2Nd Semester Lab Dx Unit 2

Front 1

CML
MYELOFIBROSIS
LEUKEMOID REACTION
anemia?

Back 1

+/-
YES
+/-

Front 2

CML
MYELOFIBROSIS
LEUKEMOID REACTION

Teardrop RBCs?

Back 2

NO
YES
NO

Front 3

CML
MYELOFIBROSIS
LEUKEMOID REACTION

Nucleated RBC’s?

Back 3

NO
YES
NO

Front 4

CML
MYELOFIBROSIS
LEUKEMOID REACTION

WBC>100,000

Back 4

OFTEN
VERY RARE
RARE

Front 5

CML
MYELOFIBROSIS
LEUKEMOID REACTION

WBC 50-100,000?

Back 5

COMMON
RARE
COMMON

Front 6

CML
MYELOFIBROSIS
LEUKEMOID REACTION

WBC 30-50,000?

Back 6

COMMON
UNCOMMON
COMMON

Front 7

CML
MYELOFIBROSIS
LEUKEMOID REACTION

Basophils increased?

Back 7

YES
NO
NO

Front 8

CML
MYELOFIBROSIS
LEUKEMOID REACTION

Eosinophils increased?

Back 8

YES
NO
NO

Front 9

CML
MYELOFIBROSIS
LEUKEMOID REACTION

>5% Myelocytes or younger cells?

Back 9

YES
MAYBE
NO

Front 10

CML
MYELOFIBROSIS
LEUKEMOID REACTION

LAP?

Back 10

LOW
VARIABLE
HIGH

Front 11

CML
MYELOFIBROSIS
LEUKEMOID REACTION
Marrow?

Back 11

MYELOID HYPERPLASIA & BASOS & EOS

FIBROSIS

MYELOID HYPERLASIA

Front 12

CML
MYELOFIBROSIS
LEUKEMOID REACTION

Ph1 chromosome

Back 12

KEY TO DX
NO
NO

Front 13

Normal neutrophil range

Back 13

1800-6700

Front 14

Normal Lymphocyte range

Back 14

1400-3900

Front 15

Pelger-Huet anomaly

Back 15

neutrophils are generally bilobed and may be unsegmented

Front 16

May-Hegglin anomaly

Back 16

basophilic inclusions in WBC’s, associated with thrombocytopenia

Front 17

Chediak-Higashi syndrome

Back 17

giant neutrophil granules (abnormal killing of bacteria in lysosomes

Front 18

Alder’s anomaly

Back 18

deep purple granules. May have no clinical association or may be associated with mucopolysaccharide disorders.

Front 19

Maroteaux-Lamy syndrome

Back 19

abnormal red-violet granules and lymphocyte vacuoles

associated with mucopolysaccharide disorders

Front 20

which leukemia is 85% of adult acute leukemia

Back 20

ACUTE NON-LYMPHOCYTIC LEUKEMIA

Front 21

presentation of Acute Non Lymphocytic Leukemia (AML)

Back 21

Can present either as pancytopenia, or with anemia, thrombocytopenia, and elevated WBC due to the presence of myeloblasts in the peripheral blood.

Front 22

Which leukemia is 85% of childhood acute leukemia

Back 22

ACUTE LYMPHOBLASTIC LEUKEMIA

Front 23

DIFFERENTIAL DIAGNOSIS OF AN ELEVATED GRANULOCYTE COUNT (5)

Back 23

1 Infection – mild elevation of WBC, 10,000 to 15,000. Almost all polys, some bands

2 Chronic myelogenous leukemia – WBC may be very high, >50,000 or even > 100,000. At least 5% myelocytes or younger

3 Myelofibrosis - usually normal or low WBC, but may look like CML, w/out phili chrom

4 Leukamoid reaction - exaggerated response to infection. WBC>30,000 or 40,000 is generally considered a leukemoid reaction.

