; poor prognosis |
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Term
| When do you just follow CLL clinically and not treat it? |
|
Definition
| when patients are asymptomatic and at a low risk stage |
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Term
| When do you treat CLL patients with palliative chemotherapy? |
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Definition
| symptomatic with progressive disease or high risk stages |
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Term
|
Definition
| a monoclonal antibody against CD20 antigen which is present in SOME cases of CLL NOT ALL! |
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Term
| What is the typical age range of hairy cell leukemia? |
|
Definition
| middle aged men (age 50-55) |
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Term
| What are the clinical features of hairy cell leukemia? |
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Definition
| insidious onset with weakness, fatigue, weight loss. Splenomegaly and pancytopenia, with occasionally normal to elevated WBC count |
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Term
| Patients with what type of leukemia typically lack significant LAD? |
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Definition
|
|
Term
|
Definition
| slightly larger than normal with irrecular "shaggy" cytoplasmic projections |
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Term
| What test is positive for HCL? |
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Definition
| TRAP (tartrate-resistant acid phosphatase) positive |
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Term
| What are the features of bone marrow aspiration of HCL? |
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Definition
| "dry tap" on aspiration due to reticulin fibrosis |
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Term
| What's the leading cause of death in hairy cell leukemia? |
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Definition
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Term
| What's the treatment for hairy cell leukemia? |
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Definition
| splenectomy was once the firstline treatment but is now reserved for special cases; chemotherapy with purine analogues is now the treatment of choice |
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Term
| Describe the neoplastic cells of mycosis fungoides or sezary syndrome? |
|
Definition
| mature helper T cell phenotype (CD4+) that have irregular, indented, or cerebriform/convoluted nuclei |
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Term
|
Definition
| results when CD4+ T cells involve the peripheral blood (leukemic phase) causing generalized exfoliative erythroderma |
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Term
| What's another name for mycosis fungoides? |
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Definition
| cutaneous T-cell lymphoma |
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Term
|
Definition
| chronic myeloproliferative disorders= d/os with an increase in a predominant cell line |
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Term
|
Definition
| chronic myelogenous leukemia, polycythemia vera, essential thrombocythemia, idiopathic myelofibrosis |
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Term
| What is the typical age of CML? |
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Definition
| occcurs at any age but is most comon in middle-aged adults and peaks at 45-55 yoa. Uncommon in children and accoutns for <5% of all childhood leukemias |
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|
Term
| Describe the phases of CML. |
|
Definition
chronic= proliferation of granulocytic cells from myelobasts to segmented PMNs
aggresive/accelerated phase= increasing blasts |
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Term
| How long does the chronic phase of CML last? |
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Definition
|
|
Term
|
Definition
| when CML transforms into acute leukemia (blasts > 20% of all white cells in blood and/or marrow). occurs in the vast majority of patients |
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Term
| How long does it take on average after diagnosis of CML to develop a blast crisis? |
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Definition
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Term
| What is the MC cause of death in CML? |
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Definition
| complications in blast crisis (infections and hemorrhage) account for 80-90% of deaths |
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|
Term
| What are the clinical features of CML? |
|
Definition
| 1/3 are asymptomatic at diagnosis; splenomegaly (can be massive), LAD rarely prominent (unlike CLL) |
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|
Term
| What are the CBC abnormalities associated with CML? |
|
Definition
WBC count 20-500 thousand
thrombocytosis present in 30-50% of cases
eosinophilia and basophilia are characteristic |
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Term
| What are the features of PBS in CML? |
|
Definition
| granulocytic leukocytosis with all stages of myeloid maturation from myeloblasts to segs; may see some NRBCs; blasts are 5%; eosinophilia and basophilia are characteristic |
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Term
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Definition
|
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Term
| What are the two ways to diagnose CML via cytogenetics? |
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Definition
| 90-95% philadelphia chromosome by karyotype; 5% have a BCR-ABL translocation detected by molecular diagnostic tests |
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Term
| What's the difference between philadelphia chromosome of CML versus ALL? |
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Definition
| ALL is 190 kd while CML is 210 kd. The 210 kd allows for differentiation/maturation of the cell |
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|
Term
| What is imatinib (Gleevec)? |
|
Definition
| stops the tyrosine kinase activity of the BCR-ABL hybrid gene product |
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Term
| Describe the different types of blast crises that can terminate CML? |
|
Definition
myeloid (AML) in 60-70% of cases; >20% myeloblasts in peripheral blood and/or bone marrow
lymphoid (ALL): 25-30% of cases; lymphoblastic proliferation |
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|
Term
| How long after CML blast crisis do you survive? |
|
Definition
|
|
Term
| What is the first line therapy in most cases of chronic phase CML? |
|
Definition
|
|
Term
| What treatment can cure CML? |
|
Definition
|
|
Term
| How do you differentiate between leukemoid reaction and CML? |
|
Definition
| WBC can be a lot higher in CML; there is basophilia/eosinophilia present; there are no toxic changes and myeloblasts/promyelocytes are present; low to absent LAP and present philidelphia chromosome |
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