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Flashcards in Hematology 3% Deck (89):
1

Multiple myeloma shows ____ RBCs.

Rouleaux formation d/t increase plasma proteins

2

B12 and Folate deficiency presents as (macro/micro)cytic anemia and ____.

MACROcytic anemia
hypersegmented neutrophils

3

Heinz bodies =

Seen in ____

denatured Hgb in cells

Thalassemias (alpha thalassemia intermedia)
G6PD Deficiency

4

Howell-Jolly Bodies seen in ____.

Splenectomized pts

5

Target cells seen in ___.

Sickle cell disease
Thalassemia
Hgb SC

Severe Fe deficiency
Asplenia
Liver disease

6

Echinocytes (a.k.a. ____) seen in ____ (3)

Burr cells

Uremia *
Pyruvate kinase deficiency
Hypophosphatemia

7

Acanthocytes (a.k.a. ___) seen in ____ (5)

Spur cells

Liver disease (ETOHic cirrhosis)
Post splenectomy
Thalaseemia
Autoimmune Hemolytic Anemia
Renal Disease

8

Reticulocytosis =

Seen when ___

Immature RBC

Increased blood loss
Increased RBC destruction (hemolytic anemia)

9

Fe deficiency anemia
(increase/decrease) serum Fe
(increase/decrease) ferritin
(increase/decrease) TIBC

decrease serum Fe
decrease ferritin
INCREASE TIBC

10

Anemia of Chronic Disease
(increase/decrease) serum Fe
(increase/decrease) ferritin
(increase/decrease) TIBC
(increase/decrease) Haptoglobin
(increase/decrease) CRP
(increase/decrease) Hepcidin

-decrease serum Fe
-INCREASE ferritin
-DECREASE TIBC
-Increase Haptoglobin = binds free hemoglobin reducing iron availability for microbes
-Increase CRP
-Increase Hepcidin = prevent iron release from macrophages --> reducing iron availability for microbes

11

Low reticulocytes seen in ____

Fe, B12, Folate deficiencies
Anemia of chronic disease

12

High reticulocytes seen in ____

Blood loss
Hemolytic anemias

13

Indirect hyperbilirubinemia is associated with ___.
Direct hyperbilirubinemia is associated with ___.

Indirect: Jaundice

Direct: dark urine b/c water soluable

14

Hemolytic anemia:
(increase/decrease) reticulocytes
(increase/decrease) LDH
(increase/decrease) indirect bilirubin
(increase/decrease) haptoglobin

Increase reticulocytes
Increase LDH = enzyme in RBCs --> RBC destruction --> increase LDH
Increase indirect bilirubin
DECREASE Haptoglobin = increase RBC destruction --> increased free Hgb --> haptoglobin binds to free Hgb --> haptoglobin stores used up

15

Alpha Thalassemia:
Hgb ratio

Normal Hgb ratios of HgbA, A2 and F

16

Beta Thalassemia:
Hgb ratio

Decreased HgbA
Increased HgbA2
Increased HbgF

17

T/F: TTP and HUS can be distinguished via abnormal coag labs (prolonged PT and PTT).

False. Both have normal coags, can't be distinguished via labs.

Prolonged PT and PTT seen in Disseminated Intravascular Coagulation (DIC)

18

TTP presentation

Thrombocytopenia
Hemolytic anemia
Kidney damage
Neuro sx**
Fevers**

19

HUS presentation

Thrombocytopenia
Hemolytic anemia
Kidney damage

20

"Dark urine worse in AM"

Paroxysmal nocturnal hemoglobinuria

21

Schilling test used to diagnose ____.

Other findings (3)

Pernicious Anemia (B12 deficiency)

+ Intrinsic factor ab
Parietal cell ab
Increased gastrin levels

22

B12 is absorbed in ____
Folate is absorbed in ___

B12: ileum
Folate: jejunum

23

Watch for signs of ____ when giving B12 replacement

HYPOkalemia

24

T/F: Folate deficiency causes pallor, glossitis, stomatitis and neurologic sx (peripheral neuropathy, spinal cord demyelination** and degeneration)

False. B12 causes neurological symptoms, NOT folate. Otherwise, both present similarly.

