WBC Disorders

Question 1

Main causes neutropenia

Answer 1

Drug reactions (chemo)
Severe infections

Question 2

Treatment for neutropenia

Answer 2

Treat infection

If chemo induced: GM-CSF or G-CSF

Question 3

Causes lymphopenia

Answer 3

Immunodeficiency, e.g. DiGeorge
High cortisol states (causes apoptosis lymphos)
AI destruction, e.g. SLE
Whole body radiation

Question 4

Which cell in body is most sensitive to radiation?

Answer 4

Lymphocytes

Question 5

Causes neutrophilia

Answer 5

Bacterial infection
Tissue necrosis
High cortisol states: disrupts marginating pool

Question 6

Left shift

Answer 6

Inflammation causes increased immature neutrophils, marked by fewer Fc receptors (CD16)

Question 7

Causes monocytosis

Answer 7

Chronic inflamm states

Malignancy

Question 8

Causes eosinophilia

Answer 8

Neoplasia (HODGKINS--via IL-5 production)
Allergy, Asthma
Addison's
Collagen vascular diseases
Parasitic infections

Question 9

Cause basophilia

Answer 9

CML

Question 10

Causes lymphocytic leukocytosis

Answer 10

Viral infections

Bordetella pertussis

Question 11

Infectious mononucleosis

Answer 11

EBV>CMV
Reactive CD8+ cells
Generalized LAD, splenomegaly (PALS), high lympho count with atypical lymphos
Monospot test detecte heterophile IgM Abs; definitive test with EBV viral capsid antigen
Risk splenic rupture–no contact sports 1 yr

Question 12

Acute leukemia

Answer 12

Blasts >20%

Crowd out normal hematopoiesis so present with signs/sxs cytopenias: anemia, thrombocytopenia, neutropenia

Question 13

Marker for ALL

Answer 13

Tdt+

Question 14

Marker for AML

Answer 14

MPO

Question 15

Which leukemia associated with Down’s after age 5

Answer 15

ALL

Question 16

Which leukemia associated with Down’s before age 5

Answer 16

Acute megakaryoblastic anemia

Question 17

Markers B-ALL

Answer 17

CD1-, CD19, CD20

Question 18

Types B-ALL

Answer 18

t(12;21)-good prog. Kids

t(9;22)-poor prog. Adults

Question 19

Markers T-ALL

Answer 19

CD2-CD8; do not express CD10

Question 20

T-ALL presentation

Answer 20

Thymic mass in Teenager

Since causes mass in lymphoid organ, called Acute Lymphoblastic LYMPHOMA

Question 21

AML types

Answer 21

APML- t(15;17)
Acute monocytic-gums
Acute megakaryoblastic-Downs

Question 22

Myelodysplastic syndromes

Answer 22

Cytopenias, hypocellular bone marrow

Question 23

Chronic leukemia

Answer 23

Prolif MATURE lymphocytes
High white count
Insidious
Older adults

Question 24

CLL

Answer 24

Naive B cell prolif
CD5, CD20
SMudge cells
If goes to LNs=small lymphocytic LYMPHOMA
Can cause hypogammaglobulinemia (thus infection), AIHA, or can transform to diffuse large B cell lymphoma

Question 25

Hairy cell leukemia

Answer 25

``` Mature B cell prolif Stains TRAP Hairy cytoplasmic processes Splenomegaly (red pulp), dry tap, NO LAD (trapped in BM) Excellent response to 2-CDA ```

Question 26

2-CDA

Answer 26

Adenosine deaminase inhibitor. Adenosine accumulates, which is toxic to B cells

Question 27

ATLL

Answer 27

Prolif CD4+ Assoc with HTLV Rash, LAD, HSM, lytic bone lesions with hypercalcemia

Question 28

Mycosis Fungoides

Answer 28

Prolif CD4+ Rash, plaques, nodules-usu multiple Neoplastic T cells in epidermis=Pautrier's microabscesses If spreads to blood=Sezary's syndrome

Question 29

Sezary's syndrome: classical cell appearance

Answer 29

Cerebriform nuclei

Question 30

Myeloproliferative Disorders

Answer 30

Accumulation MATURE myeloid cells Neoplastic proliferation of ALL myeloid cells but named based on predominant cell Late adulthood

Question 31

Complications myeloproliferative disorders

Answer 31

Hyperuricemia/gout Progression to marrow fibrosis Transformation to acute leukemia

Question 32

CML

Answer 32

Prolif myeloid cells, esp granulocytes (BASOPHILS) t(9;22)-BCR-ABL with increased tyrosine kinase activity Tx: imantinib CAn transform to AML (2/3) or ALL (1/3)

Question 33

How do you distinguish CML from leukemoid reaction

Answer 33

CML: - LAP negative - t(9;22) - increased basophils

Question 34

Polycythemia Vera

Answer 34

Prolif myeloid cells, esp RBCs JAK2 kinase mutation Sxs hyperviscosity: blurry vision, HA, Budd-Chiari, flushed face, itching after bathing (mast cells) Tx with phlebotomy, hydroxyurea

Question 35

PV versus reactive polycythemia

Answer 35

PV: normal SaO2, decreased EPO Reactive-lung disease: decreased SaO2, increased EPO Reactive-ectopic: normal SaO2, increased EPO

Question 36

Tumors producing ectopic EPO

Answer 36

RCC HCC Cerebellar hemangioma

Question 37

Essential thrombocytopenia

Answer 37

Prolif myeloid cells, esp platelets JAK2 kinase mutation Sxs: increased risk bleeding or thrombosis Does not typically progress to marrow fibrosis or acute leuk

Question 38

Myelofibrosis

Answer 38

Prolif myeloid cells, esp MKs JAK2 kinase mutation MKs produce excess PDGF which causes marrow fibrosis Splenomegaly once BM fibrosed, leukoerythroblastic smear, increased risk infxn, thrombosis, bleeding Teardrop cells

Question 39

T(9;22)

Answer 39

CML on exams Note that can also be subtype of B-ALL. Adults, poor prognosis

Question 40

T(15;17)

Answer 40

APML | retinoic acid receptor

Question 41

T(8;14)

Answer 41

Burkitts | C-myc

Question 42

T(11;14)

Answer 42

Mantle cell | Cyclin D1

Question 43

T(14;18)

Answer 43

Follicular lymphoma Bcl-2 Centroblasts, centrocytes