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Uworld Journal > Hematology/Hematopathology > Flashcards

Hematology/Hematopathology Flashcards

1
Q

What (2) infections produce lymphocytic leukocytosis?

A
  • Viral infections (CD8+ cells increase)
  • Bordetella pertussis infection (bacteria produces lymphocytosis promoting factor which blocks lymphocytes from leaving the blood)
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2
Q

Mutations in the Myeloproliferative neoplasms (4)

A 
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

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3
Q

“starry sky” appearance

A

Burkitt Lymphoma

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4
Q

presentation: rapidly enlarging LN in an adult

A

Diffuse Large B Cell Lymphoma

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5
Q

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

A

Adult T Cell Lymphoma

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6
Q

Cerebriform nuclei

A

Mycosis fungoides/Sezary syndrome

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7
Q

Anisocytosis definition

A

variation in RBC size

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8
Q

Poikilocytosis definition

A

variation in RBC shape

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9
Q

What do the hepcidin levels indicate?

A

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)
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10
Q

Corrected reticulocyte count (%) equation

A

(% Retics observed x Hct)/ normal Hct

normal Hct = 45

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11
Q

Normal reticulocyte count =

A

1-2%

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12
Q

normal reticulocyte count response to anemia =

what does that indicate?

A

> 3%

indicates good marrow response and thus peripheral destruction

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13
Q

When do you need to correct the reticulocyte count?

A

Anemia = decrease in total RBCs can falsely elevate reticulocyte count

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14
Q

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

A

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

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15
Q

Indirect BR = unconjugated or conjugated

A

Indirect BR = Unconjugated BR

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16
Q

Direct BR = unconjugated or conjugated

A

Direct BR = Conjugated BR

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17
Q
  • Normocytic
  • Shistocytes
  • hemoglobinuria
  • jaundice
  • low serum haptoglobin
  • corrected Retic count > 3%
A

Intravascular hemolysis -> normocytic anemia

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18
Q
  • Normocytic
  • Jaundice
  • Spherocytes
  • BR gallstones
  • corrected Retic count > 3%
A

Extravascular hemolysi -> normocytic anemia

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19
Q

Cause of a corrected reticulocyte less than 3%

A

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

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20
Q

Corticosteroids ->

  • neutrophils?
  • lymphocytes?
  • eosinophils?
A
  • 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
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21
Q

Intrinsic clotting cascade players =

What test measures activity?

A

XII
XI
IX
VIII

Measured by aPTT

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22
Q

Extrinsic clotting cascade players =

What test measures activity?

A

VII

Measured by PT

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23
Q

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

thrombosis or bleeding?

A

Heparin-induced thrombocytopenia

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

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24
Q

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

pathogenesis?

A

Thrombotic thrombocytopenic purpura (TTP)

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

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25
Q

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

What disease?
Normal function of GPIb

A

Bernard-Soulier Syndrome

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

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26
Q

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

What disease?
Normal function of GPIIb/IIIa

A

Glanzmann Thrombasthenia

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

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27
Q

Budd-Chiari syndrome =

What (2) neoplasms is it seen in?

A

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

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28
Q
  • Lymphoblast cells >20% of bone marrow

- age less than 15 years old

A

Acute Lymphoblastic Leukemia (ALL)

  • B cell type in young kids
  • T cell type in older kids/adolescents and more of a mass (lymphoma)
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29
Q

t(8;14)

what genes are involved?

A

Burkitt lymphoma

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

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30
Q

t(14;18)

what genes are involved?

A

Follicular lymphoma

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

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31
Q

t(11;14)

what genes are involved?

A

Mantle cell lymphoma (MALToma)

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

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32
Q

t(15;17)

what genes are involved?

A

Acute promyelocytic leukemia

produces a PML/RARA -> abnormal retinoic acid receptor

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33
Q

Microcytic anemias (4)

A
  • Iron deficiency
  • Thalassemia
  • Sideroblatsic/lead poisoning
  • Anemia of chronic disease
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34
Q

Macrocytic anemias

  • megaloblastic (2)
  • non-megaloblastic (3)
A

Megaloblastic:

  • Folate deficiency
  • Vitamin B12 deficiency

Non-megaloblastic:

  • Alcohol
  • Liver disease
  • Bone marrow failure
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35
Q

autologous hematopoetic stem cell transplant =

A

patient is his/her own donor

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36
Q

allogenic hematopoetic stem cell transplant =

A

donor is related or unrelated but they are HLA-matched

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37
Q

syngenic hematopoetic stem cell transplant =

A

donor is the healthy identical twin

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38
Q

where are bone marrow transplants taken from?

