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23 General Pathology > 11/6 - Hematopoietic and Lymphoid System > Flashcards

11/6 - Hematopoietic and Lymphoid System Flashcards

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

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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

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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

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4
Q

imbalances of globin chains in thalassemias cause what

A

Imbalances of globin chains cause hemolysis and impaired erythropoiesis (production of RBC)

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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

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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

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7
Q

beta-thalassemia major

A
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8
Q

beta-thalassemia minor

A
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8
Q

beta-thalassemia intermedia

A
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9
Q

what are normal hemoglobins

A

F (alpha2, gamma2)
A (alpha2, beta2)
A2 (alpha 2, delta 2)

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10
Q

what are abnormal hemoglobins

A

H (beta 4)
Barts (gamma 4)

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11
Q

hemoglobin consists of what

A

iron-containing heme ring and four globin chains: two alpha and two nonalpha

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12
Q

T/F: At birth, HbF accounts for approximately 80% of hemoglobin. HbA accounts for 20 percent

A

TRUE

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13
Q

does transition from HbF to HbA begin before or after birth

A

before birth

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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

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15
Q

silent carrier

A
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16
Q

alpha-thalassemia trait

A
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17
Q

HbH disease

A
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18
Q

hydrops fetalis

A
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19
Q

what chromosome is affected for alpha thalassemia

A

chromosome 16

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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

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21
Q

CHIPMUNK face buzz

A

thalassemia

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22
Q

what can be seen on pano radiograph of patient with thalassemia

A
  1. thin mandibular inferior cortex
  2. enlarged bone marrow spaces
  3. short spiky roots of MANDIBULAR molars
  4. thin lamina dura
  5. indiscernible border of mandibular canal
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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!!

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24
Q

thalassemia complications

A
  1. multiple blood transfusions and continued absorption of intesintal iron causing iron overload
  2. endinocrinopahties
  3. splenomegaly
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25
Q

what are endocrinopathies that can cause thalassemia complications

A

hypogonadism and DM (may occur in adolescents and adults)

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26
Q

what invitably deveops in symptomatic thalassemia and can worsen the anemia and occasionally cause neutropenia and thrombocytopenia

A

splenomegaly

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27
Q

what mechanical traumas cause hemolytic anemias

A
  1. Artificial heart valves
  2. Disseminated intravascular coagulation (DIC)
  3. Hemolytic uremic syndrome
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28
Q

what is a rare life threatening disease-causing destruction of RBC, which can lead to cause of kidney failure? due to what bacteria?

A

hemolytic uremic syndrome (HUS) causing anemia due to E. Coli diarrheal infection

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29
Q

what hemolytic anemias cause infection of red cells

A

malaria

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30
Q

what are the two main mechanisms that malaria infection causes hemolytic anemia

A
  1. non-immune mediate hemolysis
  2. immune mediat ehemolysis
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31
Q

___: 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)

A

Non-immune mediated hemolysis

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32
Q

___: few cases of autoimmune hemolytic anemia in malaria patients are reported; antibodies form against the body’s own ANTIBODIES and destroy them

A

Immune mediated hemolysis

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33
Q

what are anemias of decreased erythropoiesis

A
  1. nutritional deficiences
  2. immune-mediate injury of progenitors
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34
Q

what nutritional deficiencies cause anemias of decreased erythropoisesis

A
  1. iron deficiency anemia
  2. megaloblastic anemia (B12 deficiency anemia and folate deficiency)
  3. pernicious anemia
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35
Q

Caused by bleeding or inadequate iron intake
Results in insufficient hemoglobin synthesis and hypochromatic, microcytic red cells

A

iron deficiency anemia

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36
Q

crucial biologic functions of iron

A
  1. DNA synthesis
  2. cell proliferation
  3. energy production
  4. respiration
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37
Q

how has the human body evolved to conserve iron

A

recycle of iron after breakdown of red cells

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38
Q

absorption of iron is limited to how many mg daily

A

1 to 2 mg daily

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39
Q

Most of the iron needed daily, about 25 mg per day, is provided through what

A

recycling by macrophages that phagocytose senescent erythrocytes

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40
Q

T/F: Iron-deficiency anemia is chronic and frequently`asymptomatic and may often go unnoticed

