Quiz 3 Flashcards
Formation of blood cellular components
hematopoiesis
pg 4
Where does hematopoiesis occur in children?
tibia and femur
pg 4
Where does hematopoiesis occur in adults?
pelvis, cranium, vertebral bodies, sternum, ribs
pg 4
Formation of blood cellular components outside of the medullary cavity
extramedullary hematopoiesis
pg 4
Where does extramedullary hematopoiesis occur?
spleen, liver, lymph nodes, thymus
pg 4
Categories of Anemia
1) blood loss
2) increased RBC destruction
3) decreased RBC production
pg 8
Effects of hypoxia from anemia
increased erythropoietin from kidneys, pallor, fatigue, weakness
pg 9
Anemia due to trauma, GI or gynecologic issues
anemia of blood loss
pg 10
Anemia of blood loss is ____(hypo, normo, or hyper -chromic/cytic)
normocytic/normochromic
pg 10
Anemia of blood loss may lead to ____ shock
hypovolemic shock
pg 10
Anemia due to the decreased life span of RBC
hemolytic anemia
pg 11
Decreased life span of RBC
hemolysis
pg 11
2 types of defects resulting in hemolytic anemia
intracorpuscular defects
extracorpuscular defects
pg 11
Hereditary defect resulting in hemolytic anemia
intracorpuscular defect
(abnormal membranes or disordered Hb synthesis)
pg 11
Acquired defect resulting in hemolytic anemia
extracorpuscular defect
(RBC trauma, infections, antibodies)
pg 11
Where does intravascular hemolysis occur?
peripheral circulation
pg 12
Where does extravascular hemolysis occur?
liver and spleen
pg 12
Increased Hb in urine
hemoglobinuria
pg 12
Components of intravascular hemolysis
damaged heart valve, toxins or heat; hemoglobinuria; jaundice and gallstones
pg 12
Components of extravascular hemolysis
RBC damage, antibody opsonization; splenomegaly
pg 12
Which is more common, extravascular or intravascular hemolysis?
extravascular hemolysis
pg 12
Clinical term for gallstones
cholelithiasis
pg 14
% of population affected by cholelithiasis?
10-20%
pg 15
Cholelithiasis are more commonly ____(asymptomatic or symptomatic)
asymptomatic 80%
pg 15
Which is more common: cholesterol stones or pigmented stones?
cholesterol stones
pg 15
Risk factors for cholelithiasis
family history, obesity, diabetes, hemolytic anemia, estrogen
pg 16
Abnormally shaped RBCs
poikilocyte
pg 17
Irregularly shaped RBCs, from hemolysis
schistocyte
pg 17
Teardrop shaped RBCs
dacrocyte
pg 17
RBC membrane defect(autosomal dominant)
hereditary spherocytosis
pg 21
Hereditary Spherocytosis may result in ____ ____ which may be made worse by the presence of parvovirus B19
aplastic crisis
inability to make RBCs
pg 21
Beta-globin mutation(autosomal recessive)
sickle cell anemia
pg 24
Sickle cell trait is ____zygous
heterozygous
pg 24
Sickle cell anemia/disease is ____zygous
homozygous
pg 24
Sickle cell trait effects what % of African Americans?
8%
pg 24
In what areas do RBCs sickle in those with sickle cell anemia?
sites of blood stasis; bone marrow(MC), spleen, site of infection or inflammation, and/or during dehydration
pg 25
Which has a worse prognoses; heterozygous or homozygous sickle cell?
homozygous; 50% survive beyond 5th decade
pg 27
Lethal results of sickle cell anemia
1) acute chest syndrome
2) stroke
pg 27
Effects of sickle cell anemia
thrombosis, fever, malaise, chronic low-level pain, splenomegaly/infarction, gallstones, priapism, stunted growth, osseous distortion
pg 28
Possible H-shaped vertebra are characteristic of which disease?
Sickle cell anemia (10% of patients)
pg 31
Hemoglobinopathy of mutated alpha or beta-globin genes
Thalassemia
pg 33
Hemoglobinopathy decreasing beta-globin chains
Beta-thalassemia
ph 34
Beta-thalassemia with 1 allele mutation
minor
pg 34
Beta-thalassemia with 2 allele mutation
major
pg 34
Which beta-thalassemia presents with subtle hemolysis?
minor
pg 34
Which beta-thalassemia presents with severe hemolysis, severe anemia, extramedullary hematopoiesis, and splenomegaly?
major
pg 34
What is monitored in those with beta-thalassemia minor?
serum ferritin
pg 35
Treatment for those with beta-thalassemia major
repeated transfusions and iron chelation
or bone marrow transplant (high risk)
pg 36
Life expectance for those with beta-thalassemia major
~20 years
pg 36
Eventually cause of death for those treated for beta-thalassemia major
iron overload, hemochromatosis, lethal dilated cardiomyopathy
pg 36
Radiological findings in those with beta-thalassemia major
“hair on end” or “lace-like trabeculation”
pg 37
A notable facies of beta-thalassemia major
chipmunk facies
pg 38
Hemoglobinopathy with mutated alpha-globin gene
alpha-thalassemia
pg 41
Which is more severe: alpha or beta-thalassemia?
beta-thalassemia
pg 41
What determines the severity of alpha-thalassemia?
