White Blood Cell Disorders Flashcards
What is the CD molecule for hematopoietic stem cells?
CD34
What is a normal leukocyte count?
5,000 - 10,000
What class of drugs can cause leukopenia?
alkylating agents
What cell type is the most susceptible during leukopenia?
neutrophils
Can gram-negative or gram-positive sepsis result in leukopenia?
gram-negative
What drug is used to treat leukopenia?
GM-CSG or G-CSF
What type of cell is most susceptible to radiation?
lymphocyte
What genetic disease can cause lymphopenia?
DiGeorge
What do corticosteroids do to lymphocytes?
induce apoptosis
What are the two causes of leukocytosis?
bacterial infection
tissue necrosis
What CD molecule if the Fc receptor?
CD16
Immature neutrophils are deficient in what molecule?
CD16 (Fc receptor)
What are the four downstream cells types of myeloid cells?
Megakaryoblast, Erythroblast, Monoblast, Myeloblast
What are the three types of myeloblasts?
neutrophils, eosinophils and basophils
What do steroids do to neutrophils? Why?
neutrophilia
releases marginated pool of neutrophils
What do steroids do to eosinophils? Why?
eosinopenia
sequestions eosinophils in lymph nodes
What do steroids do to lymphocytes? Why?
lymphopenia
induce apoptosis of lymphocytes
What two disease states can cause monocytosis?
chronic inflammation
malignancy
What type of Neoplasia can cause eosinophilia?
Hodgkin Lymphoma
What three cell types can increase during CML?
neutrophils, metemyelocytes, basophils
What type of lymphocyte can cause hyperplasia in response to virally infected cells?
CD8+
Which bacteria produces lymphocytosis promoting factor? How does this toxin work?
Bordatella pertussis
prevents lymphocytes from leaving the blood
What does lymphocytosis promoting factor do?
prevent lymphocytes from leaving the blood stream to enter the lymph nodes
What two viral infections can cause infectious mononucleosis?
EBV and CMV
What sort of leukocytosis can occur during Infectious Mononucleosis?
Lymphocytic Leukocytosis
What type of lymphocytes increase in the blood during Lymphocytic Leukoytosis?
CD8+
What three body parts can be infected during mono?
oropharynx
Liver
B-cells
Where in the lymph nodes do T-cells hypertrophy during mononucleosis?
Paracortex
What section of the spleen do T-cells hypertrophy during mononucleosis?
Periarterial Lymphatic Sheath (PALS)
Where in the lymph nodes do B-cells hypertrophy?
cortex
Lymphocytic leukocytosis features what type of CD8+ cell?
reactive
The monospot test detects what specifc type of immunoglobulin? What cell type can these AB’s bind?
heterophile IgM
animal RBCs
A negative monospot test is indicative of what viral infection?
CMV
A definitive test for EBV is detection of the presence of what?
EBV viral capsid antigen
What is the largest complication of EBV?
splenic rupture
A person with Infectious Mononucleosis should not be treated with what drug? Why?
Ampicillin
rash
What type of cancer can develop in patients with dormant EBV?
B-cell lymphoma
What cell organelle is abnormally found during an acute leukemia? What cell type? What does it look like?
nucleolus
immature blasts
punched out
What blast expresses TdT? What two specific cells? Where in the cell is TdT located?
lymphoblasts
pre-B cells and pre-T cells
nucleus
What is more common, B-ALL or T-ALL?
B-ALL
What three CD molecules are often found on cancerous cells in B-ALL?
CD10, CD19 and CD20
What ALL translocation has a good prognosis and is often seen in children?
t(12:21)
What ALL translocation has a poor prognosis and is often seen in adults?
t(9:22)
Is CD10 seen on pre-B or pre-T cells?
pre-B
What range of CD molecules would T-ALL present with?
CD2 - CD8
What enzyme is a classic marker for myeloblasts? What structure can this marker crystallize into?
Myeloperoxidase
auer rods
What type of enzyme is TdT?
DNA polymerase
Where in the body does T-ALL usually form a mass?
mediastinum
What is the named for aggregates of MPO?
Auer rods
Are Auer rods seen in myeloblastic leukemia or lymphoblastic leukemia?
