Hematopathology Flashcards
1
Q
blast
A
immature
2
Q
cyt
A
cell
3
Q
dys
A
bad, defective
4
Q
emia
A
blood condition
5
Q
erythro
A
red
6
Q
heme
A
blood
7
Q
leuko
A
white
8
Q
lysis
A
destruction
9
Q
macro
A
large
10
Q
myelo
A
bone marrow
11
Q
osis
A
disease or increase
12
Q
penia
A
deficiency
13
Q
thrombo
A
blood clotting
14
Q
Total WBC…
A
the summation of the five types of WBCs
15
Q
hematocrit is ____x the value of hemoglobin
A
3X
16
Q
The actual count of the cells is the ____ count
A
absolute count
17
Q
What are the “3 branches” of hematopoiesis?
A
thrombopoiesis, erythropoiesis, leukopoiesis
18
Q
What are the two major stem cell lines of hematopoiesis?
A
myeloid stem cell, lymphoid stem cell
19
Q
Thrombopoiesis takes place…
A
inside the bone marrow
20
Q
Platelets have an average life span of…
A
8-9 days
21
Q
Old platelets are destroyed in the…
A
spleen and the liver
22
Q
Erythropoiesis takes place inside the…
A
bone marrow
23
Q
An immature red blood cell is called a…
A
reticulocyte
24
Q
What do reticulocytes require to become a mature erythrocyte?
A
Vitamin B12 and Vitamin B9 (folic acid)
25
What stimulates the production of red blood cells in the bone marrow?
erythropoietin
26
Where is erythropoietin produced?
in the kidneys and the liver
27
What is erythropoietin produced in response to?
low oxygen
28
True or False: WBCs are formed from both the myeloid stem cell line and the lymphoid stem cell line
true
29
Disruption of hematopoiesis can be _____ or _____
malignant or non-malignant
30
What are some disorders of non-malignant hematopoiesis?
```
nutritional deficiencies
autoimmune disorders
infectious etiology
DIC
TTP
Hypersplenism
```
31
What are some disorders of malignant hematopoiesis?
```
leukemia
lymphoma
myeloma
myelodysplastic syndrome
aplastic anemia
```
32
What are some examples of myeloproliferative disorders? (malignant)
```
acute myeloid leukemia
chronic myeloid leukemia
myeloma
essential thrombocythemia
polycythemia vera
myelofibrosis
```
33
What are some examples of lymphoproliferative disorders? (malignant)
acute lymphoblastic leukemia
chronic lymphoblastic leukemia
multiple myeloma
lymphoma
34
Leukemia is neoplastic cells in the...
blood stream
35
lymphoma is neoplastic cells in the ...
lymph system
36
myeloma is neoplastic cells in the...
plasma cells
37
True or False...there can be overlap between leukemia and lymphoma
True- this would be in a case of having blood involvement in lymphoma
38
What are the two major types of leukemia?
acute- composed of blast cells
chronic- composed of more mature precursor cells
(acute is more severe because it is in the original stem cells)
39
CLL
chronic lymphocytic leukemia
40
CML
chronic myeloid leukemia
41
ALL
acute lymphoblastic leukemia
42
AML
acute myeloid leukemia
43
What are some risk factors for leukemia?
radiation exposure
chemotherapy exposure
benzene exposure (second hand smoke, gasoline, in plastics, resins, synthetic fibers, rubber lubricants, dyes, detergents, pesticides)
44
What are the signs and symptoms of leukemia?
leukocytosis- monocytes and lymphocytes
pancytopenia- anemia (pallor, dyspnea, fatigue), neutropenia (infections), thrombocytopenia (excess bleeding or bruising)
hypogammaglobulinemia
bone pain (expansion of bone marrow)
lymphadenopathy
splenomegaly
onset varies...acute is sudden onset of a few months and chronic can be an incidental diagnosis
45
How is the diagnosis of leukemia made?
bone marrow bx
| flow cytometry
46
What is the most common leukemia in adults?
acute myeloid leukemia (AML)
47
True or False...AML is a common form of cancer
False...while AML is the most common type of leukemia in adults, it is overall a rare disease accounting for 1% of all adult cancer deaths in the US
48
What finding is considered to be pathognomonic in AML?
