11/6 - Hematopoietic and Lymphoid System Flashcards
(143 cards)
1
Q
Thalassemias are a group of [inherited OR noninherited] caused by defects in the synthesis of one or more of the hemoglobin chains
A
inherited hematologic disorders
2
Q
___ thalassemia is caused by reduced or absent
synthesis of alpha globin chains
___ thalassemia is caused by reduced or absent
synthesis of beta globin chains
A
alpha, beta
3
Q
Genetically heterogenous disorders caused by germline mutation that decreases the synthesis of either a-globin or ß-globin, leading to anemia, tissue hypoxia, and red cell hemolysis related to imbalance in globin chain synthesis
A
thalassemia
4
Q
imbalances of globin chains in thalassemias cause what
A
Imbalances of globin chains cause hemolysis and impaired erythropoiesis (production of RBC)
5
Q
describe alpha and beta chains in thalassemia
A
The 2 a-chains of HbA are encoded by an identical pair of a-globin genes on chromosome 16
The 2 ß-chains are encoded by a single ß- globin gene on chromosome 11
6
Q
impaired beta-globin synthesis results in anemia by what mechanisms
A
The deficit of HbA (adult hemoglobin) synthesis produces “underhemoglobinized” hypochromic, microcytic red cells with subnormal oxygen transport capacity
Diminished survival of RBCs and their precursors, which results from imbalance in the a- and ß-globin synthesis
7
Q
beta-thalassemia major
A
8
Q
beta-thalassemia minor
A
8
Q
beta-thalassemia intermedia
A
9
Q
what are normal hemoglobins
A
F (alpha2, gamma2)
A (alpha2, beta2)
A2 (alpha 2, delta 2)
10
Q
what are abnormal hemoglobins
A
H (beta 4)
Barts (gamma 4)
11
Q
hemoglobin consists of what
A
iron-containing heme ring and four globin chains: two alpha and two nonalpha
12
Q
T/F: At birth, HbF accounts for approximately 80% of hemoglobin. HbA accounts for 20 percent
A
TRUE
13
Q
does transition from HbF to HbA begin before or after birth
A
before birth
14
Q
by what age do healthy infants transition to mostly HbA, a small amount of HbA2 and negligible HbF
A
six months of age
15
Q
silent carrier
A
16
Q
alpha-thalassemia trait
A
17
Q
HbH disease
A
18
Q
hydrops fetalis
A
19
Q
what chromosome is affected for alpha thalassemia
A
chromosome 16
20
Q
what disease:
- Spiky shaped and short roots
- Taurodontism
- Attenuated lamina dura
- Enlarged bone marrow spaces
- Small maxillary sinuses
- Class Il malocclusion: short mandible, a reduced posterior facial height -Chipmunk faces (Narrow maxilla, smaller incisors widths)
- Higher caries but no increase in gingivitis or periodontitis
A
thalassemia
21
Q
CHIPMUNK face buzz
A
thalassemia
22
Q
what can be seen on pano radiograph of patient with thalassemia
A
- thin mandibular inferior cortex
- enlarged bone marrow spaces
- short spiky roots of MANDIBULAR molars
- thin lamina dura
- indiscernible border of mandibular canal
23
Q
In severe cases of ___ and ___, the loss of the outer table of the calvarium results in perpendicular trabeculae radiating outward from the inner table, in response to the intracranial pressure, giving a “hair-on-end” appearance
A
Sickle Cell Anemia and thalassemia
BOARD!!