Myeloblastic
What translocation is most often seen in Acute Promyelocytic Leukemia? What gene is involved? What move?
t(15:17)
retinoic acid receptor from 17 to 15
WHat does retinoic acid receptor translocation prevent during Acute Promyelocytic Leukemia?
maturation of promyelocytes
What hematological abnormality can AML produce? Why? Which type?
DIC
promyelocytes can release primary granules
M3
What is the specific treatment for acute promyelocytic leukemia?
all-trans retinoic acid
How is acute monoblastic leukemia differentiated from acute myeloblastic leukemia?
monoblastic = lack myeloperoxidase
What part of the body does acute monoblastic leukemia often invade?
gums
What type of leukemia develops in pts with Downs Syndrome AFTER the age of 5?
ALL
What specific type of leukemia develops in pts with Downs Syndrome BEFORE the age of 5? After 5?
acute megakaryoblastic leukemia
ALL
Previous exposure to what two agents can result in AML?
alkylating agents or radiation
What is the difference between acute and chronic leukemias?
acute = neoplastic proliferation of immature cells
chronic = neoplastic proliferation of mature cells
In general, does CML effect younger or older individuals?
older
What are the two CD markers for CLL? Are these B-cells or T-cells?
CD5 and CD20
B-cells
What type of leukemia are Smudge Cells seen?
CLL
What is it called when CLL invades the lymph nodes?
Small Lymphocytic Lymphoma
What is the most common cause of death in CLL? Why?
infection
hypogammaglobulinemia
Hairy Cell Leukemia is a neoplastic proliferation of what type of cell?
Mature B-cells
What stain is used to identify Hairy Cell Leukemia?
TRAP
Where in the body do ‘Hairy Cells’ accumulate? Causing?
red pulp of spleen
splenomegaly
What type of leukemia produces bone marrow fibrosis?
Hairy Cell
What is the Tx of Hairy Cell Leukemia? What is the MOA of this drug?
cladribine
purine analogue inhibitor of adenosine deaminase
Does Adult T-cell Leukemia/Lymphoma feature a proliferation of CD4+ or CD8+ T-cells? Naive or mature?
CD4+
mature
What causes Adult T-cell Leukemia/Lymphoma? What two locations?
HTLV1
Japan and Caribbean
Does ATLL have hyer or hypo calcemia?
hyper
Mycosis fungoides features a proliferation of what cell type? Naive or mature?
CD4+
mature
What layer of the skin receives infiltrates during Mycosis Fungoides? What are these infiltrates called? What type of cells?
epidermis
Pautrier microabscesses
Neoplastic Tcells
What disease is it called if Mycosis Fungoides invades the blood?
Sezary Syndrome
What is the buzz word for what the neoplastic cells look like during Sezary Syndrome? Resembling?
cerebriform nuclei
brain
What metabolite can expand during a Myeloproliferative Disorder?
uric acid
What is Chronic Myeloid Leukemia?
neoplastic proliferation of mature myeloid cells
What cell type especially proliferates during CML? What specific cell?
granulocytes
basophils
What fusion protein drives CML?
Bcr-Abl
What is the first-line treatment of CML? What enzymatic activity does this treatment block?
imatinib
tyrosine kinase
Splenomegaly during CML suggests what?
transformation to AML or ALL
What test differentiates CML from a leukomoid rxn?
CML will not have increase leukocyte alkaline phos.
Leukomoid rxn will
What type of cell is present in a CML that is not present in a leukomoid rxn?
basophil
What three cell types increase during polycythemia vera?
RBCs
granulocytes (NEBM)
platelets
What type of mutation is present during PV?
JAK2 kinase
What is Budd-Chiari Syndrome? What condition most often causes Budd-Chiari Syndrome?
occlusion of hepatic veins leading to liver infarct
polycythemia vera
What is the characteristic sign of PV? Why does this happen?
itching after warm bathing
degranulation of mast cells release histamine
What drug is used to treat PV?
hydroxyurea
Does Polycythemia Vera have a high or low level of Epo?
low Epo
What type of neoplasia can produce high levels of Epo?
Renal Cell Carcinoma
Essential thrombocytopenia is caused by a mutation in what?
