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HEME Lymph > MIDTERM > Flashcards

MIDTERM Flashcards

1
Q

3 Etiologies of ANEMIA

A

Blood LOSS
DEC Production
INC Destruction

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2
Q

Acute vs. Chronic Etiologies Blood Loss

A

Acute Trauma/hemorrahage
Chronic: GI/GYN lesions, malignancies

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3
Q

Myelophthisic anemia, aplastic anemia and pure red cell dysplasia are due to problems in what?

A

Problem with Bone Marrow

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4
Q

What proinflammatory cytokine is seen in anemia

A

IL-6

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5
Q

= negative regulator of Fe metabolism

A

HEPCIDIN = inhibitor

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6
Q

RELATIVE causes

Reduction of plasma volume leading to hemoconcentration

Ex: Vomiting, Diarrhea and Polyuria

A

Polycythemia – Erythrocytosis

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7
Q

Polycythemia Absolute Causes: PRIMARY OR SECONDARY?

abnormal proliferation of myeloid stem cells with normal or low EPO levels

Ex: Polycythemia Vera

A

PRIMARY

problem at bone marrow

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8
Q

Polycythemia Absolute Causes: PRIMARY OR SECONDARY?

proliferation of myeloid stem cells in the setting of elevated EPO levels

A

SECONDARY

no problem at bone marrow – too much stimulus

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9
Q

Where is EPO produced and what does it stimulate?

A

Produced by kidneys, stimulates RBC synthesis in bone marrow

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10
Q

What can cause elevated EPO?

A

LOW O2
* chronic lung disease
* cyanotic heart disease
* high alt living
* EPO secreting tumors
* intake of EPO (athletes)

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11
Q

Hematopoietic stem cell –> Myeloid stem cell –> proerythroblast –> reticulocyte –> RBC

A

HEMATOPOIESIS

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12
Q

immature RBCs
too many of these means the body is losing RBCs faster than they can make them

Hemolysis hemorrhage

A

RETICULOCYTES

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13
Q

What is the function of HEPCIDIN?

A

inhibit Fe absorption from GI, sequesters Fe in liver and macrophages

release of IL6 –> Hepcidin release from liver –> DEC serum Fe

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14
Q

measures volume percentage of RBC in blood

A

HEMATOCRIT (HCT)

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15
Q

measure of the amount of Hgb per cell

A

MCH
mean corpuscular hb

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16
Q

measure of the concentration of Hgb in each RBC

A

MCHC
mean corpscular hb concentration

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17
Q

What protein STORES iron and what protein TRANSPORTS iron?

A

Stores – Ferritin
Transport – Transferrin

TIBC = how much Fe can be bound to transferrin

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18
Q

IRON Studies

A
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19
Q

Hemoglobin Electrophoresis

Order the Hb travelled from furthest to least travelled

Hb = neg charge – travells to anode

A

HbA –> HbF –> HbS –> HbC

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20
Q

Characterization based on morphology

small and pale RBCs
less than normal amt of Hb
MCV = < 80 fL

A

Hypochromic/Microcytic

Hb prob – iron def. anemia, thalassemia, anemia of chronic disease

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21
Q

Characterization based on morphology

normal color and size RBCs
MCV = 80 – 100 fL

A

Normocytic/Normochromic
NOT ENOUGH CELLS

hereditary spherocytosis, sickle cell, acute blood loss, myelofibrosis

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22
Q

Characterization based on morphology

cells are larger than usual
MCV = > 100fL

A

MACROYTIC
DNA PROBLEM

B12 deficiency anemia, folate deficiency

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23
Q

Where is TPO made ?

A

LIVER

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24
Q

Petechiae are a sign of what?

non-blanching, red lesions less than 2cm

A

THROMBOYCTOPENIA

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25
Q

Thrombocytopenia - which microangiopathic hemolytic anemia

Etiology: GI infection, pregnancy, autoimmune
TRIAD: Thrombocytopenia, Hemolysis , Kidney Disease

TREATMENT = plasmapheresis

A

Hemolytic Uremic Syndrome

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26
Q

Thrombocytopenia - which microangiopathic hemolytic anemia

Etiology: ADAMTS 12 deficiency
PENTAD: fever, anemia (hemolysis), thrombocytopenia, Renal failure, neurologic symptoms (encephalopathy)

FAT RN

A

Thrombotic Thrombocytopenic Purpura
TTP

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27
Q

thrombocytopenia

CAUSE = autoab against platelets cause thrombocytopenia
Assoc. Dx = SLE, infection, HIV
Signs/Symptoms = epistaxis, menorrhagia, petechiae

A

Immune Thrombocytopenic Purpura (ITP)

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28
Q

What causes an issue in coagulation?

