Leukocyte Disorders Foundation

Question 1

Neutropenia

Answer 1

neutropenia
secondary infections most often seen with ANC < 500/µL; severe infection with < 100/µL, recurrent infections, had pneumonia 3 times in the last 6 weeks for example

recurrent infections, fever, gingivitis

Question 2

Anemia

Answer 2

anemia
fatigue, dizziness, palpitations, exertional dyspnea, pallor

Question 3

Thromocytopenia

Answer 3

thrombocytopenia
petechiae, ecchymosis, occult and gross blood loss

bleeding

Question 4

What is a bone marrow aspiration and bx

Answer 4

A procedure in which a hole is drilled into the bone to allow for aspiration of the cellular contents of the bone marrow

Question 5

bone Bx indications

Answer 5

diagnosis, staging, and therapeutic monitoring of bone marrow disorders
unexplained elevation or decrease in any hematologic cell line
i.e anemia, leukocytosis
lymphoma, solid tumor
evaluation of iron metabolism & stores when routine testing is inadequate
fever of unknown origin (38.3C)
unexplained splenomegaly

Question 6

bone Bx CI

Answer 6

severe bleeding disorders
hemophilia, Disseminated Intravascular Coagulation (DIC)
thrombocytopenia is not a CI
consider platelet transfusion if plt count <20,000 prior to procedure

Question 7

What do they do with the bone bx after collection

Answer 7

Histology (look at through a microscope)
cytogenetic testing. (see if any genetic abnormalities)
flow-cytometry

Question 8

Where do you perform the bone marrow biopsy AND aspiration

Answer 8

Preferred - posterior iliac crest

Question 9

What if you need just the bone biopsy and not the aspiration

Answer 9

Tibia, sternum
sternum is for 13 yo + between 2nd and 3rd ics
Tibia MC for younger children

Question 10

Where is contraindicated for bone biopsy no matter what?

Answer 10

infection, injury, too much adipose tissue over it

for example cellulitis

Question 11

Tell me what the common lymphoid progenitor line is

Answer 11

Lymphoblast–>
NK, B (then plasma), T

Question 12

Tell me the myeloblast line

Answer 12

Basophils, Eosinophils, Neutrophils, monocyte that turns into both macrophage and dendritic cell

Question 13

What is the Common myeloid progenitor line roughly?

Answer 13

Thrombocyte line, proerythroblast, myeloblast for MEN+B.
Does not include lymphoblast.

Question 14

What does multipotential hematopoiteic stem cell lead to

Answer 14

common lymphoid progenitor and common myeloid progenitor

Question 15

patho of Acute Lymphoblastic leukemia

Answer 15

Lymphoblast cell mutation
-it crowds the bone marrow

Question 16

General patho of what happens to lymphocytes that have no room in bone marrow

Answer 16

They enter other lymphatic tissues -
Testes, ovaries, meninges, thymus, liver, spleen, lymph nodes

It can also go to GI tract and respiratory tract where lots of lymph nodes are, but we can’t palpate them on a PE.

It enlarges those organs/tissues

But bone marrow and thymus are responsible for the initial generation of B and T lymphocytes

Question 17

Leukostasis

Answer 17

Leukostasis (aka hyperleukocytosis) - WBC > 100,000/μL - leads to inadequate circulation- think too much ‘traffic’/WBCs on road so WBC and RBCs can’t get to destination

H-A, AMS, blurred vision, dyspnea, priaprism
-intracranial hemorrhage from too much traffic in capillaries

-risk of ICH at least 1 week after dec. WBC

-mortalities is 40% within 2 days if untreated

-must have end organ damage

-someone can have symptoms of “anemia” like with the pallor, pale conjunctiva, and have normal RBCs. This is actually from Leukostasis. Leukostasis is blocking the roads so that oxygen can’t travel to and from

Question 18

What does pancytopenia mean?

Answer 18

All cell lines are low.
Opposite of polycythemia vera.

Question 19

Function of B cells (4)

Answer 19

• Presents antigens to the T-cells
• Expresses Ig surface receptors to antigens
• Develops into memory B cells = long lasting memory
• Differentiation into plasma cells which can produce a large amount of immunoglobulins

Question 20

Patho of Chronic Lymphocytic Leukemia?

Answer 20

a malignant lymphoid neoplasm that is characterized by the accumulation of long-lived, functionally incompetent, small mature B cells

-dysfunction in the maturation of the B-cell

-results in B-cells that are unable to respond to immunologic stimulation

metaphor is - insurance agents. functionally incompetent and long lived. goverment employees from medicaid.

Question 21

Whats a neoplasm?

Answer 21

A new and abnormal growth that can be either benign or malignant

Question 22

Is Chronic Lymphocytic Leukemia old or new patients

Answer 22

old (50)

Question 23

Acute Myelogenous Leukemia Patho

Answer 23

Myeoloblast replications and does not differentiate into Basophil, Neutrophil, Eosinophil, and Monocyte cell lines.
So you lose those cell lines. (MEN + B)

-in addition the myeloblasts don’t die, they accumulate in lymph tissue and crowd

Question 24

Bone marrow failure clinical presentation

Answer 24

anemia, neutropenia and thrombocytopenia

aka
fatigue, dizziness, palpitations, exertional dyspnea
recurrent infections, fever, gingivitis
petechiae, ecchymosis, bleeding

Question 25

When leukemic cells infiltrate organs - Spleen

Answer 25

LUQ fullness
Early satiety
Splenomegaly

Question 26

When leukemic cells infiltrate organs - CNS

Answer 26

HA, increased ICP,
neuro deficits,
intracranial hemorrhage

Question 27

When leukemic cells infiltrate organs - Liver

Answer 27

RUQ discomfort/fullness
Hepatomegaly

Question 28

Chronic Myeloid Leukemia patho

Answer 28

@ t (9:22)single gene translocation results in a genetic defect known as bcr/abl gene or “Philadelphia chromosome CML”

overproliferation of Basophils, Neutrophils, Eosinophils – is affected
On a CBC, the most abundant is the Neutrophil

Question 29

Multiple Myeloma (MM) Patho

Answer 29

a neoplastic proliferation of plasma (B) cells producing bad antibodies

MGUS comes before the full blown disease