24
thalassemia complications
1. multiple blood transfusions and continued absorption of intesintal iron causing ***iron overload***
2. endinocrinopahties
3. splenomegaly
25
what are endocrinopathies that can cause thalassemia complications
hypogonadism and DM (may occur in adolescents and adults)
26
what invitably deveops in symptomatic thalassemia and can worsen the anemia and occasionally cause neutropenia and thrombocytopenia
splenomegaly
27
what mechanical traumas cause hemolytic anemias
1. Artificial heart valves
2. Disseminated intravascular coagulation (DIC)
3. Hemolytic uremic syndrome
28
what is a rare life threatening disease-causing destruction of RBC, which can lead to cause of kidney failure? due to what bacteria?
hemolytic uremic syndrome (HUS) causing anemia due to E. Coli diarrheal infection
29
what hemolytic anemias cause infection of red cells
malaria
30
what are the two main mechanisms that malaria infection causes hemolytic anemia
1. non-immune mediate hemolysis
2. immune mediat ehemolysis
31
___: direct destruction of the red blood cells (RBCs) by the PARASITE, and depends on the type of malaria and the percentage of parasitemia (at the end of infection cycle, the blood cells rupture)
Non-immune mediated hemolysis
32
___: few cases of autoimmune hemolytic anemia in malaria patients are reported; antibodies form against the body's own ANTIBODIES and destroy them
Immune mediated hemolysis
33
what are anemias of decreased erythropoiesis
1. nutritional deficiences
2. immune-mediate injury of progenitors
34
what nutritional deficiencies cause anemias of decreased erythropoisesis
1. iron deficiency anemia
2. megaloblastic anemia (B12 deficiency anemia and folate deficiency)
3. pernicious anemia
35
Caused by bleeding or inadequate iron intake
Results in insufficient hemoglobin synthesis and hypochromatic, microcytic red cells
iron deficiency anemia
36
crucial biologic functions of iron
1. DNA synthesis
2. cell proliferation
3. energy production
4. respiration
37
how has the human body evolved to conserve iron
recycle of iron after breakdown of red cells
38
absorption of iron is limited to how many mg daily
1 to 2 mg daily
39
Most of the iron needed daily, about 25 mg per day, is provided through what
recycling by macrophages that phagocytose senescent erythrocytes
40
T/F: Iron-deficiency anemia is chronic and frequently`asymptomatic and may often go unnoticed
true
41
non-specific symptoms of iron deficiency anemia
- weakness
- fatigue, difficulty in concentrating
- poor work productivity
42
symptoms of severe iron deficiency anemia in pregnancy
- increased risk of preterm labor
- low neontal weight
- increased newborn and maternal mortality
43
what this:
Caused by deficiency of Vit B12 or folate that leads to inadequate synthesis of thymidine and defective DNA replication
megaloblastic anemia
44
what this:
Results in enlarged abnormal hematopoietic precursors (megaloblasts), ineffective hematopoiesis, macrocytic anemia, and (in most cases) pancytopenia
megaloblastic anemia
45
what is neecssary for DNA synthesis
Vit B12 and folate
46
uncorrected deficiency of VitB12 leads to what
demyelinating process as well as anemia
47
in Vit B12, it initially involves peripheral nerves, but when as disease progresses, it involves the spinal cord. Is this reversible?
NO! irreversible
48
symptoms of vit B12 deficiency resulting in anemia
1. ***Symmetrical numbness of hands & feet***
2. ***Cognitive impairment*** in older individuals may resemble Alzheimer Disease; neuropsychiatric state may develop (***megaloblastic madness***)
49
oral manifestation of Vit B deficiency
erythema and depapillation of tongue
50
treatment of B12 deficiency
1. initial: parenteral injections of Vitamin B12
2. when large abnormalities: large daily does of Vit B12 can be used since 1% of oral dose is aborbed without need for intrinsic factor binding
51
what this:
Malabsorption of Vitamin B12 due to autoimmune gastritis is the most common cause
pernicious anemia
52
where is Vit B12 normally aborbed
distal ileum
53
in pernicious anemia,aAutoimmune gastritis and reduction in gastric acid ___ to ___ and subsequent binding of Vit B12- intrinsic factor complex to IF receptors in the terminal ileum
prevents binding of Vit B12 to
intrinsic factor (produced by parietal cells of stomach)
54
who produces intrinsc factor? where
parietal cells of stomach
55
in pernicious anemia,aAutoimmune
gastritis and reduction in gastric
acid preents binding of Vit B12 to intrinsic factor and ___ to __ in the ___
subsequent binding of Vit B12- intrinsic factor complex to IF receptors in the terminal ileum
56
what this:
Diminished dietary intake
Increased demand (pregnancy)
Metabolic problems (alcoholism)
Drug-induced (methotrexate)
folate (folic acid) deficiency
57
does folate (folic acid) deficiency cause demyelination as does B12 deficiency?
