Heme Malignancies

Question 1

Acute Lymphocytic Leukemia (ALL)

Main types and who they effect

Answer 1

T-ALL: Teenager

B-ALL: kids + adults

Question 2

Translocation of B-ALL

Answer 2

kids: 12;21
adults: 9;22 (“Ph+”)

Question 3

General features of ALL

Answer 3

pancytopenia
>25% blasts in marrow
increased WBC count, but it’s immature cells

Question 4

Tx (beside chemo/whatever) for B-ALL

Answer 4

scrotum + CSF prophylaxis

for your BALLs

Question 5

AML (general features and who it affects)

Answer 5

Myelogenous blast proliferation; can be several lineages

Older adults, esp with exposure to radiation/chemo

Question 6

Acute Promyelocytic Leukemia (Translocation, Path, Tx)

Answer 6

Translocation: 15;17
Path: RAR receptor disrupted; Auer rods
Tx: ATRA (causes the blasts to differentiate)

Question 7

Acute Megaloblastic Leukemia (Assoc)

Answer 7

Down Syndrom < 5 years old

Question 8

Chronic Leukemia (Basics; Who?)

Answer 8

Mature lymphocytes in circulation

Older adults

Question 9

Chronic Lymphocytic Leukemia (Path, S/S, Progression)

Answer 9

Path: naive B-cells w/ CD5 and CD20 coexpression; smudge (fade-out) cells
S/S: GenLAD possible; hypogammaglobulinemia; AIHA (shitty IgG produced)
Progression: DLBCL possible

Question 10

Hairy Cell Leukemia (Acute/Chronic? Path, S/S, Tx)

Answer 10

Chronic Leukemia with mature B-cells
Path: hairy, cytoplasmic processes; TRAP+
S/S: red pulp SM; dry tap
Tx: 2-CDA (causes adenosine to accumulate toxically in neoplastic cells); IFN-α

Question 11

Adult T-Cell Leukemia Lymphoma (Acute/Chronic? Assoc, S/S, Tx)

Answer 11

Chronic Leukemia of mature CD4’s
Assoc: HTLV-1 (Japan + Caribbean)
S/S: RASH, LAD/HSM, lytic bone lesions/hypercalcemia

Question 12

Mycosis Fungoides (Acute/Chronic? Path, S/S, Complications)

Answer 12

Chronic Leukemia of mature CD4’s
Path: Pautrier microabscesses in epidermis
S/S: rash (nodules w/plaque)
Complication is Sezary Syndrome when you get the cells in the blood; “cerebriform nuclei”

Question 13

Myeloproliferative Disorder: What is it? Findings? Who does it affect? Complications?

Answer 13

Mature myeloid-origin cell proliferation
ALL elevated, though
Late adulthood
Complications: gout; marrow fibrosis (“burnt out”); Acute leukemias

Question 14

Chronic Myeloid Leukemia (S/S, Path, Tx, Complications)

Answer 14

Granulocyte predominance (esp basophils)
S/S: SM
Path: 9;22; LAP(-)
Tx: imatinib (TK inhibitor)
Complication: transformation to acute leukemia (could be ALL, too, though!)

Question 15

Polycythemia Vera (S/S, Path, Tx)

Answer 15

RBC predominance
Path: JAK2 mutation (NRTK)
S/S: blurry vision, headache, venous thrombosis, flushing, prurits (s/p bath!)
Tx: Phlebotomy

Question 16

Follicular Lymphoma (Cells, S/S, Path incl translocation, Tx, Complications)

Answer 16

Small CD20's
S/S: painless LAD
Path: architecture disruption and 14;18
Tx: CTX + rituximab
Complications: DLBCL

Question 17

Marginal Cell Lymphoma (Cells, Associations, Path)

Answer 17

Small CD20’s
Assoc: chronic inlamation (hashimoto, sjorgren, h.pylori)
Path: post-germinal cells in marginal zone; MALT is example

Question 18

Mantle Cell Lymphoma (Cells,Path)

Answer 18

Small CD20’s

Path: 11;14; cyclin D1 overexpression (G1->S)

Question 19

Burkitt Lymphoma (Cells, S/S, Who?, Association, Path incl translocation)

Answer 19

Intermediate CD20's
S/S: extranodal mass (African = jaw, sporadic = abdomen)
Who: kiddo/young adult
Association: EBV
Path: Starry Sky, 8;14 (b/c c-myc on 8)

Question 20

DLBCL (Cells, S/S, Who?)

Answer 20

Large CD20’s
S/S: common and aggressive
Older adults (sporadic OR follicular advancement)

Question 21

Hodgkin Lymphoma (Path, Cause, S/S)

Answer 21

Path: Reed-Sternberg cells = rare, neoplastic cells with multi-lobed nuclei AND nucleoli = CD15+CD30
Cause: Cytokines attract cells which form the mass
S/S: B-symptoms

Question 22

Multiple Myeloma (Cells, Path, S/S)

Answer 22

Cells: malignant plasma cells in marrow
path: RANK activation -> punched-out bone lesions (vertebrae + skull)
S/S: Infection risk, fracture risk
Rouleaux formations of RBC’s (lack charge)
If no symptoms, just M-spike = MGUS (very common in elderly)

Question 23

Langerhan Cell Histiocytosis (Cells, Rules)

Answer 23

Neoplastic Langerans Cells (monocyte origin, present to naive T-cells) = Birbeck TENNIS racket cells
Name = malignant = skin involvement
two names means 3 y.o.

Question 24

Letterer-Siwe Disease

Answer 24

= Langerhan cell histiocytosis
malignant
baby < 2 y.o.
S/S: skin rash an cystic skeletal defects
VERY fatal quickly

Question 25

Eosinophilic Granuloma

Answer 25

= Langerhan cell histiocytosis IN BONE
benign
fracture risk

Question 26

Hand-Schuller-Christian

Answer 26

= Langerhan cell histiocytosis
malignant
child > 3 y.o.
S/S: scalp rash, lytic skull defects, DI, exopthalmos