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Flashcards in WBC Disorders Deck (43):
1

Main causes neutropenia

Drug reactions (chemo)
Severe infections

2

Treatment for neutropenia

Treat infection
If chemo induced: GM-CSF or G-CSF

3

Causes lymphopenia

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

4

Which cell in body is most sensitive to radiation?

Lymphocytes

5

Causes neutrophilia

Bacterial infection
Tissue necrosis
High cortisol states: disrupts marginating pool

6

Left shift

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

7

Causes monocytosis

Chronic inflamm states
Malignancy

8

Causes eosinophilia

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

9

Cause basophilia

CML

10

Causes lymphocytic leukocytosis

Viral infections
Bordetella pertussis

11

Infectious mononucleosis

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

12

Acute leukemia

Blasts >20%
Crowd out normal hematopoiesis so present with signs/sxs cytopenias: anemia, thrombocytopenia, neutropenia

13

Marker for ALL

Tdt+

14

Marker for AML

MPO

15

Which leukemia associated with Down's after age 5

ALL

16

Which leukemia associated with Down's before age 5

Acute megakaryoblastic anemia

17

Markers B-ALL

CD1-, CD19, CD20

18

Types B-ALL

t(12;21)-good prog. Kids
t(9;22)-poor prog. Adults

19

Markers T-ALL

CD2-CD8; do not express CD10

20

T-ALL presentation

Thymic mass in Teenager
Since causes mass in lymphoid organ, called Acute Lymphoblastic LYMPHOMA

21

AML types

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

22

Myelodysplastic syndromes

Cytopenias, hypocellular bone marrow

23

Chronic leukemia

Prolif MATURE lymphocytes
High white count
Insidious
Older adults

24

CLL

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

25

Hairy cell leukemia

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

26

2-CDA

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

27

ATLL

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

28

Mycosis Fungoides

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

29

Sezary's syndrome: classical cell appearance

Cerebriform nuclei

30

Myeloproliferative Disorders

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

31

Complications myeloproliferative disorders

Hyperuricemia/gout
Progression to marrow fibrosis
Transformation to acute leukemia

32

CML

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)

33

How do you distinguish CML from leukemoid reaction

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

34

Polycythemia Vera

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

35

PV versus reactive polycythemia

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

36

Tumors producing ectopic EPO

RCC
HCC
Cerebellar hemangioma

37

Essential thrombocytopenia

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

38

Myelofibrosis

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

39

T(9;22)

CML on exams

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

40

T(15;17)

APML
retinoic acid receptor

41

T(8;14)

Burkitts
C-myc

42

T(11;14)

Mantle cell
Cyclin D1

43

T(14;18)

Follicular lymphoma
Bcl-2
Centroblasts, centrocytes