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Flashcards in Robbins CH 14 RBCs Deck (86):
1

What does basophilic stippling of RBCs suggest?

A marrow injury--drug/toxin

2

Hypochromic RBCs are seen with what 2 disorders?

Iron deficiency and Thalassemias

Associated with reduced Hb synthesis

3

What do Schistocytes suggests?

Microangiopathic hemolytic anemia--shock/sepsis

4

What is seen with acute and chronic blood loss?

Reticulocytosis and marrow hyperplasia

Marrow responding to a decrease in RBCs

5

Very hypo cellular marrow and unable to respond to anemia--associated with pancytopenia

Aplastic marrow

6

What does the Coombs test, test for?

Testing for autoimmune hemolytic anemia

7

Impairs the ability of the marrow to mount a significant and sustained reticulocytosis. Typically microcytic and hypo chromic

Iron deficiency anemia

8

What is haptoglobin

Serum protein that binds to free Hb

9

How can SLE cause decreased haptoglobin levels?

SLE can result in hemolysis which causes a decrease in haptoglobin

10

What does an elevated D-dimer suggest?

microangiopathic hemolytic anemia

11

A condition in which a mutation affects one of several membrane cytoskeletal proteins--important in maintaining RBCs shape (spectrin, ankyrin, band 4.2 and 3, Protein 4.1, glycophorin A). The RBCs lack central pallor on peripheral blood smear...

Hereditary spherocytosis

12

What is suggested by the lack of central pallor on blood smear of RBCs?

Hereditary spherocytosis

13

What disease may lead to an aplastic crisis precipitated by a parvovirus infection?

Hereditary spheocytosis

14

What is the treatment of hereditary spheocytosis?

Splenectomy--beneficial because the spherocytes are no longer detained by the spleen

15

What are the 3 characteristics of disseminated intravascular coagulation?

Gives rise to thrombocytopenia
Bleeding
Appearance of fragmented RBCs on blood smear

16

B-thalassemia major causes a-globin chain aggregation causing apoptosis. This leads to anemia and iron overload. What can the iron overload cause?

Results in hemochromatosis with infiltrative cardiomyopathy, hepatic cirrhosis, and bronze diabetes from pancreatic islet dysfunction

17

Why are maxillofacial deformities seen with B-thalassemia major?

Severe anemia--increases EPO production which leads to the expansion of the marrow

18

What group of individuals has the highest incidence of developing B-thalassemia major?

patients from mediterranean descent

19

How can sickle cell anemia lead to a risk for infection with encapsulated bacterial organisms?

Cumulative ischemic damage to the spleen results in autosplenectomy -- impaired splenic function and resultant inability to clear bacteria from the bloodstream can occur in childhood

20

What individuals are most likely to have alpha-thalassemia major?

southeast Asian ancestry

21

What is alpha-thalaseemia major?

Complete lack of a-globin chains precludes formation of Hb A1, A2 and F

Only tetramer of gamma chains (Bart's Hb) en be made--severe fetal anemia

22

Sensitivity to oxidizing agents causing a hemolytic anemia--X-linked disorder that affects about 10% of african-american males

G6PD deficiency

23

What are the drugs that subject Hb to damage by oxidants seen with G6PD deficiency?

Primaquine
Sulfonamides
Nitrofurantioin
Phenacetin
Aspirin

24

What are bite cells?

Result from the attempts of overeager splenic macrophages to pluck out the heinz bodies--addingto the hemolysis seen with G6PD deficiency

25

Abnormal HFE gene...

seen in hereditary hemochromatosis--iron overload

26

What is the mechanism of action of hydroxyurea therapy given for sickle cell anemia?

increases the concentration of HbF in RBCs

Interferes with polymerization of HbS

27

How is B-thalassemia minor and iron deficiency anemia differentiated since both are characterized by hypo chromic and microcytic RBCs?