5 - P Vera – an elevated WBC may be present though elevated hematocrit dominates

Front 24

WHITE CELL INCLUSIONS, name 3

Back 24

Erlichiosis

Histoplasmosis

Dohle bodies – sign of infection

Front 25

The key clinical problem in AML

Back 25

infection due to neutropenia

Front 26

For AML, remission means that:

Back 26

the tumor burden has gone from 1 trillion cells to 1 million cells.

Front 27

The Ph1 chromosome is a __ translocation which creates a ___ oncogene.

Back 27

9:22

bcr-abl

Front 28

leukocyte alkaline phosphatase in CML

Back 28

low

Front 29

gleevec, what's it do, how's it work

Back 29

The bcr-abl in CML codes for a tyrosine kinase, and gleevec inhibits the tyrosine kinase,

Front 30

EVALUATION OF THE PATIENT WITH AN ELEVATE GRANULOCYTE COUNT, 5 steps

Back 30

1) markedly elevated > 30,000? slightly elevated suggests infection

2) Are 5% of the cells myelocytes or younger? If so, consider CML even if the WBC is not markedly elevated

3) Examine the patient for splenomegaly (CML, Myelofibrosis)

4) If the diagnosis is not established at this point, do a marrow aspirate and biopsy, and obtain cytogenetics.

5) If it looks like CML but the Philadelphia chromosome is not seen, obtain tests for bcr-abl.

Front 31

Neutropenia: Underproduction
7 conditions

Back 31

1) Myelophthisic – leukemia, lymphoma, non-hematologic tumor (rare)

2) Myelodysplasia – a late manifestation of disease

3) Aplastic anemia (which is actually a pancytopenia)

4) Folate and B-12 deficiency (usually only an anemia, can be pancytopenia)

5) Cyclic neutropenia – rare

6) Congenital neutropenia

7) Chediak-Higashi syndrome (problem of WBC function/also low neutrophils)

Front 32

Neutropenias of decreased survival, 4 conditions

Back 32

1) Hypersplenism

2) Autoimmune neutropenia

3) Felty’s syndrome – (Rheumatoid arthritis, splenomegaly, neutropenia)

4) Large granular lymphocyte syndrome (a T/NK cell disorder often in association with rheumatoid arthritis)

Front 33

Clinical problems of neutropenia, 3

Back 33

1 Systemic infection

2 oral ulcers

3 ecthyma (due to pseudomonas)

Front 34

Most common adult leukemia

Back 34

CHRONIC LYMPHOCYTIC LEUKEMIA

Front 35

staging of CLL

Back 35

Stage 0
Elevated lymphocytes

Stage 1
As above plus adenopathy

Stage 2
As above plus hepatomegaly or splenomegaly

Stage 3
As above, plus anemia*

Stage 4
As above, plus thrombocytopenia*

Front 36

Lab tests for EBV

Back 36

Heterophile (mono spot) antibody to sheep red cells after absorption with guinea pig sera

Specific EBV tests:

EBV Capsid antigen: IgM at presentation lasts 4-6 weeks; IgG is lifelong

EBV Nuclear Antigen appears in 3-4 weeks, lifelong.

Front 37

INFECTIOUS MONONUCLEOSIS

Back 37

A self limited malignancy EBV infects lymphocytes.

Front 38

2 major findings--physical and lab--usually found in hairy cell leukemia (besides morphology)

Back 38

splenomegaly (can be massive) and pancytopenia

Front 39

Lab tests for confirming Hairy cell leukemia, what antigens?

Back 39

diagnosis confirmed by TRAP, or by flow cytometry
CD5 negative
CD 19 +
CD 20 +
CD 11b +

Front 40

Therapy of hairy cell leukemia

Back 40

2 CDA or 2-DCF

Front 41

which immunoglobulins produced in multiple myeloma, by percentage

Back 41

Approximately 60% are IgG kappa or lambda

Approximately 20% are IgA kappa or lambda

Approximately 20% are light chain only, i.e. kappa or lambda
(These patients have no serum monoclonal spike and have only a urine spike)
IgD kappa or lambda, IgE kappa or lambda, and non-secretory myeloma are RARE.