******circulating blasts greater than 20%***** (blasts are immature myeloid cells)
auer rods on pathology
49
What is the prognosis of AML?
technically a curable disease
60-80% of young healthy patients achieve a CR but only 1/3 are ultimately cured
rapidly lethal if left untreated
50
Why do patients with AML have anemia and what can be done for them?
it is a complication of low circulating oxygen, they can be given transfusions
51
Why do patients with AML get infections?
Low normally functioning WBCs
52
Why do patients with AML experience bleeding?
Low normally functioning platelets
53
What are the other names for a stem cell transplant?
Bone marrow transplant
| hematopoietic cell transplantation (HCT)
54
What occurs during a stem cell transplant?
very high dose chemotherapy is administered, killing all normal cells in the bone marrow, including the stem cells. new stem cells are reintroduced and reestablish blood cell production in the bone marrow
55
What are the two types of stem cell transplant?
autologous vs allogenic stem cell transplant
56
What is the most common childhood malignancy? What is the peak incidence?
acute lymphoblastic leukemia (ALL)
| peak incidence between 2-5 years of age
57
What syndrome is there an increased incidence of ALL?
down syndrome
58
Is there thought to be a genetic component to ALL?
yes
59
What does pathology of ALL confirm?
lymphoblasts (lymphoid lineage)
60
Is ALL considered to be a curable disease? What is the prognosis?
Yes, ALL is considered to be a curable disease, with lots of chemo and high risk for complications later in life
five year overall survival is 85%
61
What demographics have a good prognosis with ALL?
hyperdiploid (>50 chromosomes per cell)
2-10 years of age
CD10+ (mutation)
low WBC count
62
What demographics have a poor prognosis with ALL?
```
hypodiploid (less chromosomes per cell)
less than 2 years of age
greater than 10 years of age
male gender
high WBC count (>100,000)
```
63
What is CAR-T therapy?
chimeric antigen receptor - T cells
individual's own T lymphocytes are genetically modified with a gene that encodes a chimeric antigen receptor to direct the patient's own T cells against the leukemic cells
modified ex vivo, infused back into patient, long term remission because t cells are trained
64
What is the downside to CAR-T therapy?
cytokine release syndrome occurs in a high percentage of the patients,
65
What cells undergo uncontrolled proliferation in Chronic Myeloid Leukemia?
maturing granulocytes
66
What is pathognomonic for CML?
Philadelphia chromosome
67
What is the Philadelphia chromosome?
the fusion of 2 genes- BCR on chromosome 22 and ABL1 on chromosome 9 results in an abnormal chromosome 22
68
What are the phases of CML?
CML....accelerated phase...blast crisis
| without treatment it will progress to a terminal condition
69
Outlook for CML
technically curable with a stem cell transplant but stem cell transplant poses significant risk and mortality, oral chemo can manage the disease but are not curative
70
What is the epidemiology of CML?
male predominance
| diagnosed 50 or later
71
In CLL, what kind of cells progressively accumulate?
functionally incompetent lymphocytes
72
What are the other names for CLL?
CLL= chronic lymphocytic leukemia
small lymphocytic lymphoma
non-Hodgkin lymphoma SLL
73
What is the epidemiology of CLL?
male predominant
| mostly diagnosed after age 70
74
What is the cytology in CLL?
blood lymphocyte count >5,000
gingersnap appearance of cells
cells are fragile and break during processing- smudge cells (broken cells)
75
Is CLL curable?
not considered curable but treatable
| stem cell transplant is risky with increased mortality
76
True or false...CLL frequently does not require treatment at the time of diagnosis
true
77
What are the indications for treatment "active disease" in CLL?
progressive marrow failure
progressive or massive splenomegaly
progressive or symptomatic lymphadenopathy
progressive lymphocytosis
constitutional symptoms- fatigue, night sweats, unintentional weight loss, fevers
78
Generally, what are considered "B symptoms" or "constitutional symptoms"?
fever
night sweats
unintentional weight loss
fatigue
79
What are the signs and symptoms of lymphoma?