With iron deficiency anemia there is no increase in HbA2 and there would a a decreased ferritin level as well

28

Anemia of chronic disease is associated with an increase in____.

serum ferritin

29

Disorder that results from an acquired stem cell membrane defect produced by a PIGA gene mutation that prevents the membrane expression of certain proteins that require a glycolipid anchor?

Paroxysmal nocturnal hemoglobinuria (PNH)

30

Complement distraction of RBCs, Granulocytes, and Platelets. Patient presents with hemoglobinuria (dark urine) is at an increased risk of venous thrombosis due to platelet defects and has recurrent infections caused by impaired leukocyte fucntions?

Paroxysmal nocturnal hemoglobinuria

31

What 2 disorders by results from paroxysmal nocturnal hemoglobinuria?

Acute leukemia
Aplastic anemia

32

Spectrin mutations...

Hereditary spherocytosis

33

What cause the dark urine with G6PD deficiency?

Intravascular hemolysis

34

Postive Coombs test

Indicates the presence of anti-RBC antibodies

35

IgM binds RBCs at low temperature at peripheral body sites and fix complement--within increased temp the IgM is dissociated from the cell and leaves C3b behind--extravascular hemolysis of RBCs...

Cold agglutinin disease

36

What are disorders/infections that can cause cold agglutinin disease?

Lymphoid neoplasms
Mycoplasma
EBV
HIV
Influenza
CMV

37

What phenomenon is seen with cold agglutinin disease?

Raynauds

38

What lab values are increased in cold agglutinin disease and what causes this?

Increased MCV and Bilirubin caused by increased RBC turnover

39

What immunoglobin is involved in warm antibody hemolytic anemia?

IgG--chronic and not triggered by cold

40

Spherocytes are seen with paroxysmal nocturnal hemoglobinuria, what causes this?

Incrased osmotic fragility of RBCs

41

Increased serum ferritin -mild and marked?

Anemia of chronic disease--mild increase

Hemochromatosis-- marked increase

42

What type of malaria are sickle cell trait ind. resistant to?

P. Falciparum

43

What is erythroblastosis fetalis?

Maternal antibodies coating fetal RBCs--sensitization occurred during previous delivery--IgG made crossing placenta, attaching to fetal RBCs, causing hemolysis

44

Immune-mediated mechanism caused, in many causes, by cephalosporin therapy?

Drug-induced hemolytic anemia

45

What is hepcidin?

Liver-derived plasma peptide

Iron absorption regulator

46

When are hepcidin levels increased?

when iron stores are high

47

When are hepcidin levels decreased?

hereditary and acquired hemochromatosis

48

Iron transporter that moves nonheme iron from he gut lumen to the duodenal epithelium?

DMT-1

49

What is hemosiderin?

Aggregated from of ferritin

Does not circulate like ferritin and is found in tissue

50

HFE gene mutation

Leads to excessive absorption of dietary iron and hemochromatosis

51

What is transferrin?

Transports iron btw plasma, iron stores, and developing RBCs

52

What are the characteristics of anemia of chronic disease?

Increased ferritin with reduced total iron-binding capacity

53

What is the pathophysiology of anemia of chronic disease?

Chronic inflammatory or neoplastic disease increase the secretion of cytokines--IL-1, TNF, Interferon-g--promote sequestration of iron in storage compartments and depress EPO production

54

Disease often seen with chronic alcohol abuse, Is a megaloblastic anemia. Often presents with neutrophils showing defective segmentation--extra nuclear lobes?

Folate deficiency

55

What is indicated by tear drop RBCs?

Myelophthisic disorder--immature RBCs and WBCs and splenomegaly with extensive fibrosis

56

What are the two best know causes of high MCV-- macrocytosis?

Vit B12 deficiency
Folate deficiency

57

What are causes of aplastic anemia?

Exposures to chemo drugs or chemicals

Viral infections--ALMOST NEVER Bacterial infection

58

ADAMTS13 deficiency

accumulation of large von willebrand multimers--results in thrombotic thrombocytopenia purpura (TTP)

59

Heat stroke caused by hyperthermia and loss of perspiration from dehydration leads to...

