Hematology/Hematopathology Flashcards Preview

Uworld Journal > Hematology/Hematopathology > Flashcards

Flashcards in Hematology/Hematopathology Deck (126):
1

What (2) infections produce lymphocytic leukocytosis?

-Viral infections (CD8+ cells increase)
-Bordetella pertussis infection (bacteria produces lymphocytosis promoting factor which blocks lymphocytes from leaving the blood)

2

Mutations in the Myeloproliferative neoplasms (4)

CML = Philidelphia chromosome t(9;22) -> BCR-ABL fusion protein
PV = JAK2 mutation
ET = JAK2 mutation
Myelofibrosis = JAK2 mutation

all of these (+) tyrosine kinase -> proliferation of cell production

3

"starry sky" appearance

Burkitt Lymphoma

4

presentation: rapidly enlarging LN in an adult

Diffuse Large B Cell Lymphoma

5

adult from Japan + IV drug user + rash + lytic bone lesions

Adult T Cell Lymphoma

6

Cerebriform nuclei

Mycosis fungoides/Sezary syndrome

7

Anisocytosis definition

variation in RBC size

8

Poikilocytosis definition

variation in RBC shape

9

What do the hepcidin levels indicate?

Hepcidin sequesters iron in its storage places and suppresses Epo production. Overall goal is to reduce iron in circulation.

Increased hepcidin = Decreased circulating iron (decreased TIBC, decreased serum iron)
Increased ferritin (because iron is stuck in storage)

10

Corrected reticulocyte count (%) equation

(% Retics observed x Hct)/ normal Hct

normal Hct = 45

11

Normal reticulocyte count =

1-2%

12

normal reticulocyte count response to anemia =

what does that indicate?

>3%

indicates good marrow response and thus peripheral destruction

13

When do you need to correct the reticulocyte count?

Anemia = decrease in total RBCs can falsely elevate reticulocyte count

14

What does anemia + corrected reticulocyte count of less than 3%

poor marrow response to anemia and thus this is an underproduction problem

15

Indirect BR = unconjugated or conjugated

Indirect BR = Unconjugated BR

16

Direct BR = unconjugated or conjugated

Direct BR = Conjugated BR

17

-Normocytic
-Shistocytes
-hemoglobinuria
-jaundice
-low serum haptoglobin
-corrected Retic count > 3%

Intravascular hemolysis -> normocytic anemia

18

-Normocytic
-Jaundice
-Spherocytes
-BR gallstones
-corrected Retic count > 3%

Extravascular hemolysi -> normocytic anemia

19

Cause of a corrected reticulocyte less than 3%

Aplastic anemia = failure of bone marrow therefore it cannot produce the needed cells (including reticulocytes)

20

Corticosteroids ->
-neutrophils?
-lymphocytes?
-eosinophils?

-neutrophilis: neutrophilia due to the decreased activation of neutrophil adhesion molecules therefore locking the neutrophils in the blood

-lymphocytes: lymphopenia due to increased apoptosis of lymphocytes

-eosinophils: eosinopenia due to sequestration of eosinophils in the lymph nodes

21

Intrinsic clotting cascade players =

What test measures activity?

XII
XI
IX
VIII

Measured by aPTT

22

Extrinsic clotting cascade players =

What test measures activity?

VII

Measured by PT

23

IgG to the heparin-platelet factor 4 complex found in?

thrombosis or bleeding?

Heparin-induced thrombocytopenia

Promotes thrombosis in the setting of thrombocytopenia -> makes the thrombcytopenia worse!

24

adult female + numbness and tingling on left arm and face + hematuria + proteinuria + shistocytes on peripheral blood + thrombocytopenia + fever =

pathogenesis?

Thrombotic thrombocytopenic purpura (TTP)

Pathogenesis: Deficiency in vWF protease (ADAMTS13) -> large multimers of vWF accumulate and stimulate platelet aggregation

25

Deficiency of platelet glycoprotein Ib (GPIb) -> disorder of platelet function

What disease?
Normal function of GPIb

Bernard-Soulier Syndrome

GPIb adheres vWF to the exposed subendothelial collagen. If not present, platelets cannot adhere.

26

Deficiency of platelet glycoprotein IIb/IIIa (GPIIb/IIIa) -> disorder of platelet function

What disease?
Normal function of GPIIb/IIIa

Glanzmann Thrombasthenia

GPIIb/IIIa normally aggregates the platelets to one another. If not present, platelets cannot aggregate.

27

Budd-Chiari syndrome =

What (2) neoplasms is it seen in?

