Malignant disorders of leukocytes: ALL +

Question 1

what stain(s)/identifiers are pos or neg in lymphoblasts?

Answer 1

PAS positive, TdT positive!!;

MPO and SBB neg

Question 2

what gene translocation/fusion gene is found in CML and also often in B-cell lymphoblastic leukemia/lymphoma?

Answer 2

t(9;22)/ BCR-ABL gene

Question 3

Is B-cell or T-cell ALL more common?

Answer 3

B-cell

Question 4

Give the 4 classifications by FAB of acute lymphoblastic leukemias with brief description

Answer 4

L0- more common in children/young; small homogenous lymphoblasts; best prognosis; most T-cell ALl is L0

L1- more common in adults; large HETerogenous lymphoblasts

L3- the leukemic phase of Burkitt lymphoma, always B-cell lineage. Lymphoblasts large, uniform, prominent nucleoli, deep basophilic cytoplasm; poor prognosis

Burkitt Lymphoma- the high grade NHL phase of L3

Question 5

what virus is associated with Burkitt’s lymphoma and how is presentation different on different continents?

Answer 5

EBV; In Africa where it is endemic, presents mainly with facial bone tumors; In U.S. with abdominal mass

Question 6

CD markers of a progenitor B-cell ALL:

Answer 6

CD19, CD34 (ALL WBCs), TdT. CD10 is negative bc this comes with maturation

Question 7

CD markers of Early-pre-B-cell ALL

Answer 7

CD10, CD19, CD34, & TdT

Question 8

CD MARKERS OF Pre-B-cell ALL and then B-cell ALL

Answer 8

Pre: CD10/CALLA, CD19, CD20, TdT POS

B-cell ALL: CD19 AND 20 ONLY, no TdT

Question 9

List the progression in maturity of B-cell ALL categories and the markers that are associated

Answer 9

Progenitor: CD19, CD34, TdT

Early-pre-B: CD10, CD19, CD34, TdT

Pre-B: Cd10, CD19, CD20, TdT

B-cell: CD19 and CD20 only

Question 10

Markers that help ID T-cell lineage in ALL:

Also what is positive in immature T-cells that is positive for all immature lymphocytes?

Answer 10

CD2, 3, 5 & 7

TdT

Question 11

Give the genetic translocation associated, and oncogene if were given:

FAB L3/Burkitt lymphoma

T-cell ALL

Pre-B cell ALL (this one is in CML too)

Answer 11

1. t(8:14)
2. t(7;11)
3. t(9;22)

Question 12

In what ALL is a mediastinal mass due to thymus enlargement commonly presents especially in adolescents?

Answer 12

T-cell ALL

Question 13

are cells of ALL mature or precursor type? what about CLL?

Answer 13

precursor; mature or maturing in CLL

*the acute leukemias of either origin are of less mature cell stages, and chronic leukemias of mature cells

Question 14

What kind of leukemia: Patient is 65, had lymphocytosis at 6.0 x 10⁹/L WBCs, with homogenous small hyperclumped lymphocytes predominating; Flow showed CD19 and Cd20 ……patient had no symptoms.

What can this progress to?

Answer 14

CLL, B-cell type; the lymphoma stage is SLL, small lymphocyte leukemia

Question 15

What kind of leukemia: patient presented with massive splenomegaly and a dry bone marrow tap; with pancytopenia of PB; TRAP positive cells with hairlike projections, CD19 and 20 positive

Answer 15

Hairy Cell, HCL: a B-cell malignancy more common in males over 50

Question 16

Possible diagnosis?

pt presented with massive splenomegaly, elevated WBC count over 100 x 10⁹/L; with many prolymphocytes on peripheral smear

Answer 16

PLL: Prolymphocytic leukemia which is usually B-cell but not always

Question 17

this is a general description of what leukemia? lymphocytosis > 5 x 10⁹/L; WBC btw 20 - 200; normo-normo anemia; mature-appearing lymphocytes without nucleoli and checkerboard appearance of clumping, and many smudge cells

Answer 17

CLL

Question 18

In what type of CLL do the abnormal cells have large lymphs w/ moderate cytoplasm, moderately condensed chromatin and one prominent nucleoli usually?

Answer 18

Prolymphocytic

Question 19

This leukemia is associated with HTLV-1 (human T-cell lymphotropic virus); where is it endemic and what is unique about the leukemic cells? How does it present?

Answer 19

ATLL: adult T-cell leukemia/lymphoma; endemic in Japan, some of S. Pacific; flower-like or clover-like nuclei (lobulated); widespread lymphadenopathy/liver enlarged

Question 20

Thisis a description of what syndrome:

malignant mature T-cells with cerebriform/convluted nucleoli, scant cytoplasm; Cutaneous lesions, erythroderma, lymphadenopathy,

Answer 20

Sezary syndrome (look at images on p 203 of compendium)

Question 21

what condition is related to Sezary syndrome>

Answer 21

mycosis fungoides, a T-cell malignancy of the skin