JAK2
Does Essentil thrombocytosis have an increased or decreased risk of bleeding? Why?
increased
platelets sequester too many clotting factors
Myelofibrosis features an over proliferation of what cell type? What cytokine is produced in excess that drives myelofibrosis?
megakaryocytes
PDGF
What mutation is present during myelofibrosis?
JAK2
What cytokine drives myelofibrosis?
PDGF
Why does splenomegaly occur during myelofibrosis?
extra-medullary hematopoiesis
Which lymphocyte lives in the follicle of a lymph node?
follicle = B-cell
What two (non-neoplastic) diseases can produce follicular hyperplasia?
Rheumatoid
HIV early stages
What type of lymphocyte lives in the paracortex? What type of infection would drive paracortex hyperplasia?
T-cell
viral
Hyperplasia of sinus histiocytes is seen during what disease?
lymph node draining a cancerous region
Which lymphoma spreads intranodally?
Hodgkins
Which lymphoma spreads extra-nodally?
non-Hodgkins
Which CD molecule is the key marker for B cells?
CD20
What translocation drives follicular cell lymphoma?
t(14:18)
Regarding follicular cell lymphoma, what is present on 14? 18?
14 = Ig heavy chain
18 = bcl-2
What is the function of bcl-2?
inhibit apoptosis
What is the Tx for follicular cell lymphoma? What molecule does rituximab attack?
Rituximab
CD20
If a patient with Follicular Cell Lymphoma begins to present with an enlarging lymph node, what disease should one suspect?
Diffure large B-cell lymphoma
What translocation drives mantle cell lymphoma?
t(11:14)
Regarding mantle cell lymphoma, what is on chromosome 14? Chromosome 18?
11 = cyclin D1
14 = Ig heavy chain
Which cell cycle transition is activated during mantle cell lymphoma?
G1/S
Marginal Zone lymphoma is associated with what three diseases?
H. pylori infection
Hashimoto’s Thyroiditis
Sjogren syndrome
What virus is Burkitt’s Lymphoma associated with?
EBV
What translocation is present in Burkitt’s Lymphoma? What protein is over-produced?
t(8:14)
c-Myc
What is the key phrase for the histological appearance of Burkitts Lymphoma?
Starry Sky
What is the most common type of NHL? What is the prognosis of this disease?
Diffuse large B-cell lymphoma
poor
Which CD molecules are present on Reed-Sternberg cells?
CD15 and CD30
Why do reactive inflammatory cells make up the bulk of Hodgkin Lymphoma cells?
RS cells secretet cytokines that attract them
What are the four types of HL?
Nodular sclerosing
Lymphocyte rich
Mixed cellularity
Lymphocyte depleted
What is the most common type of HL?
Nodular Sclerosing
What two locations does nodular sclerosis often present?
cervical lymph node
mediastinal lymph node
What sex does nodular sclerosis most often present? Age?
female
teenager
Which type of Hodgkins Lymphoma has the best prognosis?
Lymphocyte rich
What interleukin can Reed Sternberg cells produce? What granulocyte does this attract?
IL5
eosinophil
What type of Hodgkins lymphome is the most aggressive? What two population groups is this most often found?
lymphocyte depleted
HIV+ pt’s and the elderly
What type of cell proliferates during multiple myeloma? Where is this cell found?
Plasma
bone marrow
Which interleukin can drive Multiple Myeloma?
IL-6
What disease is associated with CRAB? What does CRAB stand for?
CRAB = multiple myeloma
elevated Calcium
real insufficiency
Anemia
bone lesions
Neoplastic plasma cells of Multiple Myeloma can activate with receptor?
RANK
What two locations of the skeleton are most susceptible to bone lesions during multiple myeloma?
skull and vertebrae
What two immunoglobulin isotypes are most often found during multiple myeloma M-spike?
IgA and IgG
What is the most common cause of death in multiple myeloma? Why?
infection
neoplastic plasma cells only produce monoclonal Ab;s
Do neoplastic plasma cells overproduce light chain or heavy chain? What can this lead to?
light chain
amyloidosis
Bence Jones protein is indicative of what disease? Where is it found? What composes it?
Multiple Myeloma
urine
Ig light chain
What is the only similarity between Multiple Myeloma and MGUS?
