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Step 1 > Hem/onc > Flashcards

Hem/onc Flashcards

1
Q

Fetal erythropoeisis

A

yolk sac (3-8 weeks)
Liver (6 weeks - birth)
Spleen (10-28 weeks)
Bone marrow (18 weeks to adult)

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2
Q

Rh hemolytic disease of the newborn

A

Rh- mother, Rh+ fetus
first pregnancy: mother exposed to fetal blood –> mom form anti D IgG
Subsequent pregnancies: anti D IgG crosses the placenta –> attacks fetal RBC –> hemolysis in fetus
presents as hydrops fetalis, jaundice shortly after birth, kernicterus

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3
Q

ABO hemolytic disease of the newborn

A

Type O mom; Type A or B fetus
preexisting maternal anti A or anti B IgG Ab cross placenta –> hemolysis in fetus
Presents as mild jaundice in the neonate within 24 hours of birth. CAN OCCUR IN FIRST BORN and less severe
Tx: phototherapy or exchange transfusion

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4
Q

Neutrophils

A

Acute inflammatory response cells
Numbers increase in bacterial infection
Phagocytic
multilobed nucleus
specific granules contain leukocyte, alkaline phosphatase, collagenase, lysozyme and lactogerrin
Azurophilic granules contain proteases, acid phosphatase, myeloperoxidase and B glucuronidase

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5
Q

Hypersegmented neutrophils

A

vit B12/ folate deficiency

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6
Q

Left shift with increased band cells

A

increase myeloid proliferation (bacterial infection, CML)

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7
Q

Neutrophil chemotaxis

A

C5a, IL8, LTB4, kalikrein, PAF

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8
Q

Erythrocytes

A

Carry O2 to tissues and CO2 to lungs
Anucleate and lack organelles, biconcave with large surface area to volume taio for rapid gas exchange.
Life span of 120 days
USE GLUCOSE

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9
Q

Thrombocytes

A

primary hemostasis
small cytoplasmic fragments derived from megakaryocytes
Contains Ca2+, ADP, 5HT, Histamine and a granules

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10
Q

Monocytes

A

found in blood, differentiate into macrophages in tissues

Large kidney shaped nucleus

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11
Q

Macrophages

A

phagocytose bacteria, cellular debris and senescent RBCs

Activated by y interferon

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12
Q

Eosinophils

A

heminth infection

Produce histamine, major basic protein, eosinophil peroxidase, cationic protein, neurotoxin

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13
Q

Basophils

A

mediate allergic reaction

heparin

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14
Q

Mast cells

A

mediate local tissue allergic reactions
Can bind IgE to membrane
histamine, heparin, tryptase and eosinophil chemotactic factors
Type 1 hypersensitivity

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15
Q

Dendritic cells

A

APC

MHC II and Fc receptors

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16
Q

Lymphocytes

A

B, T and NK cells

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17
Q

Natural killer cells

A 
innate immunity (Intracellular pathogens) 
perforin and granzymes
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18
Q

B cells

A

in follicles of LN, white pulp of spleen, unencapsulated lymphoid tissued
HUMORAL IMMUNITY

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19
Q

T cells

A

mediate CELLULAR IMMUNE RESPONSE

mature in thymus

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20
Q

Plasma cells

A

produce Ab specific to particular antigen

Bone marrow and do not circulate

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21
Q

Thrombogenesis

A

Formation of insoluble fibrin mesh
Aspirin irreversible inhibit COX –> inhibit TXA2 synthesis
Clopidogrel inhibit ADP induced expression of gpIIb/IIIa by irreversibly blocking P2Y12 receptor
Abciximab inhibit gpIIb/IIIa directly
Ristocetin activates vWF to bind gpIb. Failure of aggregation with ristocetin assay occurs in vWd and Bernard Soulier syndrome

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22
Q

Vitamin K deficiency

A

decreased synthesis of Factors 2, 7, 9, 10, protein C and S

Warfarin inhibits vitamin K epoxide reductase. FFP and PCC reverse warfarin actions

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23
Q

Anticoagulation

A

Antithrombin inhibits thrombin and factors 7a, 9a, 10a, 11a, 12a
Heparin enhances the activity of antithrombin