```
constitutional symptoms
lymphadenopathy
splenomegaly
anemia, thrombocytopenia, neutropenia
hypercalcemia
hyperuricemia
elevated LDH
monoclonal immunoglobulin (M spike) on SPEP
```
80
What is the difference hodgkin lymphoma and nonhodgkin lymphoma?
hodgkin- presence of Reed Sternberg cells
nonhodgkin- absence of Reed Sternberg cells
***
81
What are the two types of non-hodgkin lymphomas?
indolent and aggressive lymphomas
82
Follicular lymphoma- indolent or aggressive?
indolent
83
Diffuse Large B cell lymphoma (DLBCL) - indolent or aggressive?
aggressive
84
marginal zone lymphoma (MALT)- indolent or aggressive?
indolent
85
Burkitt lymphoma- indolent or aggressive?
aggressive
86
lymphoplasmocytic lymphoma-indolent or aggressive?
indolent
87
mantle cell lymphoma- indolent or aggressive?
aggressive
88
small lymphocytic lymphoma- indolent or aggressive?
indolent
89
peripheral t cell lymphomas - indolent or aggressive?
aggressive
90
Male or female predominance/peak incidence for Hodgkin lymphoma?
male
(female predominance for nodular sclerosis Hodgkin lymphoma)
peak incidence 20-50
91
What are the signs and symptoms of Hodgkin lymphoma?
painless cervical adenopathy- most common presenting symptom
1/3 of patients have constitutional symptoms
generalized pruritus in the nodular sclerosis type
pain in lymph nodes with alcohol consumption (classic description)
92
Describe the lymph node involvement in Hodgkin lymphoma
contiguous groups of lymph nodes- amenable to surgical excision
extranodal involvement is rare
mediastinal and neck nodes are most commonly involved
93
What is the neoplastic cell in Hodgkin lymphoma?
Reed Sternberg cell
94
What factors affect prognosis in Hodgkin lymphoma?
worse with mixed cell types
male gender
age over 40
95
What is most common type of lymphoma worldwide?
diffuse large B cell lymphoma
96
What is the epidemiology of DLBCL?
male predominance
| median age and presentation-64
97
What are the sxs of DLBCL?
```
rapidly enlarging symptomatic mass (neck or abdomen most common)
extranodal involvement is common
constitutional symptoms in 30%
elevated LDH in over half
somme associated with EBV infection
```
98
What is the prognosis of DLBCL?
60% present in the advanced stage of the disease
| curable in approx. half of cases with current therapy
99
What is the second most common type of non-hodgkin lymphoma?
follicular lymphoma
100
what are the sxs of follicular lymphoma?
painless peripheral adenopathy (cervical, axillary, inguinal, femoral regions-waxes and wanes but never completely goes away)
involvement of organs other than the lymphatic organs or bone marrow is uncommon
constitutional symptoms and elevated LDH are uncommon
presentation is sometimes related to organ dysfunction secondary to large tumor growth
101
what is the prognosis for follicular lymphoma?
may have waxing and waning symptoms for 5 years before diagnosis
can progress to diffuse large b cell or burkitt lymphoma
102
Which lymphoma is a highly aggressive B cell neoplasm?
burkitt lymphoma
103
burkitt lymphoma comprises ___% of pediatric lymphomas in the US
30%
104
an endemic form of burkitt lymphoma seen in Africa presents with....
jaw or facial bone tumor
105
the immunodeficiency-associated form of burkitt lymphoma is seen in which patient population?