Neurologic sx of B12 deficiency: peripheral neuropathy, ataxia, weakness, vibratory, sensory and proprioception deficits, DECREASED DTR

25

T/F: Replacing folate in pts w/ B12 deficiency will correct anemia but worsen neuro sx.

True

26

Microcytic anemia w/ normal/increased Fe or no response to Fe tx =

Thalassemia

27

HgbA =
HgbA2 =
HgbF =

HgbA = aabb
HgbA2 = aadd
HgbF =aagg

a= alpha
b= beta
d= delta
g = gamma

28

What type of thalassemia?
1. normal Hgb ratio
2. Increased HgbA2, increased HgbF, decreased HgbA
3. Increased HgbA2, increased HgbF (90%), little or no HgbA

1. Alpha thalassemia (mior, intermedia, major)
2. Beta thalassemia minor
3. Beta thalassemia major (Cooley's)

29

Hgb Barts seen in ___

alpha thalassemia major (Hydrops fetalis)
=gamma tetramers

Associated w/ stillbirth, or death soon after

30

Deferoxamine

Iron chelating agents
Tx of severe anemia, Apha/beta thalassemia major

31

Pts with beta-thalassemia major will become symptomatic at age ___.

6 months (when HgbF declines)

32

Plummer Vinson syndrome =

Dysphagia + esophageal webs + atrophic glossitis + Fe deficiency

33

Anemia of chronic disease: (macro/normo/micro)chromic (macro/normo/micro)cytic anemia

Tx:

Normochromic, normocytic anemia

Tx: Erythropoietin-alpha

34

G6PD Deficiency:
Peripheral smear shows ____
Labs:
(increased/decreased) reticulocytes
(increased/decreased) (indirect/direct) bilirubin
(increased/decreased) haptoglobin

Schistocytes
Heinz bodies

Increased reticulocytes
Increased indirect bilirubin
Decreased haptoglobin

35

Sickle cell disease dx:
Hemoglobin electrophoresis
Peripheral smear

Increased HgbF, HgbS, NO HgbA

Target cells, sickled erythrocytes, Howell-Jolly bodies (functional asplenia)

36

Sickle cell disease tx:
Pain control
Severe pain crisis
Immunization against ___

Pain control:
IV hydration and oxygen 1st step in pain crisis
Hydroxyurea: SEVERE pain crisis

Immunize against: S. pneumococcus, H. Influenza B, N. Meningococcus
(SHiN)

37

Do NOT give ____ in Sickle Cell Disease pain crisis.

Meperidine (Demerol)
May lead to seizures and renal failure

38

Signs of Sickle cell disease present at age ____.
MC 1st presentation =
Aplastic crisis associated w/ ___.
Osteomyelitis MC caused by ___.

6 months
Dactylitis
Aplastic crisis: Parvovirus B-12
Osteomyelitis: Salmonella

39

PTT measures ___ pathway. Involves ___ factors.

intrinsic and common pathway
Factors 1, 2, 5, 8**, 9**, 10, 11**, 12**

40

Warfarin therapy causes prolonged (PT/PTT)
Heparin therapy causes prolonged (PT/PTT)

Warfarin: PT
Heparin: PTT

41

PT measures ___ pathway. Involves ___ factors.

Extrinsic and common pathway
Factors 1, 2, 5, 7**, 10**

42

DIC causes prolonged (PT/PTT)
Vit K deficiency causes prolonged (PT/PTT)
vWD causes prolonged (PT/PTT)
Hemophilia A/B causes prolonged (PT/PTT)

DIC causes prolonged PT AND PTT
Vit K deficiency causes prolonged PT
vWD causes prolonged PTT
Hemophilia A/B causes prolonged PTT

43

Heparin overdose antidote =
Warfarin overdose antidote =

Heparin OD = Protamine sulfate

Warfarin OD = Vitamin K

44

Antibody against ADAMTS13 seen in ____.
PT/PTT:

Tx:

Thrombotic Thrombocytopenic Purpura (TTP)

Normal PT/PTT

Tx: Plasmapheresis ***
Immunosuppression: steroids, cyclophosphamides

45

Difference between TTP and HUS presentation

Kidney failure more common in HUS
Fever and neurologic sx (HA, CVA, AMS) in TTP, NOT HUS

46

Suspect ___ if renal failure in children with diarrhea prodrome

Hemolytic Uremic Syndrome

HUS usually preceded by Enterohemorrhagic E. Coli 0157:H&, Shigella, or Salmonella gastroenteritis

47

Platelet activation by exotoxins seen in ___.
PT/PTT:

Hemolytic Uremic Syndrome
Normal PT/PTT

48

T/F: Antibiotics are useful in treatment of HUS.