KNOW!! Deficiency does not cause demyelination as does B12 deficiency
58
what is immune mediated injury of progenitors
aplastic anemia
59
what is caused by bone marrow failure (hypocellularity) due to diverse cause
aplastic anemia
60
diverse causes causing aplastic anemia
1. Exposure to toxins and radiation
2. Idiosyncratic reactions to drugs and viruses
3. Inherited defects in telomerase and DNA repair
61
treatment of aplastic anemia
Treatment: bone marrow transplantation or
immunosuppressive therapy
62
oral manifestations of aplastic anemia
1. hemorrhage
2. candidiasis
3. infections (viral, fungal, and bacterial)
63
what is abnormally high number of circulating RBCs, usually with a corresponding increase in the hemoglobin level
polycythemia
64
is polycythemia relative or absolute?
BOTH
relative (when there is hemoconcentration due to decreased plasma volume)
absolute (when there is increase in total red cell mass)
65
types of pathophysiologic classification of polycythemia
1. absolute
2. primary (low erythropoietin)
3. secondary (high erythropoietin)
66
types of primary (low erythropropoietin)
- Polycythemia vera
- Inherited erythropoietin receptor mutations (rare)
67
types of secondary (high erythropoietin)
- Compensatory (lung disease, high-altitude, cyanotic heart disease)
- Paraneoplastic
- Erythropoietin secreting tumors (renal cell ca, hepatocellular ca)
I- nherited defects
68
what has increased marrow production of RBCs, granulocytes and platelets
polycythemia vera
69
what this
The transformed progenitor cells have markedly reduced requirement for erythropoietin and other hematopoietic growth factors due to activating mutations in the tyrosine kinase JAK2
polycythemia vera
70
disorders of white blood cells broad categories
1. leukopenias
2. proliferative disorders
71
what are defined as deficiency leukocytes
leukopenias
72
what is an expansion of leukocytes
proliferative disorders
73
what is a reduction in number of neutrophils in the blood
neutropenia
74
what is a marked reduction in neutrophils; makes individuals susceptivle to batcterial and funal
agranulocytosis
75
what is neutropenia pathogenesis caused by
- Inadequate and ineffective granulopoiesis
- Increased destruction or sequestration of neutrophils in the periphery
76
what is the most common cause of agranulocytosis pathogenesis
drug toxicity
77
what this:
Signs and symptoms related to infections
Malaise, chills, and fever, followed by marked weakness and fragility
Decrease in neutrophil counts (chemotherapy/myelodysplasia) may be associated with painful mouth ulcerations
Antibiotic prophylaxis for a neutropenic patient reduces bacterial oral infections
agranulocytosis
78
what is this:
Increased number of white blood cells in the blood
It is a common reaction to a variety of
inflammatory states
leukocytosis
79
___ leukocytosis seen in acute bacterial infections
Neutrophilic
80
___ leukocytosis seen in allergic disorders like asthma, hay fever, allergic skin diseases, parasitic infections
Eosinophilic
81
___ seen in chronic infections tuberculosis, Systemic lupus erythematosus
Monocytosis
82
___ seen in chronic immunologic stimulation, and viral infections (EBV, Cytomegalovirus)
Lymphocytosis
83
infectious mononucleosis (EBV) is type of what
lymphocytosis
84
A benign, self-limiting lymphoproliferative
disorder caused by Epstein Barr Virus
infectious mononucleosis
85
who gets infectious mono
Occurs principally in young adults of upper
socioeconomic classes and higher income
nations
86
what is Characterized by fever, sore throat,
generalized lymphadenopathy,
splenomegaly, and the appearance in the