HIV positive patients
106
burkitt lymphoma typically presents as...
an abdominal or retroperitoneal mass
107
What is tumor lysis syndrome?
release of large amounts of potassium, phosphate, and nucleic acids into the systemic circulation, causing hyperuricemia
108
Which lymphoma has a high propensity for tumor lysis syndrome?
burkitt lymphoma
109
Mantle cell lymphoma can involve....
| occasionally presenting as....
any region of the GI tract, occasionally presenting as lymphomatous intestinal polyposis
110
In mantle cell lymphoma, nuclear staining for _____ is present in 95% of cases
cyclin D1
111
What is the median overall survival in modern trials incorporating intensive therapy for mantle cell lymphoma?
8-10 years
112
What is the full name for MALT lymphoma?
extranodal marginal zone lymphoma of mucosa associated lymphoid tissue
113
Marginal zone lymphoma is frequently due to what kind of infections?
chronic gastritis from H. pylori infections
114
what epithelial tissues does MALT lymphoma arise in?
```
stomach*
salivary gland
lung
small bowel
thyroid
ocular adnexa
skin
(bx can be done during upper endoscopy)
```
115
what are other types of marginal zone lymphoma other than MALT?
nodal marginal zone lymphoma
| splenic marginal zone lymphoma
116
lymphoplasmacytic lymphoma is also called...
waldenstrom macroglobulinemia
117
which lymphoma is associated with an IgM monoclonal gammopathy, over-producing immunoglobulin?
lymphoplasmacytic lymphoma
118
At what stage is lymphoplasmacytic lymphoma most often diagnosed?
more than 70% of patients have stage IV disease by virtue of bone marrow involvement at the time of diagnosis
119
in lymphoplasmacytic lymphoma, cryoglobulins produce...
raynaud phenomenon
120
What type of cells are present in lymphoplasmacytic lymphoma?
smudge cells
121
non-hodgkin lymphoma
discontinuous groups of lymph nodes- not amenable to surgical excision for cure
extranodal involvement is possible
spleen is often involved
often disseminated at time of dx
painless lymphadenopathy- neck, inguinal and axillary regions MC
constitutional symptoms in 20%
122
multiple myeloma is the neoplastic proliferation of...
plasma cells producing a monoclonal immunoglobulin
123
in MM, proliferation of plasma cells causes...
skeletal destruction with osteolytic lesions, osteopenia, and/or pathologic fracture
124
what is the epidemiology in MM
older age
| mean age 66
125
sxs of MM
anemia
hypercalcemia
renal dysfunction
increased total serum protein concentration
M-spike (monoclonal protein) on SPEP
Bence-jones protein (light chains from M-protein) in urine *
lytic lesions on skeletal imaging (punched out lesions)
bone pain
pathologic fractures
fatigue
weight loss
126
What are the variations of MM
MGUS- monoclonal gammopathy of unknown significance
smoldering myeloma
myeloma with amyloidosis
127
MM diagnosis
clonal bone marrow plasma cells greater than 10 percent or biopsy-proven bony or soft tissue plasmacytoma
+
presence of related organ or tissue impairment (anemia, hypercalcemia, renal insufficiency, bone lesions, or greater than 60% of plasma cells)
128
What are the lab findings in MM
```
SPEP shows M-spike (monoclonal gammopathy)
elevated immunoglobulin (IgM, IgA, IgG), kappa or lambda, heavy or light chains
```
129
What is the prognosis for MM
without effective therapy, symptomatic patients die within a median of 6 months
non-curable malignancy
stem cell transplant is a treatment option to prolong event free and overall survival
130
myelodysplastic syndrome
heterogenous group of malignant hematopoietic stem cell dysplastic and ineffective blood cell production
reduction in the production of cells or reduction in function of existing cells (anemia, thrombocytopenia, leukopenia)
risk progression to AML increased male prevalence, median age at diagnosis is above 65
prognosis is based on risk- 6-54 month survival from the time of diagnosis
131
describe the process of hemostasis
1. endothelial injury and formation of the platelet plug
2, propagation of the clothing process by the coagulation cascade
3. termination of clotting by antithrombotic control mechanisms
4. removal of the clot by fibrinolysis
132
What are the disorders of hemostasis?