Hemoconcentration

60

Can cause arterial and DVTs with increased risk of cerebral artery thrombosis. Patient often false positively tests for syphilis? A thrombocythopenia often present. Women with multiple miscarriages should be consider for this disease?

Antiphospholipid syndrome

false + caused by anticardiolipin antibody

61

With DIC what lab values are increased and what are decreased?

PT/PTT increased

Thrombocytopenia
Low fibrinogen concentration

62

Helmet cells

Typical of conditions that den produce a microangiopathic hemolytic anemia--DIC, thrombocytopenia purpura, SLE, hemolytic-uremic syndrome, lamignant HTN

63

Platelets destroyed in spleen after being coated with antibodies to platelet membrane glycoproteins IIb/IIIa or Ib-IX

Immune thrombocytopenic purpura (ITP)

64

Does giving a blood transfusion to a patient with immune throbocytopenic purpura help?

No--antibodies coat both the patient's platelets and any transfused platelets

65

What is the the treatment for immune throbocytopenic purpura if corticosteroid therapy fails?

Splenectomy

66

Seen with Glanzmann thrombasthenia and Chronic immune throbocytopenic purpura?

Glycoprotein IIb/IIIa dysfunction/deficiency

67

Von willebrand factor metalloproteinase deficiency is a feature of what disorder?

Thrombotic thrombocytopenia purpura

68

What is the pathophysiology of heparin-induced thrombocytopenia?

Formation of IgG antibodies to heparin-platelet factor 4 complexes that bind to Fc receptors on the surface of platelets--causes platelet activation and thrombosis

69

What isn't given if a patient present with a protein C deficiency?

Warfarin

70

What is the classic pentad of Thrombotic thrombocytopenia purpura?

Transient neurologic problems
Fever
Thrombocytopenia
Microangiopathic hemolytic anemia
Acute renal failure

71

The activity of what is decreased in thrombotic thrombocytopenia purpura?

ADAMTS13--which acts as a von willebrand factor multiuser protease

72

Patients with liver disease have defects in what clotting factors?

Extrinsic pathway coagulation--causing changes in PT

73

Inherited bleeding disorder with normal platelet count and prolonged bleeding time?

Von willebrand disease

74

Which clotting factor will be decreased with von willebrand disease?

Factor VIII-because vWF acts as a carrier for this factor

75

What is the pathophysiology of von willebrand disease?

Reduction in quantity of vWF impairing platelet adhesion to damaged vessel walls

76

HUS is related to what?

preceding infectious gastroenteritis with diarrhea E. coli infection

77

What clotting factor is decreased with Hemophilia A?

Decreased factor VIII activity

78

What is the characteristic finding of hemophilia A?

Bleeding into soft tissue--joints

79

If gone untreated what can hemophilia A lead to?

Hemathroses

80

What lab value is prolonged due to hemophilia A?

PTT--intrinsic pathway affected

81

If a patient with hemophilia A gets a transfusion to + factor VIII and their PTT is not corrected then what has occur?

An inhibitor of factor VIII is present

If there is no inhibitor then the PTT will be corrected with transfusion

82

What deficiency is involved in hemophilia B?

Factor IX deficiency

83

A rare autosomal recessive disorder that involves defective platelet aggression from deficiency or dysfunction of glycoprotein IIb/IIIa?

Glanzmann thrombasthenia

84

Caused by antibodies to platelet membrane glycoproteins IIb/IIIa or Ib/IX?

Immune thrombocytopenic purpura

85

If a patient receives a platelet transfusion what are they more likely to contract?

More likely to cause sepsis due to the storage at room temp making platelets more susceptible to contamination

86

Caused when donor plasma contains HLA or granulocyte-specific antibodies--granulocyte enzymes are released and increase capillary permeability and result in sudden pulmonary edema and respiratory distress.

Transfusion-related acute lung injury (TRALI)