Budd-Chiari Syndrome = splenic or hepatic vein thrombosis

Seen in:
-Polycythemia vera (PV)
-Essential thrombocythemia (ET)

Both types of myeloproliferative neoplasms that have a JAK2 gene mutation

28

-Lymphoblast cells >20% of bone marrow
-age less than 15 years old

Acute Lymphoblastic Leukemia (ALL)
- B cell type in young kids
- T cell type in older kids/adolescents and more of a mass (lymphoma)

29

t(8;14)

what genes are involved?

Burkitt lymphoma

c-myc (chr 8) and heavy chain Ig (chr 14)

30

t(14;18)

what genes are involved?

Follicular lymphoma

heavy chain Ig (chr 14) and BCL-2 (chr 18)

31

t(11;14)

what genes are involved?

Mantle cell lymphoma (MALToma)

cyclin D1 (chr 11) and heavy chain Ig (chr 14)

32

t(15;17)

what genes are involved?

Acute promyelocytic leukemia

produces a PML/RARA -> abnormal retinoic acid receptor

33

Microcytic anemias (4)

-Iron deficiency
-Thalassemia
-Sideroblatsic/lead poisoning
-Anemia of chronic disease

34

Macrocytic anemias
-megaloblastic (2)
-non-megaloblastic (3)

Megaloblastic:
-Folate deficiency
-Vitamin B12 deficiency

Non-megaloblastic:
-Alcohol
-Liver disease
-Bone marrow failure

35

autologous hematopoetic stem cell transplant =

patient is his/her own donor

36

allogenic hematopoetic stem cell transplant =

donor is related or unrelated but they are HLA-matched

37

syngenic hematopoetic stem cell transplant =

donor is the healthy identical twin

38

where are bone marrow transplants taken from?

posterior iliac crests

39

marker for the pluripotent hematopoetic stem cell

CD34+

40

Prophylaxis of acute GVHD?

Calcineruin inhibitor (cyclosporin, tacrolimus) + Methrotrexate

calcineurin inhibitor = decreases action of IL2 + block IL2 synthesis = induce apoptosis of CD4 T cells and inhibit T cell clonal expansion

41

What is a langerin?

protein found inside the Birbeck granules which are found in Langerhans Cell Histiocytoses

42

HLA-DR+, S100+, CD1+

Langerhans Cell

43

Differential diagnosis for an anterior mediastinal mass?

4 T's:

-Thymus - thymoma, thymic cyst
-Thyroid mass
-Teratoma (germ cell tumor)
-"Terrible" lymphoma - B (Hodgkin) or T cell (acute T cell)

44

What test monitors warfarin therapy?

PT

45

What test monitors heparin therapy?

PTT

46

3 year old + epistaxis + petechiae + upper respiratory infection last week + sudden onset of symptoms =

Treatment?

Acute immune thrombocytopenic purpura (ITP)

Treatment:
-self limiting in 6 months
-restriction of activity

47

What is platelet satelliteism?

Platelets clump around WBC and therefore the machine indicates thrombocytopenia even though there isn't. There is no risk of bleeding in the patient.
Occurs due to the EDTA anti-coagulant in the collection tubes.
Re-draw blood in a different type of tube (Citrate anti-coagulant).

48

What type of bleeding is: menorrhagia?

Platelet-type

49

What type of bleeding is: epistaxis?

Platelet-type

50

To dx sickle cell disease (2)

-Hemoglobin electrophoresis
-Metabisulfite screen

51

Antithrombin (AT) inhibits factors _

II
VII
IX
X
XI
XII

(main effect is via inhibition of II and X)

52

APC + PS complex inhibits factors _

V
VIII

53

30 year old Cacusaian female with multiple DVTs and a PE.
Patient's blood has normal PTT.
Add APC+PS to patient's blood and PTT does not change.

What mutation does the patient have?

Factor V Leiden mutation

Adding APC+PS to the patient's blood should have caused resulted in an increase in PTT due to the inhibition of factor V. If the patient has a resistant Factor V, then APC+PS will have no effect on it.

54

Vitamin K dependent factors =

II
VII
IX
X
protein C
protein S

55

a lack of CD59 + CD55 is indicated in?

Paroxysmal Nocturnal Hemoglobinuria

They are the markers that protect the RBC from compliment mediated lysis.

56

Where is factor VIII synthesized?

Endothelial cells

Along with VWF

57

Ristocetin aggregation test measures what?

vWF and GPIb adhesion/aggregation

58

Ristocetin aggregation test is negative/abnormal. What two diseases are indicated and how to tell them apart?

Abnormal ristocetin aggregation tests implicate vWF deficiency and Bernard-Souiler Syndrome.

vWF deficiency: aggregation will normalize when mixed with normal blood, patients will also have symptoms that indicate factor VIII deficiency

Bernard-Souiler Syndrome: aggregation will not normalize when mixed with normal blood, peripheral smear will also show large platelets

59

Diagnostic blood smear for malaria shows what two things?