primary and secondary
133
primary disorders of hemostasis
- primary= initial steps in clot formation, which mostly rely on vessel wall and platelet function
- disorders of primary hemostasis often present with mucocutaneous bleeding or petechiae
134
secondary disorders of hemostasis
secondary= the subsequent formation of the fibrin-based clot, which mostly relies on coagulation factors
disorders of secondary hemostasis present with deep tissue hematoma or joint bleeding
135
what are the disorders of primary hemostasis
increased destruction of platelets
decreased production of platelets
platelet dysfunction
136
which disorders of increased destruction of platelets are immune mediated?
immune thrombocytopenic purpura (ITP)
| heparin induced thrombocytopenia (HIT)
137
which disorders of increased destruction of platelets are thrombotic microangiopathy (TMA)
thrombotic thrombocytopenia purpura (TTP)
| hemolytic uremic syndrome (HUS)
138
what is DIC
disseminated intravascular coagulation (increased destruction of platelets)
139
What is immune thrombocytopenic purpura?
antibody mediated destruction of platelets
aka idiopathic thrombocytopenic purpura ITP
can be primary or secondary (autoimmune or infectious)
140
what is the timeline of immune thrombocytopenic purpura?
new diagnosed <3 months
persistent 3-12 months
chronic >12 months
141
sxs of immune thrombocytopenic purpura
petechia, purpura, bleeding
142
What is heparin induced thrombocytopenia
antibody mediated destruction of platelets
acquired with the use of heparin
antibody platelet factor 4 and heparin which triggers thrombosis
143
what are the complications of heparin induced thrombocytopenia
stroke
MI
limb ischemia
DVT
144
what is thrombotic thrombocytopenic purpura
when abnormalities in the vessel wall of arterioles and capillaries lead to microvascular thrombosis
promote platelet adherence and activation without exposing the blood to large amounts of procoagulant substances
pentad- fever, thrombocytopenia, renal failure, neurologic changes, and micoangiopathic anemia (hemolytic)
145
what is hemolytic uremic syndrome?
abnormalities in the vessel wall of arterioles and capillaries that lead to microvascular thrombosis- similar to TTP
complement induced damage to the endothelium from shiga toxin produced by shigella dysenteriae and some kinds of e coli
renal dysfunction is more common than with TTP
146
what is DIC
disseminated intravascular coagulation
dysfunction of platelets and coagulation cascade
activation of clotting and fibrinolytic system
thrombin contain fibrin and platelets
release of tissue factor resulting in damage to endothelial cells
147
what are some causes of DIC
placental abruption, retained fetus, septic abortion, amniotic fluid embolism
sepsis, meningiococcemia and histoplasmosis
carcinomas of pancreas, prostate, and lung
massive trauma
148
what are the secondary disorders of hemostasis
von willebrand disease
hemophilia A
hemophilia B
149
what is the most common inherited bleeding disorder ?
von willebrand disease
150
what is the deficiency in von willebrand disease?
deficiency of von willebrand factor (carrier for factor VIII)
151
most cases of von willebrand disease are autosomal...
dominant
152
sxs of von willebrand disease
```
bleeding in mucous membranes
epistaxis
increased bleeding after trauma
increased bleeding with menorrhagia
elevated bleeding time and PTT
PT is normal
```
153
Which factor has a deficiency in Hemophilia A?
factor VIII
154
what kind of genetic disorder is hemophilia A
X linked recessive
155
sxs of Hemophilia A
hemorrhage after trauma or surgery
hemorrhage into points and soft tissue
intracranial bleeding is MC cause of death
elevated PTT with normal PT and bleeding time
156
what is the deficiency in hemophilia B
deficiency of Factor IX
157
what is a hypercoagulable state?
increased risk of thromboembolism
158
what are some inherited disorders that cause hypercoagulable states?
```
Factor V Leiden mutation
Prothrombin gene mutation
Protein S deficiency
Protein C deficiency
Antithrombin deficiency
```