A

true

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41
Q

non-specific symptoms of iron deficiency anemia

A
  • weakness
  • fatigue, difficulty in concentrating
  • poor work productivity
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42
Q

symptoms of severe iron deficiency anemia in pregnancy

A
  • increased risk of preterm labor
  • low neontal weight
  • increased newborn and maternal mortality
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43
Q

what this:

Caused by deficiency of Vit B12 or folate that leads to inadequate synthesis of thymidine and defective DNA replication

A

megaloblastic anemia

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44
Q

what this:

Results in enlarged abnormal hematopoietic precursors (megaloblasts), ineffective hematopoiesis, macrocytic anemia, and (in most cases) pancytopenia

A

megaloblastic anemia

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45
Q

what is neecssary for DNA synthesis

A

Vit B12 and folate

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46
Q

uncorrected deficiency of VitB12 leads to what

A

demyelinating process as well as anemia

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47
Q

in Vit B12, it initially involves peripheral nerves, but when as disease progresses, it involves the spinal cord. Is this reversible?

A

NO! irreversible

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48
Q

symptoms of vit B12 deficiency resulting in anemia

A
  1. Symmetrical numbness of hands & feet
  2. Cognitive impairment in older individuals may resemble Alzheimer Disease; neuropsychiatric state may develop (megaloblastic madness)
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49
Q

oral manifestation of Vit B deficiency

A

erythema and depapillation of tongue

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50
Q

treatment of B12 deficiency

A
  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
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51
Q

what this:

Malabsorption of Vitamin B12 due to autoimmune gastritis is the most common cause

A

pernicious anemia

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52
Q

where is Vit B12 normally aborbed

A

distal ileum

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53
Q

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

A

prevents binding of Vit B12 to
intrinsic factor (produced by parietal cells of stomach)

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54
Q

who produces intrinsc factor? where

A

parietal cells of stomach

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55
Q

in pernicious anemia,aAutoimmune
gastritis and reduction in gastric
acid preents binding of Vit B12 to intrinsic factor and ___ to __ in the ___

A

subsequent binding of Vit B12- intrinsic factor complex to IF receptors in the terminal ileum

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56
Q

what this:

Diminished dietary intake
Increased demand (pregnancy)
Metabolic problems (alcoholism)
Drug-induced (methotrexate)

A

folate (folic acid) deficiency

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57
Q

does folate (folic acid) deficiency cause demyelination as does B12 deficiency?

A

KNOW!! Deficiency does not cause demyelination as does B12 deficiency

58
Q

what is immune mediated injury of progenitors

A

aplastic anemia

59
Q

what is caused by bone marrow failure (hypocellularity) due to diverse cause

A

aplastic anemia

60
Q

diverse causes causing aplastic anemia

A
  1. Exposure to toxins and radiation
  2. Idiosyncratic reactions to drugs and viruses
  3. Inherited defects in telomerase and DNA repair
61
Q

treatment of aplastic anemia

A

Treatment: bone marrow transplantation or
immunosuppressive therapy

62
Q

oral manifestations of aplastic anemia

A
  1. hemorrhage
  2. candidiasis
  3. infections (viral, fungal, and bacterial)
63
Q

what is abnormally high number of circulating RBCs, usually with a corresponding increase in the hemoglobin level

A

polycythemia

64
Q

is polycythemia relative or absolute?