-bananna shaped gametocytes
-intraerythrocyte ring forms

60

Heterophile antibodies are found in =

EBV-induced infectious mononucleosis

Monospot test is looking for heterophile antibodies

61

anaplastic cells definition and features (5)

neoplastic cells that demonstrate a complete lack of differentiation

features:
-loss of cell polarity and architecture
-cellular pleomorphism
-nucleus variations
-mitotic figures
-giant multinucleated tumor cells

62

CD14 =

surface marker of monocyte-macrophage lineage

63

Mutation in BRAF oncogene leads to what 2 cancers?

What is BRAF?

Hairy Cell Leukemia
Melanoma

BRAF = Ras signal transduction

64

HIV+ patient + large mass surrounding small intestine + uniform round basophilic cells with proliferation fraction (Ki-67) > 99% =

Burkitt Lymphoma

65

Proliferation fraction (Ki-67) indicates =

mitotic index

66

Genetics of sickle cell disease?

AR

Both parents have to have sickle cell trait (heterozygous carrier).

67

What does JAK2 gain of function mutation do?

Renders the Hematopoetic stem cells more sensitive to growth factors.

68

Waxing and waning painless lymphadenopathy + indolent =

Follicular lymphoma

69

hospitalized patient + prolonged PT + prolonged PTT + decreased # platelets + decreased fibrinogen + bleeding =

Disseminated Intravascular Coagulation
-consumption of coagulation factors and platelets and fibrinogen
-symptoms of bleeding due to the consumption

70

Trisomy 21 patients are likely to develop which leukemia (2 types)

-AML
-ALL

71

what bug is likely to induce an aplastic crisis in a sickle cell patient?

Parvovirus B19

72

Aplastic crisis definition

massive reduction in RBC synthesis

73

Mechanism of warfarin induced skin necrosis?

Decreased production of protein C -> prothrombotic state -> thromboses in the skin -> necrosis

74

"dewdrops on a rose petal" skin condition on a child

Varicella zoster - chicken pox

75

diagnosis of antiphospholipid antibody syndrome =

-2 findings of antiphospholipid antibody in the serum 12 weeks apart
-at least one clinical feature of the disease - thrombosis (arterial or venous), pregnancy morbidity

76

Iron deficiency anemia + Esophageal webs + Atrophic glossitis =

Plummer Vinson Syndrome

77

Dry tap (3)

-Hairy cell leukemia
-Aplastic anemia
-Myeloproliferative disorder - myelofibrosis

78

Leukemia with splenomegaly, dry tap, no lymphadenopathy =

Hairy cell leukemia

79

Blue color on Wright-Giemsa stain in reticulocytes is

residual rRNA

80

PECAM-1 is expressed on endotheial cells during

WBC transmigration

81

DIC in pregnancy is caused by =

placental injury leading to the release of tissue factor into maternal circulation

82

Decreased LAP vs Increased LAP

LAP = leukocyte alkaline phosphatase

decreased LAP = CML
(increased WBC with left shift)

increased LAP = leukemoid reaction (increased WBC with increased neutrophils + left shift)

83

Decreased haptoglobin indicates

intravascular hemolysis

RBC breaks -> releases hemoglobin -> hemoglobin + haptoglobin travel to the spleen

84

Increased MCHC =

hereditary spherocytosis

85

increased TT indicates =

direct or indirect thrombin inhibition

86

Increased PTT, PT, and TT indicates =

Direct Xa inhibitor

-Rivaroxaban
-Apixaban

87

Patients with ALL have an increased risk for (3)

-Down syndrome
-Ataxia telagiectasia
-Neurofibromatosis 1

88

65 year old man, neutrophils with two lobes, hypercellular bone marrow =

Myelodysplastic syndrome

Pseudo-Pelger-Huet anomaly = neutrophils with two lobes

89

Blood transfusion risks (3)

-Iron overload -> hemochromatosis
-Hypocalcemia due to citrate chelation (the lack of Ca is also the reason why the blood packets do not clot)
-Hyperkalemia due to RBCs lysing over time

90

Sideroblastic anemia inhibits what enzyme?

ALA Synthase or decreases the concentration of its cofactor (vitamin B6/pyridoxine)

91

Porphyria cutanea tarda inhibits what enzyme?

Uroporphyrinogen decarboxylase

92

Acute intermittent porphyria inhibits what enzyme?

PBG deaminase with a subsequent upregulation of activity of ALA synthase (because no product is made)

93

Lead poisoning inhibits what two enzymes?