# of altered genes pg 41
1 deletion of alpha-globin
silent carrier of alpha–thalassemia
pg 42
4 deletions of alpha-globins
no O2 capacity, lethal hydrops fetalis
pg 42
Deficiency results in a decrease in GSH
Glucose-6-phosphate dehydrogenase deficiency
pg 43
GDPD is typically asymptomatic until exposed to…
1) infections
2) fava beans
3) ADRs
pg 45
Symptoms/signs of G6PD
acute onset, fatigue, pallor, splenomegaly, back/abdominal pain, hemosiderinuria
pg 47
Risks of G6PD
males, African descent, areas of endemic malaria
pg 47
Microscopic findings of G6PD
bite cells and Heinz bodies
pg 46
Dark urine upon waking along with an acquired PIGA gene mutation
paroxysmal nocturnal hemoglobinuria
pg 48
PIGA gene mutation results in…
deactivated complement inhibitors
pg 49
Features of paroxysmal nocturnal hemoglobinuria
complement-mediated hemolysis, mild/chronic low-level anemia, increased risk for venous thrombosis
pg 49
Treatment for paroxysmal nocturnal hemoglobinuria
antibodies that inhibit MAC, marrow transplant(risky)
pg 49
Repetitive physical trauma to RBCs
traumatic hemolysis
pg 51
Causes of traumatic hemolysis
prosthetic heart valves, narrowing of vessels (microangiopathic hemolytic anemia)
pg 51
Schistocytes
“burr cells”
“helmet cells”
pg 52
Disease caused by plasmodium falciparum
malaria
pg 54
MC area affected by malaria
Asia and Africa
pg 54
“Blackwater fever”
dark urine associated with malaria
pg 54
Features of malaria
episodic shaking, chills, fever, vomiting sweating jaundice, splenomegaly, HA, joint pain, hemoglobinuria, renal failure
pg 55
Malaria with CNS involvement
cerebral malaria
pg 55
Additional features of cerebral malaria
seizures convulsions, coma/death (within days)
pg 55
Treatment for malaria
chloroquine, primaquine, chemotherapy
pg 55
Anemias of diminished erythropoiesis
1) iron deficiency anemia
2) megaloblastic anemias
3) anemia of chronic disease
4) aplastic anemia
5) myelopththisic anemia
pg 56
World’s MC nutritional deficiency and cause of anemia
iron deficiency
pg 57
____(men or women) store more iron
Men
pg 57
Where is iron stored in the body?
liver, spleen, marrow, skeletal muscle
pg 57
Hb makes up __% of body’s iron
80%
pg 57
Iron is transported by…
transferrin (serum ferritin)
pg 57
Conditions creating poor bioavailability of iron
decrease in dietary intake, chronic blood loss (GI or gynecological), malabsorption, increased metabolic demands
pg 58
Features of iron deficiency anemia
fatigue, pale skin, lack of energy, impaired cognition, decreased immunity, fingernail “spooning”, pica
pg 59
Appetite for substances of non-nutritive value
pica
pg 59
A craving for ice is indicative of what deficiency?
iron
pg 59
MC cause of iron deficiency in the US
chronic blood loss
pg 60
Deficiency in folate or vitamin B12 results in what type of anemia?
megaloblastic anemia
pg 61
Folate and vitamin B12 are required for…
DNA synthesis
pg 61
Features of folate deficiency anemia
fatigue, weakness, sore tongue, macrocytes in peripheral blood
pg 62
Vitamin B12 is needed for…
DNA synthesis and maintenance of PNS and spinal cord
pg 63
MC cause of vitamin B12 deficiency anemia
chronic malabsorption
pg 63
Features of vitamin B12 deficiency anemia
fatigue, pallor, weakness, dyspnea, demyelination of PNS and CNS, numbness, tingling, burning, decreased proprioception, ataxia
pg 64
Treatment of vitamin B12 deficiency anemia
injected vitamin B12
pg 64
Anemia with inflammation decreasing erythropoiesis
anemia of chronic disease
pg 65
Anemia of chronic disease is MC among what population?
hospitalized
(infections, autoimmunity, cancers)
pg 65
Features of anemia of chronic disease
similar to iron deficiency anemia + decreased iron-binding capacity and increased iron storage in marrow
pg 65
Anemia with suppression of myeloid stem cells, “bone marrow failure”, pancytopenia
aplastic anemia
pg 67
Features of aplastic anemia
anemia (weakness, pallor, dyspnea), Thrombocytopenia(petechiae), granulocytopenia (infections)
pg 68/69
In which anemia is splenomegaly “characteristically absent”?
