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Flashcards in Hematology/Oncology Deck (109):
1

Erythrocytosis
Anisocytosis
Poikilocytosis
Reticulocyte

-Erythrocytosis = polycythemia = increased RBCs
-Anisocytosis = varying sizes
-Poikilocytosis = varying shapes
-Reticulocyte = immature RBC

2

Spectrin

supplies the biconcavity/flexibility of RBCs

3

1/3 of platelets are stored in?

Spleen (lifespan = 8-10 days)

4

Causes of polycythemia/erythrocytosis?

-Any time have increased EPO (hypoxia, renal cell carcinoma, HCC, pheochromocytoma, hemangioblastoma)
-Down's babies at birth
-Polycythemia vera --> have increased RBCs, but normal EPO

5

Barr bodies

=inactivated X in neutrophils
-->seen in all women + KF men

6

When do you see hypersegmented polymorphic neutrophils?

-Vitamin B12/Folate deficiency

7

Neutrophil chemotactic factors?

-Leukotriene B4
-IL-8
-C5a

8

CD14 = cell marker for?

Macrophages

9

Causes of Eosinophilia?

*DNA-CAAPA:
-Drugs
-Neoplastic
-Asthma (and Churg-Strauss)
-Collagen Vascular diseases
-Allergic processes
-Addison's (adrenal insufficiency)
-Parasites (invasive)
-Acute Interstitial Nephritis

10

Phagocytes:
-in brain
-in tissue
-in liver
-in joints

-brain = microglia
-tissue = macrophages
-liver = kupffer cells
-joints = A-cells

11

Mast cells vs Basophils?

Both are similar, mediate allergic rxn
-Basophils in Blood
-Mast cells in tissue

-mast cells are involved in type 1 hypersensitivity rxns

12

Langerhans cells

-dendritic cells on skin and mucous membranes
-act as professional APCs
-possess Birbeck granules = racquet-shaped intracytoplasmic granules

13

Main inducers of primary antibody response?

Dendritic cells = professional APCs

14

clock-face chromatin

Plasma cells (B cells differentiate into plasma cells; plasma cells produce lots of antibody specific to a particular antigen)

15

plasma cell neoplasm?

Multiple Myeloma --> make a whole bunch of one particular type of B cell, all making one type of antibody (so see monoclonal antibody spike)

16

CD3

on ALL T-cells
-->Th also have CD4
-->Cytotoxic also have CD8

17

anti-AB antibodies (Ig type?)

-IgM --> don't cross placenta

18

anti-Rh antibodies (Ig type?)

=IgG --> can cross placenta and cause hemolytic disease of newborn (erythroblastosis fetalis) if fetus is Rh+

19

Blood type A (example)

-have A antigen on RBC surface
-B antibody in plasma

20

erythroplastosis fetalis
-cause?
-symptoms?
-hypersensitivity type?
-how to prevent?

-type 2 hypersensitivity rxn
-cause: Rh- mother exposed to Rh+ fetal blood during delivery; makes anti-Rh IgG --> can cross placenta in subsequent pregancies, causing hemolytic disease of newborn in next fetus who is Rh+
-Symptoms in infant: hepatosplenomegaly, severe anemia, jaundice, demise/death
-Treat/Prophylaxis = give mother Rhogam = Rho (D) Ig at first delivery to prevent all future erythroblastosis

21

Deficiency of Factor VIII?

Hemophilia A
("Aight")

22

Deficiency of Factor IX?

Hemophilia B
("Benine")

23

Vitamin K deficiency?

decreased synthesis of "diSCo 1972"
-proteins C and S
-Factors X, IX, VII, II (10, 9, 7, 2)

*Vitamin K normally catalyzes carboxylation of glutamic acid residues on proteins involved with blood clotting

24

Vitamin K antagonist?

Warfarin
-->acts by inhibiting Epoxide Reductase (vitamin K-->activated vitamin K)

25

Antithrombin inhibits?

Antithrombin inhibits:
-thrombin
-factors VIIa, IXa, Xa, XIa, XIIa

26

Drug that activates antithrombin?

