CLL

Question 1

Disorder of morphological mature but immunologically incompetent lymphocytes

Answer 1

Chronic Lymphocytic Leukemia (CLL)

Question 2

Most patients die from _________

Answer 2

Complications of CLL

Question 3

Median age at diagnosis

Answer 3

70 years

Question 4

Cells of origin in most patients

Answer 4

Clonal B cells arrested in B cell differentiation pathway

Question 5

Morphological in peripheral blood, CLL cells resemble

Answer 5

Mature lymphocytes

Question 6

CLL B lymphocytes typically show

Answer 6

B cell surface antigens

Question 7

2-5% of patients with CLL exhibit

Answer 7

T cell phenotype (CD5)

Question 8

A _______ associated with 2 to 8 fold risk for Developement of CLL

Answer 8

Positive family history

Question 9

CLL often diagnosed following incidental detection of

Answer 9

Lymphocytes on routine blood tests or asymptomatic lymphadenopathy

Question 10

What symptoms are associated with more advanced disease

Answer 10

Abdominal fullness
Fatigue
Reduced exercise tolerance
Weight loss
Night sweats
Recurrent infections

Question 11

Hepatomegaly due to

Answer 11

CLL infiltration of liver

Question 12

How does extranodal involvement manifest

Answer 12

Skin lesions
Pulmonary nodules

Question 13

Nights sweats or low grade fevers due to

Answer 13

Opportunistic infectious etiologies

Question 14

_____ and _____ in CLL patients may lead to exaggerated transient increase in lymphadenopathy or splenomegaly

Answer 14

Infections and vaccinations

Question 15

Diagnosis of CLL

Answer 15

Greater than 5x10 to power of 9 clonal B lymphocytes per L

Question 16

Confirm diagnosis of CLL

Answer 16

Peripheral blood flow cytometry

Question 17

_________ may be indicated for patients developing repeated infections

Answer 17

Immunoglobulin testing

Question 18

CLL cells are

Answer 18

Small to medium sized mature appearing lymphocytes with round nuclei, clumped chromatin, scant cytoplasm

Question 19

Bare nuclei that appear squashed, classic feature of CLL

Answer 19

Smudge cells

Question 20

should be obtained before treatment and in patients with anemia.

Answer 20

Direct anti globulin test

Question 21

Serum immunoglobulins typically ______ with disease duration

Answer 21

Decrease

Question 22

is commonly elevated and often increases with disease bulk.

Answer 22

Beta-2-microglobulin (B2M)

Question 23

have been associated with inferior treatment response and survival

Answer 23

B2M levels (>3-5 mg/L

Question 24

Elevated LDH is seen with

Answer 24

AIHA and may be modestly increased in rapidly progressive disease

Question 25

may be a sign of disease transformation

Answer 25

High or rapidly rising levels of LDH

Question 26

DDX of CLL,

Answer 26

Acute Lymphoblastic Leukemia (ALL) Acute Promyelocytic Leukemia Diffuse Large B-Cell Lymphoma (DLBCL) Follicular Lymphoma Hairy Cell Leukemia Lymphoblastic Lymphoma Mantle Cell Lymphoma Non-Hodgkin Lymphoma (NHL)

Question 27

Binet staging

Answer 27

Stage A – Hemoglobin 10 g/dL or higher, platelets 100000/ul or higher, and fewer than three lymph node areas involved Stage B – Hemoglobin and platelet levels as in stage A and three or more lymph node areas involved Stage C – Hemoglobin less than 10 g/dL or platelets less than 100000/ul, or both

Question 28

Most patients with early-stage CLL are

Answer 28

asymptomatic and have a relatively good long-term prognosis

Question 29

Treatment should be reserved for

Answer 29

active disease

Question 30

Active disease

Answer 30

Constitutional symptoms due to CLL such as fevers greater than or equal to 38 °C for 2 or more weeks without evidence of infection, significant fatigue, night sweats for 1 month or more without evidence of infection, weight loss (≥10% within the previous 6 months) Symptomatic, progressive, or massive lymphadenopathy Symptomatic, progressive, or massive splenomegaly Progressive marrow failure: worsening anemia (Hb < 10 g/dL) and/or thrombocytopenia (platelets <100 × 109 /L) Rapidly progressive lymphocytosis as defined by either an increase of greater than or equal to 50% over 2 months or a lymphocyte doubling time less than 6 months Steroid-refractory autoimmune cytopenias (immune thrombocytopenia [ITP], AIHA, pure red cell aplasia [PRCA]) Symptomatic or functional extra nodal involvement

Question 31

an active first-line treatment for physically fit patients

Answer 31

Fludarabine, cyclophosphamide, and rituximab (FCR)

Question 32

the only known curative therapy for CLL

Answer 32

Allogeneic stem cell transplantation

Question 33

The prognosis of patients with CLL

Answer 33

varies widely at diagnosis.

Question 34

In most patients CLL initially has

Answer 34

relatively benign course, but eventually enters a progressive, treatment-resistant phase.