blood of atypical activated T lymphocytes
(mononucleosis cells)
infectious mono
87
what do some mono patients develop
hepatitis, meningocephalitis, pneumonitis
88
what organs are infected in infectious mononucleosis
1. spleen
2. liver
89
what happens to spleen in infectious mononucleosis
Spleen is often enlarged and susceptible to rupture if injured, but may also rupture spontaneously
90
what happens to liver in infectious mononucleosis
Liver is affected often causing a subclinical hepatitis with abnormal liver function. May resemble other forms of viral hepatitis
91
what are lymphoid neoplasms
1. lymphoma
2. leukemia
3. plasma cell tumors
92
what is proliferation of white cells, typically lymphocytes, that usually present as discrete tissue masses
lymphomas
93
what is widespread involvement of bone marrow and peripheral blood
leukemias
94
which cells are more common neoplasms b or t cell
b cell more common than t cell
95
what are 2 main categories of lymphoma
hodgkin lymphoma (HL) and non-hodgkin lymphoma (NHL)
96
2/3 of NHL and virtually all HL present as what
enlarged nontender lymph nodes
97
remaining NHL present with symptoms are related to what
involvement of extranodal sites (skin, stomach or brain)
98
what lymphoma:
Neoplastic proliferation of lymphoid cells
Usually arising in lymph nodes (65%) - Remainder in parenchymal organs or soft tissue sites
nonhodgkin lymphoma
99
oral manifestations of NHL present how
1. Intra oral mass
2. Non healing ulceration with ill-defined irregular margins
3. Periapical pathology and mimics toothache
100
what can induce oral manigestations of NHL
complications of therapy (chemotherapy induced mucositis)
101
burkitt lymphoma is a type of what
peripheral B-cell neoplasms
102
hodgkin lymphomafalls under what lymphoid neoplasm category
peripheral T-cell and NK-cell neoplasms
103
types of burkitt lymphoma
1. African (endemic) BL
2. Sporadic (nonendemic) BL
3. Subset of aggressive lymphomas occurring in individuals infected with HIV
104
all forms of burkitt lymphoma associated with what
All forms of BL are associated with
translocation of the MYC
gene on
chromosome 8 that leads to increased
MYC protein levels t(8;14)
105
Essentially all endemic BL are latently infected with ___, which is also present in about 25% of all HIV-associated tumors and 15% to 20 % of sporadic cases
EBV
106
what type of BL:
- Seen in parts of Africa
- Often presents with massive involvement of maxilla and mandible
- Common in children aged 4 to 7 who have malaria and Epstein-Barr virus
endemic BL
107
what type of BL:
- Seen worldwide
- Involves the abdomen area
- 60% over the age of 40 years; 40% children (Account for -2% of adult lymphoma cases)
sporadic BL
108
morphology of BL
- tissues effaced by diffuse infiltrate of intermediated sized lymphoid cells
- high mitotic index and contains numerous apoptotis cells, the nuclear remnants of which are phagocytosed by interspersed benigh macrophages
- STARRY SKY PATTERN
109
what is the most curable forms of non-hodgkin lymphooma
burkitt lymphoma
110
A rare monoclonal B-cell lymphoid
neoplasm
hodgkin lymphoma HL
111
feature of HL
1. Usually presents in young adults
2. Commonly arises in cervical lymph nodes
3. Involves scattered large mononuclear Hodgkin and multinucleated Reed-Sternberg cells on a background of non-neoplastic inflammatory cells
4. Characteristic neoplastic cells are often surrounded by T lymphocytes
112
what involves scattered large mononuclear Hodgkin and multinucleated Reed-Sternberg cells on a background of non-neoplastic inflammatory cells
HL
113
who presents class reed sternberg cell
HL
114
2 distinct categories of HL