Heparin

27

Coagulase

Produced by S. aureus
-->can convert fibrinogen-->fibrin

28

tPA, Streptokinase, Urokinase:

All facilitate: plasminogen--> plasmin
(so, stimulate breakdown of clots!)

29

vWF receptor on platelets? Fibrinogen receptor on platelets?

-vWF --> GpIb
-Fibrinogen --> GpIIb/IIIa

***vWF and Fibrinogen are both inside platelets

30

ESR in pregnancy?

increased

31

Decreased ESR:

-polycythemia
-sickle cell anemia
-CHF
-microcytosis
-hypofibrinogenemia

32

Spur cell (Acanthocyte)

Liver disease, Abetalipoproteinemia
-->irregularly spiked RBCs

33

basophilic stippling

TAIL:
-Thalassemias
-Anemia of chronic disease
-Iron deficiency
-LEAD poisoning!

-->have denatured RNA within RBCs

34

type of anemia caused by folate or B12 deficiency?

Macrocytic, Megaloblastic anemia

35

iron deficiency anemia + esophageal web + atrophic glossitis?

Plummer-Vinson syndrome

36

"crew cut" on skull x-ray?

Bone Marrow expansion --> see in beta-thal major
-thalassemias
-Sickle cell disease

37

chipmunk facies

beta-thal major

38

Ringed sideroblasts

sideroblastic anemia = defect in heme synthesis (x-linked defect in ALA synthase gene)
-treat with B6 (Pyridoxine)

39

Lead poisoning symptoms: LEEAADDS

-Lead lines on gingivae (Burton's lines) and on epiphyses of long bones on x-ray
-Encephalopathy
-Erythrocyte basophilic stippling
-Abdominal colic
-Anemia (sideroblastic anemia)
-Drops (wrist and foot drop)
-Dimercaprol and EDTA = treatment
-Succimer = treatment for kids

40

Dimercaprol

treatment for lead poisoning (also EDTA)

41

Succimer

=treatment for kids with lead poisoning ("SUCks to be a kid with lead poisoning")

42

Hypersegmented neutrophils + glossitis +
increased homocysteine and:
-normal methylmalonic acid?
-increased methylmalonic acid?

-if normal methylmalonic acid: Folate deficiency
-if increased methylmalonic acid: B12 deficiency

***both cause megaloblastic macrocytic anemia (so have impaired DNA synthesis and ineffective erythropoiesis = pancytopenia)

43

Megaloblastic anemia that's not correctable by vitamin B12 or Folate?

--> Orotic Aciduria

44

Pancytopenia =

-Anemia
-Neutropenia
-Thromboctyopenia

45

Fanconi's anemia

DNA repair defect --> get aplastic anemia

(NOT the same as Fanconi's syndrome = proximal tubule reabsorption defect in kidneys!)

46

Why anemia in kidney disease?

decreased EPO --> decreased hematopoiesis

47

Aplastic anemia

-Pancytopenia
-Normal cell morphology, but hypocellular bone marrow with fatty infiltration

48

Hemolytic anemia in a newborn?

Pyruvate kinase deficiency --> decreased ATP --> rigid RBCs

49

HbC defect

Glutamic acid-to-lysine mutation

*pts with HbSC (1 of each mutant gene) have milder disease than HbSS pts (full sickle cell)

50

older pt, unexplaned anemia:

must rule out colon cancer

51

reversible etiologies of sideroblastic anemia?

-alcohol
-lead

52

what vitamin should be supplemented in vegans/vegetarians?

B12 (may get deficiency --> megaloblastic anemia)

53

Hepcidin

released by liver, binds ferroportin on intestinal mucosal cells and macrophages
-increased in cases of inflammation: increased hepcidin-->decrease release of iron from macrophages

*this is what happens in anemia of chronic disease: decreased iron, decreased TIBC, increased ferritin

54

decreased iron + decreased TIBC + increased ferritin

anemia of chronic disease (inflammation--> increased hepcidin --> decreased release of iron from macrophages

55

Cause of hereditary spherocytosis?