A

posterior iliac crests

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39
Q

marker for the pluripotent hematopoetic stem cell

A

CD34+

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40
Q

Prophylaxis of acute GVHD?

A

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

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41
Q

What is a langerin?

A

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

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42
Q

HLA-DR+, S100+, CD1+

A

Langerhans Cell

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43
Q

Differential diagnosis for an anterior mediastinal mass?

A

4 T’s:

  • Thymus - thymoma, thymic cyst
  • Thyroid mass
  • Teratoma (germ cell tumor)
  • “Terrible” lymphoma - B (Hodgkin) or T cell (acute T cell)
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44
Q

What test monitors warfarin therapy?

A

PT

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45
Q

What test monitors heparin therapy?

A

PTT

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46
Q

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

Treatment?

A

Acute immune thrombocytopenic purpura (ITP)

Treatment:

  • self limiting in 6 months
  • restriction of activity
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47
Q

What is platelet satelliteism?

A

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).

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48
Q

What type of bleeding is: menorrhagia?

A

Platelet-type

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49
Q

What type of bleeding is: epistaxis?

A

Platelet-type

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50
Q

To dx sickle cell disease (2)

A
  • Hemoglobin electrophoresis

- Metabisulfite screen

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51
Q

Antithrombin (AT) inhibits factors _

A 
II
VII
IX
X
XI
XII

(main effect is via inhibition of II and X)

52
Q

APC + PS complex inhibits factors _

A

V

VIII

53
Q

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?

A

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
Q

Vitamin K dependent factors =

A 
II
VII
IX
X
protein C
protein S
55
Q

a lack of CD59 + CD55 is indicated in?

A

Paroxysmal Nocturnal Hemoglobinuria

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

56
Q

Where is factor VIII synthesized?

A

Endothelial cells

Along with VWF

57
Q

Ristocetin aggregation test measures what?

A

vWF and GPIb adhesion/aggregation

58
Q

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

A

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
Q

Diagnostic blood smear for malaria shows what two things?

A
  • bananna shaped gametocytes

- intraerythrocyte ring forms

60
Q

Heterophile antibodies are found in =

A

EBV-induced infectious mononucleosis

Monospot test is looking for heterophile antibodies

61
Q

anaplastic cells definition and features (5)

A

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
Q

CD14 =

A

surface marker of monocyte-macrophage lineage

63
Q

Mutation in BRAF oncogene leads to what 2 cancers?

What is BRAF?

A

Hairy Cell Leukemia
Melanoma

BRAF = Ras signal transduction

64
Q

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

A

Burkitt Lymphoma

65
Q

Proliferation fraction (Ki-67) indicates =

A

mitotic index

66
Q

Genetics of sickle cell disease?

A

AR

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

67
Q

What does JAK2 gain of function mutation do?

A

Renders the Hematopoetic stem cells more sensitive to growth factors.

68
Q

Waxing and waning painless lymphadenopathy + indolent =

A

Follicular lymphoma

69
Q

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

A

Disseminated Intravascular Coagulation

  • consumption of coagulation factors and platelets and fibrinogen
  • symptoms of bleeding due to the consumption
70
Q

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

A
  • AML

- ALL

71
Q

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

A

Parvovirus B19

72
Q

Aplastic crisis definition

A

massive reduction in RBC synthesis

73
Q

Mechanism of warfarin induced skin necrosis?

A

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

74
Q

“dewdrops on a rose petal” skin condition on a child

A

Varicella zoster - chicken pox

75
Q

diagnosis of antiphospholipid antibody syndrome =

A
  • 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
Q

Iron deficiency anemia + Esophageal webs + Atrophic glossitis =

A

Plummer Vinson Syndrome

77
Q

Dry tap (3)

A
  • Hairy cell leukemia
  • Aplastic anemia
  • Myeloproliferative disorder - myelofibrosis
78
Q

Leukemia with splenomegaly, dry tap, no lymphadenopathy =

A

Hairy cell leukemia

79
Q

Blue color on Wright-Giemsa stain in reticulocytes is

A

residual rRNA

80
Q

PECAM-1 is expressed on endotheial cells during

A

WBC transmigration

81
Q

DIC in pregnancy is caused by =

A

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

82
Q

Decreased LAP vs Increased LAP

A

LAP = leukocyte alkaline phosphatase

decreased LAP = CML
(increased WBC with left shift)