A

Decreased or dysfunctional clotting factors

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29
Q

Where are clotting factors synthesized?

A

LIVER

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30
Q

What is an important cofactor of the coagulation cascade?

A

VITAMIN K

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31
Q

What disease?

Autosomal Dominant
Defect in Factor 8 & vWF complex
Symptoms: excessive bleeding, easy brusing, menorrhagi, prolonged bleeding

Treatment = DESMOPRESSIN

A

Von Willebrand disease

labs show DEC ristocetin cofactor assay

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32
Q

What disease?

X-Linked Recessive
Factor 8 deficiency

Treatment = Factor 8

A

Hemophilia A

**Hemophilia B = Factor 9 deficiency **

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33
Q

Mononucleosis is caused by what?

lymphocytosis of activated, CD8+ T cells (atypical lymphocytes)

A

EBV

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34
Q

severe neutropenia =
Risk of infection : < 500 cells /uL

treatment = G-CSF

A

AGRANULOCYTOSIS

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35
Q

inherited intrinsic defect in RBC membrane (ankyrin,
spectin, band3)

A

heriditary spherocytosis

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36
Q

inherited defect in the hemoglobin molecule B globin chain

A

Sickle cell anemia

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37
Q

inherited disorders in globin genes that decrease the synthesis of alpha or beta globin

A

Thalassima

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38
Q

acquired mutation in the PIGA gene

Dark Tea Urine, hemoglobinuria, blood clots in VEINS

A

paroxysomal nocturnal hemoglubinuria

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39
Q

caused by defects that increase destruction of RBCs by phagocytes in the spleen
Hyperbilirubinemia and jaundice

A

EXTRAVASCULAR

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40
Q

RBC explode within circulation due to injury of the RBC membrane
Hemoglobinemia, Hemoglobinuria and HEMOSIDERINURIA

Loss of iron

A

INTRAVASCULAR HEMOLYSIS

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41
Q

RBCs look more rounded with missing central pallor

A

Hereditary Spherocytosis

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42
Q

Screening test for hereditary Spherocytosis

A

Osmotic fragility

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43
Q

Pathogenesis: (HbS) Single amino acid substitution in B globin chains –> deoxygenated hemoglobin leading to distrotion of RBC shape

(HbC) LYSINE residue in place of GLUTAMIC ACID – only trait

A

SICKLE CELL ANEMIA
hemoglobinopathy/hereditary

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44
Q

Severe Hemolytic Anemia is due to what?

A

Severe membrane damage

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45
Q

Deoxygenation causes sickling →

A

Microvascular Occulusion

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46
Q

this disease is due to abnormally low production of alpha or beta globin chain

A

THALASSEMIA

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47
Q

Diagnosis of Alpha vs. Beta thalassemia

A

Alpha – small RBC
Beta – hemolytic anemia/high fetal HbF

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48
Q

Which chromosome is associated with Alpha and Beta Thalassemia

A

ALPHA = 16
BETA = 11

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49
Q

The severity of alpha thalassemia INCREASES based on what

A

3 genes –> 0 genes
MOST SEVERE = 0 genes present

Hb Barts

50
Q

Barts hemoglobin on screening newborns means what are present?

Hydrops fetalis (alpha thalassemia
major

A

Alpha gene deletions are present

Left Shift in Curve – No O2 to tissues– baby dies in utero

51
Q

Pt. presents with severe anemia w/ abnormal facial appearance, upper teeth protrusion, physical and mental delays

LOW reticulocyte count/crew cut skull X Ray

A

BETA THALASSEMIA MAJOR

52
Q

Pt. Presents with jaundice and splenomegaly, Labs show Hb and MCV decreased
Fe/RBC are normal
Blood Smear – microcytosis

A

Beta Thalassemia MINOR

53
Q

You see Heinz Bodies & Bite Cells on peripheral smear

A

G6PD Deficiency

G6PD reduces NADP+ to NADPH while oxd G6PD

54
Q

You see BURR cells on peripheral smear
Inhibits prod. of ATP and pyruvate by blocking glycolytic pathway

A

Pyruvate Kinase Deficiency

55
Q

hereditary, aut. recessive blood disorder in which large numbers of RBC are ellipitical in shape
RBC membrane defect
Most common defects are in genes for the polypeptides alpha spectrin and beta spectrin

A

ELLIPTOCYTOSIS (Ovalocytosis)

56
Q

In patients with iron loss name two serum lab tests that will change first?