A

BOTH

relative (when there is hemoconcentration due to decreased plasma volume)

absolute (when there is increase in total red cell mass)

65
Q

types of pathophysiologic classification of polycythemia

A
  1. absolute
  2. primary (low erythropoietin)
  3. secondary (high erythropoietin)
66
Q

types of primary (low erythropropoietin)

A
  • Polycythemia vera
  • Inherited erythropoietin receptor mutations (rare)
67
Q

types of secondary (high erythropoietin)

A
  • Compensatory (lung disease, high-altitude, cyanotic heart disease)
  • Paraneoplastic
  • Erythropoietin secreting tumors (renal cell ca, hepatocellular ca)
    I- nherited defects
68
Q

what has increased marrow production of RBCs, granulocytes and platelets

A

polycythemia vera

69
Q

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

A

polycythemia vera

70
Q

disorders of white blood cells broad categories

A
  1. leukopenias
  2. proliferative disorders
71
Q

what are defined as deficiency leukocytes

A

leukopenias

72
Q

what is an expansion of leukocytes

A

proliferative disorders

73
Q

what is a reduction in number of neutrophils in the blood

A

neutropenia

74
Q

what is a marked reduction in neutrophils; makes individuals susceptivle to batcterial and funal

A

agranulocytosis

75
Q

what is neutropenia pathogenesis caused by

A
  • Inadequate and ineffective granulopoiesis
  • Increased destruction or sequestration of neutrophils in the periphery
76
Q

what is the most common cause of agranulocytosis pathogenesis

A

drug toxicity

77
Q

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

A

agranulocytosis

78
Q

what is this:

Increased number of white blood cells in the blood
It is a common reaction to a variety of
inflammatory states

A

leukocytosis

79
Q

___ leukocytosis seen in acute bacterial infections

A

Neutrophilic

80
Q

___ leukocytosis seen in allergic disorders like asthma, hay fever, allergic skin diseases, parasitic infections

A

Eosinophilic

81
Q

___ seen in chronic infections tuberculosis, Systemic lupus erythematosus

A

Monocytosis

82
Q

___ seen in chronic immunologic stimulation, and viral infections (EBV, Cytomegalovirus)

A

Lymphocytosis

83
Q

infectious mononucleosis (EBV) is type of what

A

lymphocytosis

84
Q

A benign, self-limiting lymphoproliferative
disorder caused by Epstein Barr Virus

A

infectious mononucleosis

85
Q

who gets infectious mono

A

Occurs principally in young adults of upper
socioeconomic classes and higher income
nations

86
Q

what is Characterized by fever, sore throat,
generalized lymphadenopathy,
splenomegaly, and the appearance in the
blood of atypical activated T lymphocytes
(mononucleosis cells)

A

infectious mono

87
Q

what do some mono patients develop

A

hepatitis, meningocephalitis, pneumonitis

88
Q

what organs are infected in infectious mononucleosis

A
  1. spleen
  2. liver
89
Q

what happens to spleen in infectious mononucleosis

A

Spleen is often enlarged and susceptible to rupture if injured, but may also rupture spontaneously

90
Q

what happens to liver in infectious mononucleosis

A

Liver is affected often causing a subclinical hepatitis with abnormal liver function. May resemble other forms of viral hepatitis

91
Q

what are lymphoid neoplasms

A
  1. lymphoma
  2. leukemia
  3. plasma cell tumors
92
Q

what is proliferation of white cells, typically lymphocytes, that usually present as discrete tissue masses

A

lymphomas

93
Q

what is widespread involvement of bone marrow and peripheral blood

A

leukemias

94
Q

which cells are more common neoplasms b or t cell

A

b cell more common than t cell

95
Q

what are 2 main categories of lymphoma

A

hodgkin lymphoma (HL) and non-hodgkin lymphoma (NHL)

96
Q

2/3 of NHL and virtually all HL present as what

A

enlarged nontender lymph nodes

97
Q

remaining NHL present with symptoms are related to what

A

involvement of extranodal sites (skin, stomach or brain)

98
Q

what lymphoma:
Neoplastic proliferation of lymphoid cells
Usually arising in lymph nodes (65%) - Remainder in parenchymal organs or soft tissue sites

A

nonhodgkin lymphoma

99
Q

oral manifestations of NHL present how

A
  1. Intra oral mass
  2. Non healing ulceration with ill-defined irregular margins
  3. Periapical pathology and mimics toothache
100
Q

what can induce oral manigestations of NHL

A

complications of therapy (chemotherapy induced mucositis)