False. May actual worsen condition d/t increased verotoxin release w/ cell lysis.

49

Disseminated Intravascular Coagulation (DIC)
(increase/decrease) PTT
(increase/decrease) PT
(increase/decrease) INR
(increase/decrease) fibrinogen

Increase PTT/PT/INR
Decrease fibrinogen

Also increased D-dimer

50

Tx of Disseminated Intravascular Coagulation

Fresh Frozen Plasma: if severe bleeding
Cryoprecipiate: replace fibrinogen
Platelet transfusion: ONLY if <20K

51

T/F: Idiopathic (Autoimmune) Thromobocytopenic Purpura presents with mucocutaneous bleeding (purpura, bruises, petechiae, bullae) and splenomegaly.

False. NO splenomegaly

52

Acute ITP seen in MC___

children/boys after viral infection --> self limited

53

Tx of ITP

Children: observation +/- IVIG
Adults: Corticosteroids --> IVIG --> splenectomy if refractory

54

T/F: Petechiae is commonly seen in Hemophilia A/B.

False. Not common in Hemophilia A/B. Common in Von Willebrand Disease.

55

Von Willebrand Disease:
Bleeding time and PTT prolongation worsen with ___.

Gold standard dx:

worsen with aspirin

Decreased Ristocetin activity test: No platelet aggregation w/ Ristocetin

56

Von Willebrand Disease:
Prolonged (PTT/PT)
(Does/does not) correct w/ mixing study.

PTT
DOES correct

57

Tx of Type I Von Willebrand Disease
Mild:
Moderate:
Severe:

Mild: NO treatment
Moderate: DDAVP
Severe: Cryoprecipitate

58

Reed sternberg cells is pathognomonic for ____.

Hodgkin's Lymphoma

59

T/F: Hodgkin lymphoma is more difficult to cure than Non-Hodgkin lymphoma.

False. Hodgkin lymphoma is highly curable compared to NHL.

60

Hodgkin or Non-Hodgkin lymphoma:
1. Upper body lymph nodes
2. MC in >50 y/o
3. Associated w/ clonal B-cell proliferation
4. Extra nodal sites are common

1. Upper body lymph nodes: HL
Peripheral lymph nodes: NHL

2. MC in >50 y/o: NHL
Bimodal 20 y/o and > 50 y/o: HL

3. Associated w/ clonal B-cell proliferation: HL

4. Extra nodal sites are common: NHL
Pel-Ebstein fevers: HL

61

MC indolent Non-hodgkin lymphoma

Follicular: Not curable generally

62

MC aggressive Non-hodgkin lymphoma

Diffuse Large B cell: curable w/ chemo

63

Clinical manifestation of Multiple Myeloma

"BREAK"
Bone pain
Recurrent infections
Elevated calcium
Anemia
Kidney Failure

64

Increased risk of multiple myeloma seen in ___

>65 y/o
African Americans
men

65

Single clone of plasma cells, accumulate in bone marrow interrupting marrow's normal cell production.

Multiple myeloma

Increased monoclonal Ab (esp IgG, IgA)

66

Dx of Multiple Myeloma

Serum Protein Electrophoresis: monoclonal protein spike
Urine Protein Electrophoresis: Bence-Jones proteins

Rouleaux formation, Increased ESR
Skull xray: Punched out lesions

Bone scan NOT helpful

67

Bence Jones proteins seen in ___

Multiple Myeloma

68

Philadelphia Chromosome = translocation between chromosome ___, causing fusion gene ___.

Seen in ___.