A

Ferritin and TIBC

57
Q

Compared to a patient in NEG. IRON Balance, a patient with IRON DEFICINET ERYTHROPOIESIS, now has changes in what 3 lab tests?

A

DEC Serum Iron (SI) / % Saturation
INC RBC protoporhyrin

58
Q

IS IT AN UPPER OR LOWER GI BLEED

If a patients presents with Hematemesis, coffee ground emesis, nause/vomitting, epigastric pain, melena, or syncope?

A

UPPER GI BLEED

59
Q

IS IT AN UPPER OR LOWER GI BLEED

If a patient presented with any of the following: hematochezia, tenesmus, diarrhea

A

LOWER GI BLEED

60
Q

What are treatment options for anemia of chronic inflammation?

A

Treat the underlying condition
* Rhematoid Arthritis: with disease modifying agents (DMARDS)
* EPO for severe and symptomatic anemia

61
Q

what is an acute phase reactant that binds ferroportin –> DEC dietary iron transport and macrophage recycling (from dying RBCs)

A

HEPCIDIN

62
Q

A bone marrow biopsy shows FATTY HYPOCELLULAR bone marrow cells

A

APLASTIC ANEMIA

63
Q

What lab serum level is only elevated in B12 deficiency and not in folate deficiency?

A

Methylmalonic acid (MMA)

homocysteine is elevated in both

64
Q

Presence of Anti-intrinsic factor antibodies and anti-parietal cell antibodies are diagnostic for what?

A

PERNICIOUS ANEMIA

65
Q

pernicious anemia causes an INCREASED risk for what type of cancer?

A

GASTRIC CANCER

66
Q

Fe2+ is absorbed in the duodenum via what receptors?

A

DMT1 and HCP1 receptors

67
Q

PARENTERAL IRON Forms

What two ways can iron deficient anemia patients with malabsorption syndromes or pts. intolerant to oral iron be treated ?

A

Ferric hydroxide + dextran

Iron Sucrose

68
Q

Treatment for Iron TOXCITIY

A

administration of deferoxamine

69
Q

A decrease in production of what results in acquired penicious anemia

A

INTRINSIC Factor

70
Q

What oral B12 therapy is preferred because it is more highly protein bound and therefore remains longer in the circulation.

A

Hydroxocobalamin

71
Q

What symptoms differentiates folate deficiency and Vit B12 deficiency?

A

VIT B 12 –> NEURO SYMPTOMS

72
Q

What is the best treatment option for megaloblastic anemia?

folate can mask b12 deficiency/cause irreversible neuro dysfunction

A

Combo of FOLATE + CYANOCOBALAMIN

73
Q

What is the treatment for patients with anemia and chronic renal failure

A

EPO

74
Q

What is the major side effect of EPO therapy?

A

ELEVATED DIASTOLIC BP

75
Q

gram - rod

What pathogen is responsible for Cat-Scratch Disease?

Fever, malaise/swollen lymph nodes

A

Bartonella henselae

transmitted via cat saliva

76
Q

A patient presents with a nodular lymphangitis, says they were gardening

A

Sporothrix Schenckii
Cutaneous SPOROTRICHOSIS

DIMORPHIC FUNGI

77
Q

What disease is transmitted via SANDFLY

protozoan parasite

A

Leishmania

78
Q

Ixodes Tick causes what

Protozoa

A

Babesia
Blood Smear –> MALTESE CROSS pattern

79
Q

What are transfusion trigger symtoms?

A

serere blood loss
Collapse, air Hunger, Anuria (no urinary output)

80
Q

cryopreciptate and desmopressin (DDAVP) provide what?

used in tx of hemophilia A, von wilebrnad dx, hypofibrinogenemia

A

Factor 8, vVWF, and fibrinogen

81
Q

Lyophilized Factor IX has freeze dried factors ??

A

2, 7, 9, snd 10

82
Q

What determines the blood type?

A

Agglutinogens = RBC surface antigen

83
Q

What blood type = UNIVERSAL DONOR

A

O neg

univ. reciever = AB+

84
Q

febrile non-hemolytic reaction can occur during a transfusion due to what?