-extravascular intrinsic hemolytic normocytic anemia
-caused by defect in proteins interacting with RBC membrane skeleton and plasma membrane (ankyrin, band 3, protein 4.2, spectrin)
-premature removal of RBCs by spleen --> splenomegaly, aplastic crisis; eventually need splenectomy

56

Labs for PNH (paroxysmal nocturnal hemoglobinuria)?

increased urine hemosiderin

57

complication of PNH?

thrombosis

58

Treatment for sickle cell anemia?

-Hydroxyurea --> increases HbF
-Bone marrow transplant = ultimately

59

Coomb's positive anemia?

autoimmune hemolytic anemia (ie warm agglutinin or cold agglutinin anemias)

60

microangiopathic anemia

-see schistocytes
-RBCs damaged when passing through obstructed or narrowed vessel lumina
-see in: DIC, TTP-HUS, SLE, malignant HTN; prosthetic valves, aortic stenosis...

61

rate limiter of heme synthesis?

ALA synthase (delta-aminolevulinic acid synthase)

62

effect of heme on ALA synthase activity

low heme--> increased ALA synthase
lots of heme-->decreased ALA synthase

63

Rate limiter of heme synthesis

ALA synthase (requires B6!)

64

tea-colored urine + blistering cutaneous photosensitivity?

-Porphyria cutanea tarda (most common porphyria)
-d/t deficiency of Uroporphyrinogen Decarboxylase (needed for heme synthesis); get accumulation of Uroporphyrin (hence the tea-colored urine)

65

painful abdomen + red-wine colored urine + polyneuropathy + psych disturbances; precipitated by drugs:

Acute Intermittent Porphyria
-d/t deficiency of Porphobilinogen Deaminase (aka uroporphyrinogen-I-synthase)
-accumulate Porphobilinogen, ALA, and uroporphyrin (in urine)

66

Treatment for Acute Intermittent Porphyria?

-Glucose and Heme --> inhibit ALA synthase

67

Defect in factors I, II, V, VII, X -->?

increased PT (extrinsic pathway defect)

68

Defect in all factors EXCEPT VII and XIII?

increased PTT

69

Defects in platelet plug formation?

increased BT (bleeding time)

70

Bernard-Soulier disease:
-defect
-labs (BT =bleeding time; PC=platelet count)

defect in Gp1b--> so vWF can't bind platelets for platelet plug formation

*increased BT
*decreased PC

71

Glanzmann's thrombasthenia:
-defect?
-BT/PC?

-defect in GpIIb/IIIa --> platelets can't link to eachother, so no platelet aggregation for platelet plug formation
-increased BT
-no effect on PC

72

ITP = Idiopathic Thrombocytopenic Purpura:
-defect?
-labs?

-defect:have anti-GpIIb/IIIa antibodies--> antibodies bind platelets --> complex gets consumed by splenic macrophages
-increased BT
-decreased PC
-also have increased megakaryocytes (trying to make more platelets!)

73

TTP = Thrombotic Thrombocytopenic Purpura
-defect?
-labs?

deficiency of vWF metallprotease --> decreased degradation of vWF multimers: so: increased vWF multimers--> increased platelet aggregation and thrombosis--> decreased platelet survival
-increased BT
-decreased PC
-schistocytes
-increased LDH

74

Affect of aspirin on BT/PC?

increased BT
no effect on PC
(like Glanzmann's)

75

All platelet disorders effects on BT/PC?

-all increase BT
-all decrease PC, except Glanzmann's

76

most common inherited bleeding disorder?

von Willebrand's disease

77

PC, BT, PT, PTT findings in von Willebrand's disease?

-increased BT
-increased or normal PTT (depends on severity)
-normal PC and PT

78

-treatment for von-Willebrand's disease?

DDAVP = Desmopressin (releases vWF stored in endothelium)

79

PC, BT, PT, PTT findings in DIC?
-other lab findings?

-decreased PC
-increased BT
-increased PT
-increased PTT

-also: schistocytes, increased D-dimers (fibrin split products), decreased fibrinogen, decreased factors V and VIII)

80

Causes of DIC?

"STOP Making New Thrombi"
-Sepsis
-Trauma
-Obstetric complications
-acute Pancreatitis
-Malignancy
-Nephrotic syndrome
-Transfusion

81

Factor V Leiden

most common cause of inherited hypercoagulability
-production of mutant factor V, that can't be degraded by protein C

82

Cryoprecipitate

contains fibrinogen, factor, VIII, factor XIII
-used to treat coagulation factor deficiencies (if deficient in fibrinogen or factor VIII)

83

CD30+ and CD15+ cells of B-cell origin?