M-spike
What Ig isotype is produced during Waldenstroms Macroglobulinemia?
IgM
What two systems are primarily effected during Waldenstroms Macroglobulinemia? Such as?
vision and CNS
retinal damage and stroke
Does Waldenstroms Macroglobulinemia have an increased or decreased liklihood of bleeding? Why?
increased
viscous serum results in defective aggregation
What cells are Langerhans cells derived from? What type of cells are langerhans cells?
monocytes
dendritic cells
What type of protein is found in the urine of pt’s with Langerhans Cell Histiocytosis?
Birbeck granules
What are the three immunohistochemical markers of Langerhans Cell Histiocytosis?
CD1a and S100 and CD207
Proliferation of what type of cell is seen during Letterer-Siwe disease?
Langerhans cells
What is the classic presentation of a patient with Letterer-Siwe Syndrome? What age group?
Skin rash and cystic skeletal lesions
Infant
What cell type proliferates during an Eosinophilic Granuloma? Where?
Langerhans
Bone
What cell type proliferates during Hand-Schuler-Christian Disease?
Langerhans
What is the triad of Hand-Schuler-Christian Disease?
diabetes insipidus
lytic bone lesions
exopthalmos
What does a high cortisol state due to neutrophil numbers? Why?
increases
causes neutrophils to be released from marginated pool (lungs)
Basophilis is clasically associated with what disease?
CML
What are the two main causes of lymphocytic leukocytosis?
Viral infection
Bordatella Pertussis Infection
Why does splenomegaly occur during mononucleosis?
T-cell hyperplasia in PALS
Infectious mononucleosis features what specific type of T-cell?
reactive CD8+ T-cell
Acute leukemia is defined as greater than what percent of blasts in the bone marrow?
20%
What two sites require ancillary chemotherapy during ALL? Why?
scrotum and CSF
BTB and BBB
Where does T-ALL usually manifest? What age group?
thymus
teenagers
What is another name for a T-ALL located in the thymus?
Acute Lymphoblastic Lymphoma
How is acute monocytic leukemia differentiated from acute promyelocytic leukemia?
monocytic leukemia = no myeloproxidase
Where does Acute Monoblastic Leukemia clasically infiltrate?
gums
Do megakaryocytes have MPO?
no
What are the three major complications of CLL?
Hypogammaglobulinemia
AI hemolytic anemia
Transformation into diffuse large B-cell lymphoma
What is the transformation of CLL to Diffuse large B-cell lymphoma characterized by?
growing lymph node
What are the three common features of Hairy Cell Leukemia?
Splenomegaly
Bone Marrow fibrosis (dry tap)
no lymphadenopathy
What cause the splenomegaly in Hairy Cell Leukemia?
hairy cells accumulating in red pulp of spleen
Lytic bone lesions is a knee-jerk rxn for what disease? Lytic bone lesions with a rash is indicative of what disease?
multiple myeloma
Human T-cell leukemia/lymphoma
During a myeloproliferative disorder, which cells increase in number?
all cell types increase in number
What is the definition of a myeloproliferative disorder?
neoplastic proliferation of mature cells of the myeloid lineage
What is a common complication of all myeloproliferative disorders?
hyperuricemia
What two states can a myeloproliferative disorder progress to?
marrow fibrosis
acute leukemia
What is the accelerated phase of CML marked by?
splenomegaly
What are the three physical symptoms of polycythemia vera?
blurry vision and headache
flushed face
itching after bathing
Other than RBCs, what other cell often expands in number during polycythemia vera that can cotribute to its diagnosis?
mast cells
What are the two treatments of polycythemia vera?
- Phlebotomy
2. Hydroxyurea
What myelodysplastic syndrome does not have an increased risk of hyperuricemia?
Essential thrombocythemia
Why does myelofibrosis result in extramedullary hematopoiesis?
no bone marrow for hematopoiesis
What protein prevents immature blood cells from leaving the bone marrow? Is this protein present in the spleen?
reticulin
no
What non-metastatic and non-infective disorder can cause follicular lymphadenopathy?
Rheumatoid Arthritis
What virus is known to cause follicular lymphadenopathy?