A

anti-wbc or anti-platelet antibodies insenistized patient

STOP TRANSFUSION

85
Q

patient recieved blood transfusion, now is in hypoxic resp distress, has pulm crackles w/o signs of CHF
CXR –> bilateral pulm edema
White out patchy infiltrates

A

Transfusion Related Acute Lung Injury

86
Q

Virchows Triad

A

Stasis of blood
Endothelial injury
Hypercoagable states

87
Q

Heparin/Heparan sulfate activates what

A

Antithrombin 3 –> inactivates 2, 9, 10, 11, 12

88
Q

protein C breaks down what factors

A

5 and 8

the Cat ate (8) my hand (5)

89
Q

most common inherited thrombophilia identified =

A

Factor V Leiden Mutation
factor 5 resist breakdown by protein C

90
Q

Prothrombin G20210A

A

pt. mutation where adenine subs for guanine at position 20210
END RESULT = higher circulating prothrombin

adenine = ADDs, guanine = GONE

91
Q

thrombosis (recurrent) +/-
morbidity associated with preg. complications
Positive AB: ABC
Lupus Anticoagulant
Anti Beta 2 glycoprotein 1 ab
Anto Cardiolipin ab

A

Antiphospholipid Syndrome

92
Q

Heparin Induced Thrombocytopenia – two types

A

Type 1 – non immune transient 30% dec in platelets - no thrombosis
Type 2 – immune mediated (ab complex with hep and PF4)

93
Q

Paroxysmal Nocturnal Hemoglobinuria

What proteins inhibit the complement pathway?

A

CD55DAF and CD59 MIRL

94
Q

Paroxysmal Nocturnal Hemoglobinuria

What is the anchor protein that anchors CD55DAF and CD59 MIRL to the membrane of the myeolid cell?

A

GPI
if theres a deficiency in this
Complement pathway INC

development of venous thrombosis in atypical placess

95
Q

What is the gold standard to detect absence of CD55 and CD59?

A

Flow Cytometry

96
Q

what binds exposed collagen?

A

vWF

97
Q

platelet adhesion uses what receptor

A

Gp-1b

98
Q

platelet aggregation uses what receptor?

want 2 b 3

A

Gp2b and 3a
and thromboxane TXa2= vasoconsrtictor

99
Q

labs show prolonged PTT

intrinsive pathway: 12, 11, 9, 8

A

Factor VIII deficiency = Hemophilia A

x-linked recessive

100
Q

How can DDVAP be used as a treatment for Hemophilia A

A

DDAVP (vasopressin) inc factor 8 stores by stimulating vWF from endothelial cells

101
Q

vWF is coupled with what factor?

A

Factor 8

important for platelet adhesion – bleeding time can be prolonged

102
Q

if the only deifciency is BLEEDING time then the problem is with what?

A

problem with platelets
BERNARD SOULIER DISEASE = defect with platelet adhesion

103
Q

what disorder has a defect in platelet aggregation?

A

glanzmann’s thrombastenia which decreases GP2b-3a

104
Q

what are the vitamin K dependent factors?>

A

2,7,9, 10 and C + S

105
Q

warfarin inactivates what vitamin?

A

VIT K

106
Q

Hemostatsis occurs in TWO stages

A
  1. Platelet pLug
    1. stabilize the platelet plug (coag cascade)
107
Q

platelets are granular cells derived from what?

A

Megakryocyets

108
Q

primary cells responsible for hemostasis

A

Platelets

109
Q

What hormone regulates the formation of platelets?

A

Thrombopoietin

110
Q

What is the first factor to join the intrinsic cascade?

A

factor 12

111
Q

How does factor 7 get activated in the Extrinsic pathway/?

A

It has to be exposed to Tissue Factor (Factor3)

112
Q

What factor is needed for cross-linking the fibrin mesh

A

Factor 13

113
Q

What factor is called the stuart prower factor

A

Factor 10

114
Q

What prevents formation of embolus?

A

Anticoagulation

115
Q

What cofactor is vital for coagulation?

A

Ca2+

116
Q

What vitamin is used in the production of Factors 2, 7, 9, and 10

A

VITAMIN K

kale, spinach, collards

absence of K – leads to uncontrolled bleeding

117
Q

What vitamin is used in the production of Factors 2, 7, 9, and 10

A

VITAMIN K

kale, spinach, collards

absence of K – leads to uncontrolled bleeding

118
Q

Vitamin K hydroquinone acts as a cofactor to

A

gamma-glutamly carboxylase

119
Q

how do we recyle vitamin K

A

vitamin K epoxide reductase

blood thinners INHIBIT this

warfarin or coumadin

120
Q

thrombodulin and thromin activate what protein

A

PROTEIN C

121
Q

deficiencies of proteins C and S can cause

A

more clotting than you need

HEME Lymph (2 decks)

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