1. classical HL
2. nodular lymphocyte-predominate HL (NLP-HL)
115
subgroups of classical HL
1. Nodular sclerosis (NSHL)
2. Lymphocyte-rich (LRHL)
3. Mixed cellularity (MCHL)
4. Lymphocyte-depleted (LDHL)
116
what this:
It has a bimodal distribution
Most of the affected patients are between ages 20 to 40 years
There is another peak from age 55
years and older
> males
HL
117
what this:
- Patients treated with radiation may have dry mouth if submandibular and sublingual glands are in the field
- Need prophylactic fluoride gel, varnish or toothpaste to prevent dental caries
HL
118
119
Cancers of the blood cells
leukemia
120
what does type of leukemia depend on
Type of blood cell that becomes cancer
Whether it grows quickly or slowly
121
signs and symptoms of leukemia
1. losing weight without trying
2. swollen lymph nodes, enalarged liver or spleen
3. easy bleeding or bruising
4. petechiae
122
most common leukemia among the adult population
acute myeloid leukemia
123
what is characterized by clonal expansion of immature "blast cells" in the peripheral blood and bone marrow resulting in ineffective erythropoiesis and bone marrow failure
acute myeloid leukemia (AML)
124
cure rates of AML
Up to l 5% in patients older than 60 years
About 40% in patients below 60 years of age
125
is prognosis good or poor in elderly population for AML
POOR
126
what is the most common malignancy of childhood
acute lymphocytic leukemia
127
survival rate for all acute lymphocytic leukemia (ALL)
under age of 18. peak age diagnosis between 2-10 years of age
The 5-year survival rate for people aged
20 and older is 40%
The 5-year survival rate for people under
age 20 is 89%
128
oral manifestations of acute leukemias
1. petechiae - thrombocytopenia
2. leukemic infilratration of gingiva
129
Classified as a myeloproliferative neoplasm
predominantly composed of proliferating
granulocytes
chronic myeloid leukemia
130
what is determined to have the philadelphia chromosome/translocation t(9;22)
chronic myeloid leukemia
131
what does chronic myeloid leukemia affect
both peripheral blood and bone marrow
132
there is increased incidence of SML in who? what are predisposing risk factors?
actomic bomb survivors - risk factors unknown
133
CML is due to fusion of oncoprotein of what
BCR-AML I defines CML
134
chroinc and accelerated phase of CML
Chronic phase: 4-5 years
Accelerated phase 6-18: months
135
what this:
May terminate as "myeloid blast crisis"
A new oral drug «Gleevec" has proven
to be a dramatic step forward in CML
treatment
Bone marrow transplantation effective
CML
136
T/F: Individuals diagnosed with chronic phase CML
are expected to reach normal or near-normal
life expectancy
TRUE
137
___ disorder
characterized by monoclonal B cell
proliferation
Chronic lymphoproliferative
138
what is Most common adult leukemia in Western
populations and comprises 25 to 30 percent
of leukemias in the United States
CLL
139
what is:
Indolent malignancy (low growing and can
remain stable without treatment for years)
Exact etiology unknow; genetic factors
more than environmental factors
implicated
CLL
140
who gets CLL
Mostly seen in adults with an average age of
70 years
Can affect adults as young
as 30 years of age
Extremely rare in children
The survival period could range from 2 to 20 years with a nedian survival of 10
years
A more aggressive expression of CLL is seen in untreated patients
141
in contrast to acute leukemia, oral changes in chronic leukemia is [common OR uncommon]
uncommon (may cause bleeding)
142
chemoterapy results in what chronic leukemia oral manifestations
Infections with herpes and candida
Mucositis and thrombocytopenia
