Mature Lymphoproliferative Disorders

Question 1

What is the difference between leukemias and lymphomas?

Answer 1

leuk - malign involving blood and marrow
Lymph - localized malignancy in tissues

Question 2

What does MBL
SLL
CLL
stand for and briefly describe them

Answer 2

MBL monoclonal B cell lymphoctosis- asymtomatic small # of clonal B cells in blood
SLL - small lymph lymphoma - in the lymph nodes and organs
CLL chronic lymph leukemia - peripheral blood (most common/old adults/B cell leukemia/can become autoimmune)

Question 3

Lab findings of CLL
WBC counts
specific cells? morphology?
Ig ? BM?

Answer 3

High WBC count
predominant of small lymphs with cracked mud chromatin
monotonous cell morphology
suppress IGs
BM infiltration

Question 4

Describe PLL (pro lymph leukemia)
common? Ages?
WBC
Cell types

Answer 4

rare - derived from B/T cell origin, early 70yrs - poor prognosis
WBC >100,000
half of cells are prolymphs
B cell - classic pro lymph w predominant nucleoli
T cell - not as classic may not have Dom nucleoli

Question 5

Describe Hairy Cell Leukemia
ages
Cell involved
lab findings

Answer 5

confused B cell with mono features
NSE + produces Its
middle aged pts
ISOTYPE 5 RESIST TO TARTIC ACID
splenomegaly
low WBC TRAP pos

Question 6

T/F lymphoma diagnosis is usually through a biopsy since dry taps are common

Answer 6

true

Question 7

Describe Hodgkins lymphoma
malignant cells
what do cells look like?

Answer 7

malignant clonal proliferation of B cells
(REED STEURNBERG CELLS)
large bi/poly lobed nucleus (eosinophilic)
OWL EYES
Hodgkin cell is variant but is not diagnostic

Question 8

Describe Non Hodgkins lymphoma
prerequisite of development
inciting agent

Answer 8

lymph malignancy
chromosomal abnormality
damage to region of genetic code that does growth/reproduc

inciting agent- mutagenic factor such as chem/raditation..etc

Question 9

Describe burkettes lymphoma
What cell derivation
associated w what other virus
what ages?
what does it look like?

Answer 9

B cell derivation leukemic conversion of L3
associate w epstine barr virus
USUALLY KIDS/AIDS ADULTS
punch out vacuoles
starry night

Question 10

T CELL lymphomas (cutaneous) Describe mycosis fungoides

Answer 10

skin lymphoma
characteristic lesions
progression to other organs

Question 11

(T cell lymphomas cutaneous) Describe Sezary syndrome

Answer 11

malign cells in blood+skin/organs
mature lymphs with cerebriform folding

Question 12

Plasma cell disorders:
Describe Solitary plasmacytoma

Answer 12

malign plasma clone localized to one area of bone/soft tissue
high tendency to develop MM

Question 13

Describe MM and key facts
plasma cells/wbc
lesions
Igs
protiens

Answer 13

few/low plasma cells in blood
low WBC/PLT/RBC
Osteolytic lesions
Monoclonal IG production (IgG)
bence jones proteins
>50% have proteinurime

Question 14

T/F: excretion of light chains does not damage

Answer 14

false it damages the nephron (renal failure)

Question 15

As Igs polymerize what does it cause?
rbc morph?

Answer 15

increased plasma viscosity
rouleaux
bind PLT/clotting factors

Question 16

Describe Plasma cell leukemia
where is it seen?
rarely is what?

Answer 16

seen in terminal acute phase of MM
>20% plasma cells in blood
rarely plasma primary cell leukemia (many immature in blood)

Question 17

Describe Waldenstrom Macroglob
what type of cells
what proteins produced
what phenomena
what gets coated

Answer 17

malignant lymph/plasma proliferation in BM
monoclonal production of IgM
hyper viscosity (near problems/cryoglobulins)
RAYNAURDS PHEN
thrombosis
PLTs coated with IgM

Question 18

Describe MGUS (monoclonal gam of unknown significance)
age range

Answer 18

3% of adults >70yrs
mild plasmacytosis
25% progress to MM

Question 19

Describe heavy chain disease (HCD)

Answer 19

rare, excess production of heavy chain portion of Ab