Front 42

Clinical problems of multiple myeloma involve which three organ systems:

Back 42

Lytic bone lesions: pain or fractures

Decreased normal globulins (and therefore bacterial infections)

Renal disease due to light chains plugging up renal tubules.

Front 43

Lab features of multiple myeloma

Back 43

1Normochromic normocytic anemia

Anemia of chronic disease (renal disease and malignancy)

Myelophthisic anemia

2) 80% of cases show a monoclonal spike on serum protein electrophoresis

Front 44

Marrow of multiple myeloma

Back 44

Marrow: Sheets of plasma cells, at least one-half of the marrow

Front 45

WALDENSTROM’S MACROGLOBULINEMIA

Back 45

Clonal disorder of plasmacytoid lymphocytes associated with IgM production.

Front 46

major clinical manifestation of waldenstrom's macroglobulinemia

Back 46

hyperviscosity

Front 47

symptoms of hyperviscosity in waldenstrom's macroglobulinemia, 5

Back 47

Hyperviscosity:
1 Weakness
2 fatigue
3 blurred vision
4 peripheral neuropathy
5 tinnitus

Front 48

how high of an IgM in waldenstrom's macroglobulinemia will produce symptoms of hyperviscosity

Back 48

IgM of 3000

Front 49

blood smear of waldenstrom's macroglobulinemia

Back 49

Normochromic normocytic smear. Rouleaux formation if IgM is extremely high.

Front 50

Marrow in waldenstrom's macroglobulinemia

Back 50

Marrow shows increase in plasmacytoid lymphs

Front 51

MONOCLONAL GAMMOPATHY OF UNKNOWN SIGNIFICNACE

(previously called benign monoclonal gammopathy)

Back 51

A monoclonal serum spike, or rarely monoclonal urine light chains, without proof of myeloma, i.e. the bone marrow contains elevated plasma cells but not enough for a diagnosis and there are no lytic lesions.

Front 52

When trying to decide between myeloma and MGUS, 3 steps (pre-marrow)

Back 52

1 An IgG > 3 (k?) or an IgA >2 suggests myeloma.

2 Decreased levels of the other immunoglobulins, i.e. decreased IgA and IgM in cases of elevated IgG, suggest myeloma. In MGUS the “other” globulins are usually normal.

3 Lytic bone lesions, unless due to another process, would establish myeloma.

Front 53

2 most common B-Cell disorders resulting in impaired host defense

Back 53

Chronic lymphocytic leukemia and myeloma

Front 54

Diagnosis of B-cell disorders:

Back 54

Quantitative IgG, IgA, IgM

Front 55

Treatment of B cell disorders

Back 55

Treatment of CLL or myeloma rarely corrects the defect

Immunoglobulin replacement IVIG 200 mg/kg q 4 weeks

Problem with replacing IgA deficient patients – allergic reactions

Treat the infections

Front 56

Early complement deficiency: what infections

Back 56

pyogenic infections

Front 57

Late complement deficiency: what infections

Back 57

neisseria infections

Front 58

name the opportunistic parasites, 3

Back 58

Pneumocystis carinii
toxoplasma gondi
cryptosporidium

Front 59

opportunistic mycobacteria, 2

Back 59

M tuberculosis
M. avium

Front 60

opportunistic viruses, 6

Back 60

1 Cytomegalovirus
2 herpes simplex
3 herpes zoster
4 Epstein-Barr virus
5Papovavirus-JC
6 adenvirus

Front 61

opportunistic fungal infections, 4

Back 61

1 Cryptococcus neoformans
2 histoplasmosis
3 candida, sp,
4 aspergillosis

Front 62

opportunistic bacterial infections, 3

Back 62

Nocardia, legionella, listeria

Front 63

Diagnosis of T cell disorders

Back 63

Quantitive T and B-lymphocytes

Skin test to recall antigens (mumps, TB)