9 and 22
gene bcr-abl

CML

69

MC childhood leukemia malignancy

Bone marrow shows:

ALL

Hypercellular > 20% blasts

70

Auer rods seen in ___

AML

71

MC form of leukemia in adults:
MC acute leukemia in adults:

MC form of leukemia in adults: CLL

MC acute leukemia in adults: AML

72

Tumor lysis syndrome caused by ___.
Management:

chemotx initiation d/t large number of cells being destroyed
- Hyperuricemia, HyperK, HypoCa, HyperPhosphate, acute renal failure

Management: Allopurinol

73

Acquired myeloproliferative d/o w/ overproduction of all 3 stem cell lines.

Polycythemia Vera

74

Polycythemia Vera:
(increase/decrease) Hematocrit/RBC
(increase/decrease) erythropoietin levels
(increase/decrease) WBCs
(increase/decrease) Leukocyte alkaline phosphatase
(increase/decrease) B-12

Increased Hematocrit/RBC
Decreased erythropoietin levels
Increased WBCs
Increased Leukocyte alkaline phosphatase
Increased B-12

75

Polycythemia Vera tx

Phlebotomy**
Hydroxyurea

76

Primary erythrocytosis is distinguished from secondary erythrocytosis by ____.

normal WBC/platelets in 2ry

Secondary d/t:
Hypoxia (ex COPD)
Pathologic (renal dz)
Reactive polycythemia (normal RBCs mass in setting of decreased plasma volume)

77

C282Y HFE genotype seen in ____

hereditary hemochromatosis

78

Hereditary hemochromatosis
(increase/decrease) serum iron
(increase/decrease) serum transferrin saturation
(increase/decrease) ferritin
(increase/decrease) TIBC

INCREASED iron, transferrin, ferritin

NORMAL/DECREASED TIBC

79

Gold standard of dx for hereditary hemochromatosis

Tx

Liver biopsy: increased liver parenchymal hemosiderin

Tx: Phlebotomy

80

Coagulopathy of Advanced Liver Disease:
(increase/decrease) PT
(increase/decrease) albumin
(increase/decrease) PTT

Tx:

Increased PT
Decreased albumin
Increased PTT (in advanced disease)

Tx: Fresh Frozen Plasma

81

T/F: Increased association w/ EBV seen in Non-Hodgkins Lymphoma

False. In Hodgkins Disease. Rare in most NHL.

82

Antibodies against RBC's surface causing increased RBC destruction by macrophages, spleen and complement

Dx:

Autoimmune Hemolytic Anemia

Dx:
+ Direct Coombs test
Cold agglutinin study
Peripheral smear: microspherocytes, polychromasia, agglutination of RBCs

83

+ Coombs distinguishes ____

Autoimmune Hemolytic Anemia from Hereditary Spherocytosis

84

Warm agglutinin =___ , causes ___. Etiology:
Cold agglutinin =___, causes ___. Etiology:

Seen in ___

Warm = IgG Ab --> SPLENIC macrophage RBC destruction via phagocytosis
Etiology: SLE*** MC (Autoimmune d/o)

Cold = IgM Ab -->INTRAVASCULAR complement-mediated RBC lysis, especially in COLD temperatures
Etiology: May follow infection (Mycoplasma**, EBV**)

Seen in Autoimmune Hemolytic Anemia

85

Tx of Autoimmune Hemolytic Anemia

Warm: Steroids**, immunosuppressants, splenectomy (removes site of RBC destruction)

Cold: avoid cold exposure**, warm fluids. Rituximab

86

Osmotic fragility test used to dx ____

Coombs test +/-
Peripheral smear:
(Hyper/normo/hypo)chromic (macro/normo/micro)cytosis

Hereditary spherocytosis

Coombs test negative
Smear: 80% spherocytes, HypERchromic MICROcytosis

87

Hereditary spherocytosis:
Autosomal ___ intrinsic hemolytic anemia
Causes defect in ____ --> ____ and ___ --> Increased RBC hemolysis in ___.

Autosomal dominant

Defect in RBC membrane/cytoskeleton --> cell fragility and sphere-shaped RBCs --> hemolysis in SPLEEN

88

Treatment of choice of Hereditary spherocytosis in severe disease

Splenectomy*

Folic acid not curative but helpful

89

Von Willibrand disease will have increased ____.

Bleeding time
PTT