Reed-Sternberg cells

84

Most common Hodgkin's lymphoma? Prognosis?

-Nodular Sclerosing
-Excelent prognosis (b/c Lymphocytes>>>R-S cells)
-see collagen banding in lymph node
-mostly young adults

85

t(8;14) c-myc gene

Burkitt's lymphoma (non-Hodgkin lymphoma; neoplasm of mature B cells)

86

Most common adult non-Hodgkin lymphoma?

Diffuse large B-cell lymphoma
-->20% of cases are in kids though

87

t(11;14); affects cyclin D regulatory gene

Mantle Cell Lymphoma
-affects older males; poor prognosis

88

t(14;18) bcl-2 overexpression

-Follicular Lymphoma
-affects adults
-indolent course, but difficult to cure
-bcl-2 inhibits apoptosis (normally; it's a proto-oncogene)

89

Lymphoma caused by HTLV-1?

Adult T-cell lymphoma
-->adults present with cutaneous lesions
-->aggressive

90

Mycosis fungoides/Sezary syndrome

-mature T-cell lymphoma; adults present with cutaneous lesions
-indolent

91

Bence-Jones protein

Multiple Myeloma
-Ig light chains in urine (don't see on urinalysis though)

92

Rouleaux formation

Multiple Myeloma

93

most common primary tumor arising within bone in elderly?

Multiple Myeloma

94

CRAB symptoms of Multiple Myeloma:

-hyperCalcemia
-Renal insufficiency
-Anemia
-Bone lytic lesions/Back pain

95

Down syndrome pts: increased risk of which cancers?

-ALL
-AML

96

stains TRAP positive (tartrate-resistant acid phosphatase)

Hairy cell leukemia
-mature B-cell tumor in elderly; cells have filamentous, hairlike projections

97

Age group affected by ALL?

<15 years old
-most responsive to therapy
-may spread to CNS and testes
-t(12;21) = better prognosis

98

Hallmark of Acute Leukemias?

-lots of blast cells (>20% blasts)
-rapid onset and progression

99

Auer rods

AML = t(15;17)
-responds to vitamin A

100

Which leukemia can be treated with Vitamin A?

AML
-t(15;17)
-auer rods

101

t(9,22), bcr-abl

Philadelphia chromosome
-CML
-30-60 yrs old
-more mature cells (<5% blasts); insidious onset
-resonds to imatinib = bcr-abl tyrosine kinase inhibitor

102

Imatinib

treatment for CML
-inhibits bcr-abl tyrosine kinase

103

Teardrop cell

Myelofibrosis
-->fibrosis of bone marrow (
-->bone marrow is crying because it's fibrosed :(

104

JAK2 mutations: positive/negative?
Philadelphia chromosome?
-polycythemia vera
-essential thromboyctopenia
-myelofibrosis
-CML

*JAK2:
-positive in polycythemia vera, essential thrombocytosis, myelofibrosis
-negative in CML
*Philadelphia:
-only positive in CML

105

thombolytics mechanism? (streptokinase, urokinase, tPA, APSAC)

aid conversion of plasminogen-->plasmin, which cleaves thrombin and fibrin clots

106

Affect of aspirin on BT, PT, PTT

increased BT
-no effect on PT, PTT

107

Ticlopidine toxicity?

-Neutropenia (should monitor CBC during first few months of trtmt)
(Ticlopidine = ADP-receptor blocker, so inhibits platelt aggregation; like Clopidogrel)

108

Drug side effect = tinnitus?

Aspirin!

109

Raloxifene vs Tamoxifen: Which is better for osteoporosis prevention?

-both are SERMs
-->antagonists in breast (treat breast cancer)
-->agonists in bone (bone-building)

-Tamoxifen may increase risk of endometrial carcinoma, b/c it's a partial endometrial agonist
-Raloxifene is an endometrial antagonist, so no increased risk of endometrial carcinoma; so, better choice for osteoporosis trtmnt