HIV
Where is the lymph node do sinus histiocytes live?
medulla
What is a lymphoma?
neoplastic proliferation of lymphoid cells that form a mass
When does follicular lymphoma usually present?
late adulthood
What does a follicular lymphoma look like histologically?
dozens of follicles
What can a follicular lymphoma transform into?
Diffuse Large B-cell Lymphoma
Where in the lymph node do the follicles live?
cortex
What is the difference between follicular cell lymphoma and follicular hyperplasia?
lymphoma = follicles all over cell instead of just cortex
Which form, hyperplasia or lymphoma, results with tingible body macrophages?
hyperplasia due to infection
Which is monoclonal, follicular lymphoma or follicular hyperplasia?
lymphoma
What is the proliferation of kappa to lambda light chain in non-neoplastic B-cells?
3:1
When does mantle cell lymphoma present?
late adult hood
What condition produces a marginal zone of a lymph node?
chronic inflammation
What is the most distinguishing characteristic of a Reed-Sternberg cell?
prominent nucleoli
Why is a mass produced during Hodgkin lymphoma?
RS cells secrete cytokines that attract other cells
Why does Hodgkin Lymphoma present with typical B-cell symptoms?
RS secretion of cytokines
What is the specific cell type found in HL Nodular Sclerosis?
Lacunar cell
What type of HL produces IL5? What cell type does this attract
mixed-cellularity
eosinophil
Is the M-spike of multiple myeloma caused by monoclonal or polyclonal Ab’s?
Monoclonal
What causes a Rouleaux formation? What disease?
high protein in serum decreases charge between RBCs
Multiple Myeloma
What protein deposits in the urine during Multiple Myeloma? Composed of?
bence jones
Ig light chains
What age group is MGUS most prevalent?
elderly
What type of specific neoplasia is Waldenstroms Macroglobulinemia?
B-cell lymphoma
Does Waldenstroms Macroglobulinemia present with lytic bone lesions?
no
Does Waldenstroms Macroglobulinemia have an M-spike?
yes
Why does Waldenstroms Macroglobulinemia present with CNS complications?
high IgM makes blood hyper-viscous
What is the specific function of Langerhans cells?
present antigen to naive T-cells
Is eosinophilic granuloma benign or pathological?
benign
What is the classic presentation for a patient with eosinophilic granuloma?
pathological bone fracture in adolescent
What type of aggressive neoplasm can follicular lymphoma transform into?
diffuse large B-cell lymphoma
Letterer-Siwe Disease is a malignant proliferation of what cell?
Langerhans cell
What is the classic presentation of Hand Schuler Christian Disease?
scalp rash and lytic bone defects
Do high cortisol levels cause eosinophilia or eosinopenia?
eosinopenia
Do megakaryocytes express MPO?
no
Are B-cells in CLL naive or mature?
Naive
Are myeloproliferative disorders an expansion of mature or immature cells?
mature
Does an Eosinophilic Granuloma involve a skin rash?
no
What virus can cause a nasopharyngeal carcinoma?
EBV
Where does EBV maintain dormancy?
B-cells
Which form of ALL usually presents in teenagers?
T-ALL
What is the average age of development of AML?
50-60
How can PV be distinguished from Essential Thrombocytosis?
PV runs a risk or hyperuricemia
ET does not
Which form of HL causes the lacunar type of RS cell?
Nodular Sclerosing
What two forms of Hodgkins Lymphoma is EBV infection associated with?
mixed-cellularity
lymphocyte depleted
How is Waldenstroms Treated?
plasmapheresis
What is the difference between an Eosinophilic Granuloma and Letterer-Siwe Disease?
Eosiniphilic granuloma doesnt involve a rash
Which cell is most often found in an Eosinophilic Granuloma?
Eosinophils
Greater than what percent of blasts have to be present in the blood to Dx an Acute Leukemia?
20%
What are the two translocations of ALL?
12: 21
9: 22
Which translocation of ALL has a poor prognosis?
9;22
Which translocation of ALL has a good prognosis?
12:21
What is a Richter Transformation?
CLL into large B-cell lymphoma
Is lymphocytic leukocytosis caused by viruses or bacteria?
virus
Which two leukemias can CML transform into?
AML or ALL
Where is the mantle located in the lymph node?
immediately adjacent to the follicle
