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Step 1 > Hematology > Flashcards

Hematology Flashcards

1
Q

Neutrophilic Chemotactic Agents

A

C5a, IL-8, LTB4, kallikrein, platelet-activating factor

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2
Q

What binds CD14 on macrophage?

A

Lipid A from bacterial LPS –> septic shock

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3
Q

Basophilia

A

CML

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4
Q

What prevents mast cell degranulation?

A

Cromalyn sodium

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5
Q

Primary target of HIV?

A

CD4+

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6
Q

Which Ig crosses placenta?

A

IgG

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7
Q

ABO Hemolytic Disease of Newborn

A

Type O mom –> anti-A and anti-B IgG cross placenta –> mild jaundice in neonates that are type A, B, or AB

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8
Q

What do Weibel-Palade bodies produce?

A

P-selectin

vWF

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9
Q

Ristocetin?

A
  • Activates vWF to bind GpIb

- Failure of agglutination with ristocetin assay = vWF disease and Bernard-Soulier Syndrome

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10
Q

Acatnthocytes = “spur cells”

A

Liver disease, abatelipoproteinemia (cholesterol dysregulation)

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11
Q

Basophilic stippling

A

Lead poisoning

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12
Q

“Bite cell”

A

G6PD Deficiency

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13
Q

Echinocyte = “burr cell”

A

Renal/liver DZ, pyruvate kinase deficiency

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14
Q

Ringed Sideroblast

A

Sideroblastic anemia - increased Fe in mt

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15
Q

Schistocyte = “helmet cell”

A

DIC
TTP/HUS
HELLP
Mechanical hemolysis

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16
Q

Spherocyte (no central pallor)

A 
Hereditary spherocytosis (spectrin, ankyrin)
Drug- and infection-induced hemolytic anemia
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17
Q

Target Cell (decreased cytoplasm or increased membrane)

A

HbC Disease
Asplenia
Liver DZ
Thalssemia

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18
Q

Heinz Bodies

A

G6PD Deficiency (Hb precipitation)

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19
Q

Howell-Jolly Bodies

A

Functional hyposplenia or asplenia

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20
Q

Plummer Vinson Syndrome

A

Fe deficient anemia + esophageal webs + dysphagia

- and atrophic glossitis (beefy red tongue)

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21
Q

Labs in Fe deficient anemia?

A 
Decreased iron
Increased TIBC (transport)
Decreased ferritin (decreased Fe stores)
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22
Q

Alpha Thalassemia Mutation

A
  • Alpha-globin gene deletion –> decreased alpha-globin synthesis
  • Cis: both deletions on same Chr (Asian)
  • Trans: deletions occur on separate Chr (African)
  • 4 allele deletion = no alpha-globin –> excess gamma-globin forms gamma4 = Hb Barts
  • 3 allele deletion = very little alpha-globin –> excess beta-globin forms beta4 (HbH)
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23
Q

Beta-Thalassemia Mutation

A
  • Point mutation in splice site and promotor sequence –> defective mRNA –> beta-globin synthesis decreased
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24
Q

How is beta-thalassemia minor confirmed?

A

Increased HbA2 on electrophoresis

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25
Q

What does peripheral smear look like of beta-thalassemia major?

A

Microcytic, hypochromic anemia
Target cells
Anisopoikilocytosis

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26
Q

Rx for beta-thalassemia major?

A

Blood transfusion –> risk of secondary hemochromatosis

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27
Q

Presentation of beta-thalassemia major?

A

“Crew cut” on skull x-ray
“Chipmunk” facies
Extramedullary hematopoeisis –> hepatosplenomegaly

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28
Q

Electrophoresis of beta-thalassemia major?

A

Increased HbF (alpha2gamma2)

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29
Q

What are beta-thalassemia major patients at increased risk for?

A

Parvovirus-B19 induced aplastic crisis

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30
Q

Lead poisoning enzymes

- Microcytic anemia

A

Ferrochelatase and ALA dehydratase

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31
Q

Buzzwords for lead poisoning

A 
BASOPHILIC STIPPLING
Led lines on gingivae (Burton lines) 
Sideroblastic anemia
Wrist/foot drop
Increased protoporphyrin
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32
Q

Treatment of lead poisoning

A

Dimercaprol and EDTA are 1st line

Succimer in kids

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33
Q

Sideroblastic Anemia Mutation

- Microcytic anemia

A

X-linked defect in S-ALA synthase gene (rate-limiting step in protoporphyrin synthesis)
- Requires B6 as cofactor

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34
Q

Causes of sideroblastic anemia?

A
  • Genetic
  • Acquired (myelodysplastic syndrome)
  • Reversible (alcohol, lead, B6 deficiency, copper deficiency, Isoniazid)
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35
Q

Sideroblastic anemia labs?