24
Q

Acanthosis

A

Liver Disease, abetalipoproteinemia

Projections of varying sizes at irregular intervals

25
Q

Echinocytes

A

Liver disease, ESRD, pyruvate kinase deficiency

Smaller and more uniform projections

26
Q

Dacrocytes

A

teardrop cells
Bone marrow infiltration
Mechanically squeezed out of bone marrow

27
Q

Schistocytes

A

MAHA, mechanical hemolysis

fragmented RBC

28
Q

Degmacytes

A

G6PD deficiency

removal of heinz bodes by splenic macrophages

29
Q

Elliptocytes

A

Hereditary elliptocytosis

mutation in genes encoding RBC membrane proteins

30
Q

Spherocytes

A

Hereditary spherocytosis, autoimmune hemolytic anemia

small spherical cells without central pallor

31
Q

Macro ovalocytes

A

Megaloblastic anemia

32
Q

Target cells

A

HbC disease, asplenia, liver disease, thalassemia

33
Q

Sickle cell

A

Sickle cell anemia

sickling occurs with low O2 conditions

34
Q

Iron granules

A

Sideroblastic anemias
perinuclear mitochondria with excess iron
REQUIRE PRUSSIAN BLUE STAIN

35
Q

Howell Jolly Bodies

A

functional hyposplenia, asplenia
Basophilic nuclear remnants
removed by splenic macrophages usually

36
Q

Basophilic stippling

A

sideroblastic anemia, thalessemia

37
Q

Pappenheimer bodies

A

sideroblastic anemia

basophilic granules

38
Q

Heinz bodies

A

G6PD deficiency

denatured and precipitated Hb

39
Q

Microcytic, hypochromic anemias

A 
Iron deficiency
a thalassemia
B thalassemia
Lead poisoning
Sideroblastic anemia
40
Q

Iron Deficiency Anemia

A

via chronic bleeding, malnutrition, absorption disorders, , GI surgery or increase demand –> low final step in heme synthesis
Low Fe, high TBC, low ferritin, high free erythrocytic protoporphyrin, high RDW, low RI
Sx: fatigue, pallor, PICA, spoon nails
glossitis, cheilosis, Plummer Vinson

41
Q

a thalassemia

A

a globin gene deletion on Chromosome 16 –> low a globin synthesis
More deletion –> more microcytic hypochromic anemia
3 deletions –> B4 (HbH)
4 deletions –> y4- hydrops fetalis

42
Q

B thalassemia

A

point mutation in splice sites and promoter sequences on chr 11 –> decrase B globin synthesis

43
Q

B thalassemia minor

A

B chain is underproduced
Asymptomatic
Dx via high HbA2 on electrophoresis

44
Q

B thalassemia major

A

B chain absent –> severe microcytic hypochromic anemia with target cells
Marrow expansion –> skeletal deformities. Extramedullary hematopoiesis –> hepatosplenomegaly
Increase risk of parvovirus B19 aplastic crisis

45
Q

HbS/ B thalassemia heterozygote

A

mild to moderate sickle cell disease

46
Q

Lead poisoning

A

Inhibits ferrochelatase and ALA dehydratase –> low heme synthesis and high RBC protophyrin
inhibits rRNA degradation –> RBCs retain aggregates of rRNA
Lead lines gingivae and metaphyses of long bones on Xray, encephalopathy and erythrocyte basophilic stippling, ab colic and sideroblastic anemia, wrist and foot drop
Tx Dimercaprol and EDTA

47
Q

Sideroblastic anemia

A

X linked defect in ALA synthase gene, myeloblastic syndrome and alcohol, lead poisoning, drugs
High iron, normal TIBC, high ferritin
ringed sideroblasts in bone marrow.
Tx pyridoxine

48
Q

Megaloblastic anemias

A

impaired DNA synthesis –> maturation of nucleus of precursor cells in bone marrow delayed relative to maturation of cytoplasm.
Causes vit B12 deficiency, folate deficiency, meds

49
Q

Folate Deficiency

A

malnutrition, malabsorption, drugs, high requirement
Increased homocysteine, normal methylmalonic acid
NO NEURO

50
Q

Vit B 12 deficiency

A

pernicious anemia, malabsorption, pancreatic insufficiency, gastrectomy, insufficient intake
High homocysteine, methylmalonic acid
NEURO: reversible dementia
Folate correct anemia but worsens neuro

51
Q

Orotic aciduria

A

inability to convert orotic acid to UMP because defect in UMP synthase.
AR
failure to thrive, developmental delay and megaloblastic anemia.

52
Q

Nonmegaloblastic anemia

A

macrocytic anemia in which DNA synthesis is normal
Alcoholism and liver disease
NO HYPERSEGMENTED NEUTROPHILS

53
Q

Diamond Blackfan

A

Congenital pure red cell aplasia
rapid onset anemia within first year of life due to intrinsic defect in erythroid progenitor cells
short, craniofacial abnormality, UE malformation

54
Q

Intravascular hemolysis

A

low haptoglobin
high schistocytes
mechanical hemolysis, paraxysmal nocturnal hemoglobinuria, MAHA

55
Q

Extravascular hemolysis

A

macrophages in spleen clear RBCs
spherocytes
no hemoglobinuria, hemosideriuria
can present with urobilinogen in urine

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