101
Q

burkitt lymphoma is a type of what

A

peripheral B-cell neoplasms

102
Q

hodgkin lymphomafalls under what lymphoid neoplasm category

A

peripheral T-cell and NK-cell neoplasms

103
Q

types of burkitt lymphoma

A
  1. African (endemic) BL
  2. Sporadic (nonendemic) BL
  3. Subset of aggressive lymphomas occurring in individuals infected with HIV
104
Q

all forms of burkitt lymphoma associated with what

A

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
Q

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

A

EBV

106
Q

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
A

endemic BL

107
Q

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)
A

sporadic BL

108
Q

morphology of BL

A
  • 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
Q

what is the most curable forms of non-hodgkin lymphooma

A

burkitt lymphoma

110
Q

A rare monoclonal B-cell lymphoid
neoplasm

A

hodgkin lymphoma HL

111
Q

feature of HL

A
  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
Q

what involves scattered large mononuclear Hodgkin and multinucleated Reed-Sternberg cells on a background of non-neoplastic inflammatory cells

A

HL

113
Q

who presents class reed sternberg cell

A

HL

114
Q

2 distinct categories of HL

A
  1. classical HL
  2. nodular lymphocyte-predominate HL (NLP-HL)
115
Q

subgroups of classical HL

A
  1. Nodular sclerosis (NSHL)
  2. Lymphocyte-rich (LRHL)
  3. Mixed cellularity (MCHL)
  4. Lymphocyte-depleted (LDHL)
116
Q

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

A

HL

117
Q

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
A

HL

118
Q
A
119
Q

Cancers of the blood cells

A

leukemia

120
Q

what does type of leukemia depend on

A

Type of blood cell that becomes cancer
Whether it grows quickly or slowly

121
Q

signs and symptoms of leukemia

A
  1. losing weight without trying
  2. swollen lymph nodes, enalarged liver or spleen
  3. easy bleeding or bruising
  4. petechiae
122
Q

most common leukemia among the adult population

A

acute myeloid leukemia

123
Q

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

A

acute myeloid leukemia (AML)

124
Q

cure rates of AML

A

Up to l 5% in patients older than 60 years
About 40% in patients below 60 years of age

125
Q

is prognosis good or poor in elderly population for AML

A

POOR

126
Q

what is the most common malignancy of childhood

A

acute lymphocytic leukemia

127
Q

survival rate for all acute lymphocytic leukemia (ALL)

A

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
Q

oral manifestations of acute leukemias

A
  1. petechiae - thrombocytopenia
  2. leukemic infilratration of gingiva
129
Q

Classified as a myeloproliferative neoplasm
predominantly composed of proliferating
granulocytes

A

chronic myeloid leukemia

130
Q

what is determined to have the philadelphia chromosome/translocation t(9;22)

A

chronic myeloid leukemia

131
Q

what does chronic myeloid leukemia affect

A

both peripheral blood and bone marrow

132
Q

there is increased incidence of SML in who? what are predisposing risk factors?

A

actomic bomb survivors - risk factors unknown

133
Q

CML is due to fusion of oncoprotein of what

A

BCR-AML I defines CML

134
Q

chroinc and accelerated phase of CML

A

Chronic phase: 4-5 years
Accelerated phase 6-18: months

135
Q

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

A

CML

136
Q

T/F: Individuals diagnosed with chronic phase CML
are expected to reach normal or near-normal
life expectancy

A

TRUE

137
Q

___ disorder
characterized by monoclonal B cell
proliferation

A

Chronic lymphoproliferative

138
Q

what is Most common adult leukemia in Western
populations and comprises 25 to 30 percent
of leukemias in the United States

A

CLL

139
Q

what is:

Indolent malignancy (low growing and can
remain stable without treatment for years)
Exact etiology unknow; genetic factors
more than environmental factors
implicated

A

CLL

140
Q

who gets CLL

A

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
Q

in contrast to acute leukemia, oral changes in chronic leukemia is [common OR uncommon]

A

uncommon (may cause bleeding)

142
Q

chemoterapy results in what chronic leukemia oral manifestations

A

Infections with herpes and candida
Mucositis and thrombocytopenia

23 General Pathology (24 decks)

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