-Ferrochelatase
-ALA dehydratase

94

Basophilic stippling

Lead poisoning

95

Patient with blisters on backs of hands and forearms that heal with hyperpigmentation after rupturing. What enzyme is deficient?

Uroporphyrinogen Decarboxylase deficiency
-Porphyria cutanea tarda
-May also have tea colored urine due to the uroporphyrin build up and urinary excretion

96

Sickle cell trait has resistance to what infection?

P. falciparum

Only trait! (heterozygotes)

97

Things that cause warm agglutinins

SLE, CLL, Drugs (alpha-methyldopa)

-Produces IgG
-Positive direct coombs test
-Extravascular hemolysis

98

Anti-A Ab and Anti-B Ab in a woman with O type blood are what type of antibodies?

IgG - therefore they can cross the placenta and cause fetal RBC hemolysis

99

Anti-B Ab in a woman with A type blood are what type of antibodies?

IgM - therefore the cannot cross the placenta

Same with anti-A ab in a B type woman

100

What can be given to help treat the symptoms of folic acid deficiency?

Thymidine supplementation

Decreased folic acid -> decreased dTMP -> decreased DNA synthesis

Thymidine -> increased dTMP (via a different pathway) -> increased DNA synthesis

101

What receptor in the area postrema can be antagonized to treat chemotherapy-induced vomiting?

Neurokinin-1 (NK-1)

Antagonists block this receptor and therefore decrease substance P release

102

CYP450 inducers/inhibitors cause warfarin action to be enhanced? (increases risk of bleeding)

inhibitors

103

CYP450 inducers/inhibitors cause warfarin to be less effective? (decreases INR)

inducers

104

When does fetal hemoglobin become adult hemoglobin?

Fetal Hb production before at 8 weeks gestation

Major form from 14 weeks through the first few months (6 months?) of life

105

Fetal hemoglobin =

Alpha2 + Gamma2

106

53 yo man with frequent headaches, dizziness, sweating, facial plethora, splenomegaly and increased # RBCs

Polycythemia vera
Mutation in JAK2 - cytoplasmic tyrosine kinase (non-receptor tyrosine kinase)

107

Aplastic anemia + absent thumbs + short stature =

Fanconi anemia

108

Reddish skin discoloration + tachypnea + tachycardia + headache + dizziness

Cyanide poisoning -> inhibits cytochrome c complex in the ETC
Labs: lactic acidosis, narrowing of venous-arterial PO2 gradient
Treatment: make methemoglobin to soak up the cyanide

109

Osteoblastic/sclerotic bone metastases (3)

-Prostate cancer
-Small cell lung cancer
-Hodgkin lymphoma

Indicates a more indolent course

110

Osteolytic/lucent bone metastases (5)

-MM
-Non-small cell lung cancer
-Non-hodgkin lymphoma
-RCC
-Melanoma

Indicates a more aggressive course

111

G6DP genetics

X linked recessive

112

Hereditary spherocytosis genetics

Autosomal dominant

113

Pure red cell aplasia associations/causes (3)

Pure red cell aplasia = anemia (low RBC#) with normal platelet and WBC #

-Thymoma
-Lymphocytic leukemias
-Parvovirus B19

114

Heavy menstrual periods + increased nosebleeds =

Von WIllebrand disease

115

Tumor expansion/depth of invasion - stage or grade?

Stage

116

Microangiopathic hemolytic anemias - form schistocytes (6)

DIC
HUS
TTP
Mechanical damage
HELLP syndrome
SLE

117

To make the dx of CML, what cells do you need to see?

-Basophils
-Mature granulocytes

118

Which leukemia is most responsive to treatment?

ALL

119

How does uremic platelet dysfunction present?

Increased BT, normal PT, PTT, Platelet count.
Usually occurs in patients with ESRD/uremia. Improved with dialysis

120

Glutamic acid -> Lysine
in beta globin

HbC defect
-in beta globin gene (chr 11)
-AR

121

Glutamic acid -> Valine
in beta globin

Sickle cell anemia
-in beta globin gene (chr 11)
-AR

122

Two things that cause eosinopenia

-Cushings
-Corticosteroids

123

mechanism of pyruvate kinase deficiency hemolytic anemia

decreased pyruvate kinase -> decreased ATP production -> rigid RBCs -> increased activity of splenic red pulp to increase remove these bad RBCs = splenic hyperplasia

AR

124

Function of the thymic cortical epithelial cells in T cell maturation?

Positive selection

turns double positive T cells into single positive T cells

125

Function of the thymic medullary epithelial cells and dendritic cells in T cell maturation?

Negative selection

turns single positive T cells into mature helper or cytotoxic T cells

126

3 stimuli that cause sickling

-increased acidity
-decreased oxygen (increased altitude)
-decreased blood volume (dehydration)