aplastic anemia
pg 69
Treatment of aplastic anemia
immunosuppressive meds, regular transfusions, marrow transplant (risky)
pg 69
Anemia with extensive marrow infiltration
myelophthisic anemia
pg 70
MC cause of myelophthisic anemia
metastasis to bone (lung breast, prostate)
pg 70
Causes of myelophthisic anemia
met to bone, granulomatous disease, lipid-storage disease
pg 70
Features of myelophthisic anemia
anemia, thrombocytopenia, dacrocytes
pg 70
Treatment for myelophthisic anemia
marrow transplant
pg 70
Where are leukocytes produced?
marrow
pg 71
Leukocytes make up __% of total blood volume
1%
pg 71
Increase in WBCs
leukocytosis
pg 71
Decrease in WBCs
leukopenia
pg 71
Granulocytes
neutrophils, eosinophils, basophils
pg 72
Agranulocytes
monocytes, lymphocytes
pg 72
Categories of WBCs disorders
1) reactive
2) neoplastic
pg 73
Decrease in granulocytes
neutropenia <500 cell/microL
pg 74
SEVERE neutropenia
agranulocytosis <100/microL
pg 74
Infection causes by Epstein-Barr virus (HHV-4)
infectious mononucleosis
pg 76
Features of infectious mononucleosis
reactive lymphocytosis, fever, pharyngitis, fatigue, splenomegaly and generalized lymphadenitis
pg 76
Microscopic finding of infectious mononucleosis
“atypical lymphocytes”
pg 77
Infectious mononucleosis is most commonly spread via…
saliva
pg 78
Disease caused by bartonella henselae
cat-scratch disease
pg 80
MC population affected by cat-scratch disease
pediatrics (90%)
pg 80
Features of cat-scratch disease
cervical and axillary lymphadenopathy, splenomegaly, pharyngitis, malaise, anorexia, irregular stellate necrotizing granulomas
pg 80
Neoplastic proliferations of WBCs
1) lymphoid neoplasms
2) myeloid neoplasms
3) histiocytic neoplasms
pg 81
Classifications of lymphoid neoplasms
1) leukemia
2) lymphoma
pg 84
WBC cancer involving marrow or blood
leukemia
pg 84
WBC cancer involving lymphatic tissue
lymphoma
pg 84
Sudden onset with abnormal lymphoblasts suppressing marrow
acute leukemia
pg 85
Categories of acute leukemia
1) acute lymphoblastic leukemia
2) acute myeloid leukemia
pg 85
MC presenting feature of acute leukemia
anemia
pg 85
Aggressive cancer of lymphoblasts, arrested maturation of B cells or T cells
acute lymphoblastic leukemia
pg 88
MC pediatric leukemia
acute lymphblastic leukemia (80%)
pg 88
Treatment of acute lymphblastic leukemia
chemotherapy
80% cure rate
pg 88
Cancer of B cells, MC leukemia of adulthood
chronic lymphocytic leukemia
pg 89
MC age of diagnosis of chronic lymphocytic leukemia
60 years
pg 89
Features of chronic lymphocytic leukemia
pancytopenia, anemia/fatigue. cachexia, generalized lymphadenopathy, hepatosplenomegaly, suppression of B cells
pg 90
Prognosis of chronic lymphocytic leukemia
4-6 years
if it becomes aggressive ~1 year
pg 90
B cell cancer with a nodular appearance
Follicular lymphoma
pg 91
Lymphocytes with cleaved nuclei
centrocytes
pg 91
Centrocytes are associated with which B cell cancer
follicular lymphoma
pg 91
Follicular lymphoma makes up what % of non-Hodgkin’s lymphoma?
40%
pg 91
Features of follicular lymphoma
painless generalized lymphadenopathy, adults >50years
pg 92
40% of cases of follicular lymphoma progress into…
diffuse B cell lymphoma
pg 93
t(14;18), BCL2 gene is associated with which B cell cancer
follicular lymphoma
pg 93
Cancer of B cells with t(11;14)
mantle cell lymphoma
pg 94
Population affected by mantle cell lymphoma
males, >50 years
pg 94
Cancer of B cells, MC lymphoma of adulthood
diffuse large B cell lymphoma
pg 95
Population affected by diffuse large B cell lymphoma
elderly, 60 years
pg 95
Risks for diffuse large B cell lymphoma
EBV infection, immunosuppression
pg 95
Features of diffuse large B cell lymphoma
aggressive, rapidly-enlarging mass(es), commonly extranodally
pg 96
Treatment of diffuse large B cell lymphoma
high-dose chemotherapy
(~80% enter remission, 50% cured)
pg 96
Cancer of B cells, classically affect African children, “starry ski” histology”
Burkitt lymphoma
pg 97
Risks for Burkitt lymphoma
EBV infection, mutated MYC gene via t(8;14)
pg 97
Population MC affected by Burkitt lymphoma
Africans
pg 99
Fastest growing human tumor
Burkitt lymphoma
pg 99
Burkitt lymphoma MC develops…
extranodally
Africa- maxilla and mandible
USA- abdomen, GI, ovaries
pg 99
Prognosis of Burkitt lymphoma
great with aggressive chemotherapy
pg 99
