Lymphoproliferative Disorders

Question 1

Lymphoproliferative disorder definition

Answer 1

group of malignant or benign neoplastic lesions of the lymphoid system
includes leukemias, lymphomas & disorders
LYMPHOID line including plasma cells
chronic, usually increased WBC & accumulation of malignant lymphs

Question 2

Lymphoproliferative disorders

Answer 2

1. CLL
2. Prolymphocytic Leukemia
3. Hairy cell leukemia
4. multiple myeloma
5. Waldenstrom’s macroglobulinemia
6. hodgkins lymphoma
7. non-hodgkin’s lymphoma

Question 3

Prolymphocytic Leukemia

Answer 3

rare, aggressive disorder
doesn’t respond to treatment
70% are B cell linage
predominant in males

Question 4

Prolymphocytic Leukemia lab findings

Answer 4

marked lymphocytosis - 55% of lymphs are prolymphocytes (large lymphs w/ nucleoli, CD19+)
anemia
thrombocytopenia

Question 5

Hairy Cell Leukemia (HCL)

Answer 5

rare, malignancy of B cells

does NOT develop into acute leukemia

Question 6

Epidemiology of Hairy Cell Leukemia

Answer 6

middle aged patients
more common in males than females (7:1)
mutation on x chromosome may play a role

Question 7

Hallmark of HCL

Answer 7

pancytopenia
Hairy lymphocytes!
abundant pale cytoplasm w/ cytoplasmic projections
oval nuclei
fine chromatin

Question 8

Symptoms of HCL

Answer 8

usually massive splenomegaly
weakness
fatigue
bruising

Question 9

Lab findings of HCL in PB

Answer 9

pancytopenia!
normo, normo anemia
presence of hairy cells - positive w/ TRAP cells
CD19+, CD20+, CD22+, CD25+, CD11c +

Question 10

Lab findings of HCL in BM

Answer 10

DRY TAP bc of hypercellularity

HCL is associated w/ increased amounts of reticulin

Question 11

Multiple Myeloma

Answer 11

malignancy of plasma cells in the BM
male predominance (65 yrs)

Question 12

Etiology of Multiple Myeloma

Answer 12

genetic mutation that causes cytokine IL-6 stimulation in the BM that results in excess plasma cells
interfere w/ production of other blood cells & immune system

Question 13

Characterization of MM

Answer 13

excess plasma cells -> excess antibodies -> excess protein (IgG maybe IgA) -> thick blood -> excess protein (light chains) excreted through the urine

Question 14

Bence-Jones protein

Answer 14

excess antibody light chains in the urine

positive in Multiple Myeloma

Question 15

Hallmark of MM

Answer 15

increase in plasma cells
ROULEAUX in PB
BONE LESIONS & PAIN
gamma spike on serum electrophoresis

Question 16

Symptoms of MM

Answer 16

bone pain & fracture - ‘punched out lesions’ of bones on x-rays
IL-6 is an activator of bone dissolvent
kidney issues

Question 17

PB lab findings of MM

Answer 17

leukocytosis - plasma cells
anemia
ROULEAUX
blue tinge to the slide (excess protein)
increased ESR
Bence-Jones protein in urine
increased serum protein - gamma spike

Question 18

BM lab findings of MM

Answer 18

hypercellular
increased # of plasma cells
mott cells & flame cells present

Question 19

MM prognosis

Answer 19

w/o treatment 6 months

w/ treatment average is 3 years

Question 20

Lymphoma

Answer 20

general term for cancers that start in the lymphatic system

Hodgkins & non-Hodgkins

Question 21

Causes of Lymphomas

Answer 21

gene mutations
environmental - chemical exposure
viruses - HIV, epstein-barr
idiopathic

Question 22

Diagnosis of Lymphomas

Answer 22

lymph node biopsy
imaging studies
flow cytometry immunophenotyping

Question 23

Hodgkin’s Lymphoma

Answer 23

12% of lymphomas , higher in adolescents & young adults
painless swelling of lymph nodes in neck/armpit/groin
recurrent high fever
Reed-Sternberg cells!

Question 24

Reed-Sternberg cells

Answer 24

characteristic of Hodgkin’s lymphoma
extremely large, abnormal lymphs
bi-nucleus (looks like 2 attached nuclei)

Question 25

Hodgkin's prognosis

Answer 25

one of the most curable forms of cancer

Question 26

Non-Hodgkin's Lymphomas

Answer 26

B-cell lymphomas or T-Cell lymphomas B more common than T lack Reed-Sternberg cells, resemble normal lymphoid cells

Question 27

Waldenstrom's Macroglobulinemia

Answer 27

AKA lymphoplasmacytic lymphoma similar to MM, but w/ IgM affects >65 yrs. no bone lesions or pain

Question 28

Waldenstrom's Macroglobulinemia Lab findings

Answer 28

leukocytosis - malignant cells are mixed population (lymphs & possibly plasma cells) anemia ROULEAUX elevated IgM on the monoclonal spike of SPE

Question 29

Small Lymphocytic Lymphoma

Answer 29

tissue equivalent of CLL | biopsy shows diffuse infiltrate of small mature lymphocytes

Question 30

Follicular Lymphoma

Answer 30

most common type of non-Hodgkin lymphoma 50-60 yrs biopsy shows 'nodular pattern' of lymphoid follicle CD19+, CD20+, CD5 -, sIg strong

Question 31

Mantle Cell Lymphoma

Answer 31

``` male predominance (> 60 yrs) present w/ disseminated disease involving lymph, skeletal, spleen, liver, GI systems multiple polyps involving small intestine ```

Question 32

Mantle Cell Lymphoma Immunophenotype

Answer 32

CD19+, CD20+, CD5+, CD23- , FMC-7 +, sIg + | relatively aggressive lymphoma

Question 33

Burkitt Lymphoma

Answer 33

high incidence in Africa HIV patients biopsy shows 'starry sky'

Question 34

'Starry Sky' appearance

Answer 34

characteristic of Burkett's Lymphoma sky - blue nuclei of neoplastic lymphs stars - scattered pale-staining body macrophages

Question 35

Lymphoblastic Lymphoma

Answer 35

tissue equivalent of ALL 80% T cells children affected > adults males > females