Chronic Lymphocytic Leukemia

Question 1

CLL (chronic lymphocytic leukemia) - overview

Answer 1

*cancer of the white blood cells
*malignant cell is more differentiated than CML
*result is excess numbers of mature lymphocytes
*on a continuum with SLL (small lymphocytic lymphoma) that has no circulating neoplastic cells and resides in lymph nodes

defined by CD5+ B cells! (CD5+, CD19+, CD20+)

Question 2

CBC of CLL (chronic lymphocytic leukemia)

Answer 2

*elevated WBC count
*increased lymphocytes (ABSOLUTE LYMPHOCYTOSIS)
*SMUDGE CELLS can be seen of peripheral smear

*normal neutrophils
*no presence of neutrophil precursors in peripheral blood
*normal platelets
*normal RBCs/Hb

Question 3

CLL (chronic lymphocytic leukemia) - epidemiology

Answer 3

*most common leukemia in adults
*median age of presentation: 70 yo but not uncommon to dx patients in their 30s

Question 4

CLL (chronic lymphocytic leukemia) - clinical presentation

Answer 4

*often presents with no symptoms
*if symptomatic: fevers, unintentional weight loss, night sweats, fatigue
*majority have lymphadneopathy
*splenomegaly and sometimes hepatomegaly

Question 5

diagnosis for CLL (chronic lymphocytic leukemia)

Answer 5

*do NOT need bone marrow for diagnosis

*peripheral blood sample for FLOW CYTOMETRY (looking for a B cell with expression of CD5, a T cell marker):
(+) CD5, CD19, CD23
(+/-) CD20 (weak expression)
(+) surface immunoglobulin
light chain restriction (only kappa or lambda, not both)
*send chromosomal studies to get info on prognosis

Question 6

CLL (chronic lymphocytic leukemia) - indications for treatment

Answer 6

*can wait years for treatment
*need to have symptoms from their CLL that are WORSE than potential symptoms of chemo:
-symptomatic lymphadenopathy
-symptomatic splenomegaly
-“symptomatic” counts: anemia or thrombocytopenia as a result of progression of CLL in the marrow (stage III or IV disease)

note - absolute white cell count is NOT listed as an indication to treat

Question 7

CLL (chronic lymphocytic leukemia) - immune dysregulation: insufficient immune system

Answer 7

*difficult to fight infection
-often need prolonged courses of antibiotics

*hypogammaglobulinemic
-quantitative immunoglobulins often reveal patients to be pan-hypoglobulinemic
-if patients have persistent infections or infections severe enough to require hospitalization, will treat with IVIG

Question 8

CLL (chronic lymphocytic leukemia) - immune dysregulation: overactive immune system

Answer 8

*inappropriate destruction of “self” cells
-autoimmune hemolytic anemia
-immune-mediated thrombocytopenia

*labs for AIHA are the same as with any other hemolytic anemia:
-elevated LDH, bilirubin
-low Hb, haptoglobin
-Coombs +

Question 9

hairy cell leukemia - overview

Answer 9

*very slow-growing
*B cell malignancy (CD19, 20, 22)
*has aberrant expression of T cell marker CD103
*TRAP-positive
*clinically, notable for very large spleens and dry taps on bone marrows

Question 10

hairy cell leukemia - bone marrow morphology

Answer 10

*in bone marrow, cells look like FRIED EGGS
*marrow also has lots of fibrosis

Question 11

hairy cell leukemia - treatment

Answer 11

PURINE ANALOGS:
-cladribine
-sometimes just one week of chemo, ever