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

83
Q

Decreased haptoglobin indicates

A

intravascular hemolysis

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

84
Q

Increased MCHC =

A

hereditary spherocytosis

85
Q

increased TT indicates =

A

direct or indirect thrombin inhibition

86
Q

Increased PTT, PT, and TT indicates =

A

Direct Xa inhibitor

  • Rivaroxaban
  • Apixaban
87
Q

Patients with ALL have an increased risk for (3)

A
  • Down syndrome
  • Ataxia telagiectasia
  • Neurofibromatosis 1
88
Q

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

A

Myelodysplastic syndrome

Pseudo-Pelger-Huet anomaly = neutrophils with two lobes

89
Q

Blood transfusion risks (3)

A
  • 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
Q

Sideroblastic anemia inhibits what enzyme?

A

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

91
Q

Porphyria cutanea tarda inhibits what enzyme?

A

Uroporphyrinogen decarboxylase

92
Q

Acute intermittent porphyria inhibits what enzyme?

A

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

93
Q

Lead poisoning inhibits what two enzymes?

A
  • Ferrochelatase

- ALA dehydratase

94
Q

Basophilic stippling

A

Lead poisoning

95
Q

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

A

Uroporphyrinogen Decarboxylase deficiency

  • Porphyria cutanea tarda
  • May also have tea colored urine due to the uroporphyrin build up and urinary excretion
96
Q

Sickle cell trait has resistance to what infection?

A

P. falciparum

Only trait! (heterozygotes)

97
Q

Things that cause warm agglutinins

A

SLE, CLL, Drugs (alpha-methyldopa)

  • Produces IgG
  • Positive direct coombs test
  • Extravascular hemolysis
98
Q

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

A

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

99
Q

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

A

IgM - therefore the cannot cross the placenta

Same with anti-A ab in a B type woman

100
Q

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

A

Thymidine supplementation

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

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

101
Q

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

A

Neurokinin-1 (NK-1)

Antagonists block this receptor and therefore decrease substance P release

102
Q

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

A

inhibitors

103
Q

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

A

inducers

104
Q

When does fetal hemoglobin become adult hemoglobin?

A

Fetal Hb production before at 8 weeks gestation

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

105
Q

Fetal hemoglobin =

A

Alpha2 + Gamma2

106
Q

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

A

Polycythemia vera

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

107
Q

Aplastic anemia + absent thumbs + short stature =

A

Fanconi anemia

108
Q

Reddish skin discoloration + tachypnea + tachycardia + headache + dizziness

A

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
Q

Osteoblastic/sclerotic bone metastases (3)

A
  • Prostate cancer
  • Small cell lung cancer
  • Hodgkin lymphoma

Indicates a more indolent course

110
Q

Osteolytic/lucent bone metastases (5)

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

Indicates a more aggressive course

111
Q

G6DP genetics

A

X linked recessive

112
Q

Hereditary spherocytosis genetics

A

Autosomal dominant

113
Q

Pure red cell aplasia associations/causes (3)

A

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

  • Thymoma
  • Lymphocytic leukemias
  • Parvovirus B19
114
Q

Heavy menstrual periods + increased nosebleeds =

A

Von WIllebrand disease

115
Q

Tumor expansion/depth of invasion - stage or grade?

A

Stage

116
Q

Microangiopathic hemolytic anemias - form schistocytes (6)

A 
DIC
HUS
TTP
Mechanical damage
HELLP syndrome
SLE
117
Q

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

A
  • Basophils

- Mature granulocytes

118
Q

Which leukemia is most responsive to treatment?

A

ALL

119
Q

How does uremic platelet dysfunction present?

A

Increased BT, normal PT, PTT, Platelet count.

Usually occurs in patients with ESRD/uremia. Improved with dialysis

120
Q

Glutamic acid -> Lysine

in beta globin

A

HbC defect

  • in beta globin gene (chr 11)
  • AR
121
Q

Glutamic acid -> Valine

in beta globin

A

Sickle cell anemia

  • in beta globin gene (chr 11)
  • AR
122
Q

Two things that cause eosinopenia

A
  • Cushings

- Corticosteroids

123
Q

mechanism of pyruvate kinase deficiency hemolytic anemia

A

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

AR

124
Q

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

A

Positive selection

turns double positive T cells into single positive T cells

125
Q

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

A

Negative selection

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

126
Q

3 stimuli that cause sickling

A
  • increased acidity
  • decreased oxygen (increased altitude)
  • decreased blood volume (dehydration)

Uworld Journal (12 decks)

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