A
  • Increased iron
  • Normal/decreased TIBC
  • Increased ferritin
  • RINGED SIDEROBLAST (iron-laden Prussian blue-staned mt)
  • basophilic stippling
  • Decreased protoporphyrin
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36
Q

Treatment of sideroblastic anemia?

A

B6 (pyridoxine)

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37
Q

Which tapeworm can cause B12 deiciency?

A

Diphyllobothrium latum

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38
Q

Which spinal pathways are involved in subacute combined degeneration (B12 deficiency)?

A

Spinocerebellar (ataxia)
Lateral corticospinal tract (voluntary movement)
Dorsal column (P, vibration, fine touch, proprioception)

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39
Q

Orotic Aciduria Defect

- Macrocytic anemia

A

UMP Synthase defect –> can’t convert orotic acid to UMP (de novo pyrimidine synthesis pathway)
- AR

40
Q

Treatment of orotic aciduria?

A

Uridine monophosphate

41
Q

Presentation of orotic aciduria?

A
  • Kids
  • FTT, developmental delay, megaloblastic anemia refractory to folate or B12
  • NO hyperammonemia
42
Q

Diamond-Blackfan Anemia

A

Rapid-onset anemia within 1st year of life due to intrinsic defect in erythroid progenitor cells

43
Q

Intravascular Hemolysis Findings

  • Mechanical hemolysis
  • Paroxysmal nocturnal hemoglobinuria
  • Microangiopathic hemolytic anemias
A
  • Decreased haptoglobin (binds Hb, Hb is released into circulation)
  • Increased LDH
  • Schistocytes and reticulocyts on smear
44
Q

Extravascular Hemolysis Findings

A
  • Macs in spleen clear RBCs
  • Spherocytes
  • Increased LDH
  • Increased unconjugated bilirubin –> jaundice
45
Q

Anemia of Chronic Disease MOA

A
  • Increased hepcidin (IL-6)

- Decrease release of iron from Macs, decreased iron absorption from gut

46
Q

Anemia of Chronic DZ Findings

A
  • Decreased iron
  • Decreased TIBC
  • Increased ferritin
47
Q

Aplastic Anemia Findings

A
  • Decreased reticulocyte count
  • Increased EPO
  • Pancytopenia
48
Q

What does bone marrow look like in aplastic anemia?

A
  • Hypocellular bone marrow with fatty infiltration
49
Q

What is Fanconi anemia?

A
  • DNA repair defect –> bone marrow failure

- Short stature, increased incidence of tumors/leukemia, cafe-au-lait spots, thumb/radial defects

50
Q

What cells does parvovirus B19 infect?

A

Progenitor RBCs –> temporarily halts erythropoiesis

- Significant anemia in setting of preexisting marrow stress

51
Q

Hereditary spherocytosis defect?

A
  • RBC membrane skeleton and plasma membrane defect (ankyrin, spectrin)
52
Q

Hereditary spherocytosis findings?

A
  • Spherocytes
  • Increased MCHC
  • Splenomegaly
  • Aplastic crisis (parvovirus B19)
53
Q

What is the test for hereditary spherocytosis?

A

Osmotic fragility test + (glycerol)

54
Q

G6PD deficiency defect?

A
  • X-linked recessive defect in G6PD –> decreased NADPH –> decreased glutathione –> increased RBC susceptibility to oxidant stress (sulfa drugs, antimalrials, infections, fava beans)
55
Q

G6PD Deficiency Findings?

A
  • Back pain, hemoglobinuria

- Heinz bodies, bite cells

56
Q

Pyruvate Kinase deficiency defect?

A

AR

Defect in pyruvate kinase –> decreased ATP –> rigid RBCs –> extravascular hemolysis

57
Q

HbC Disease mutation

A
  • Glutamic acid –> lysine in Beta-globulin

- Extravascular hemolysis

58
Q

HbC blood smear?

A

Hemoglobin crystals inside RBCs

Target cells

59
Q

Paroxysmal nocturnal hemoglobinuria defect?

A

Impaired synthesis of GPI anchor for decay-accelerating-factor that protects RBC membrane from complement –> increased complement-mediated intravascular RBC lysis
- Acquired in hematopoietic stem cell

60
Q

PNHb triad?

A
  • Coombs negative hemolytic anemia
  • Pancytopenia
  • Venous thrombosis
61
Q

Labs in PNHb?

A

CD55/59 negative RBCs

62
Q

Treatment of PNHb?

A

Eculizumab (terminal complement inhibitor)

63
Q

Sickle Cell Anemia Defect

A

Point mutation - glutamic acid –> valine (neutral) –> extravascular and intravascular hemolysis

64
Q

Complications in sickle cell

A
  • Crew cut on skull X-ray
  • Aplastic crisis (B19)
  • Autosplenectomy –> increased risk of infection by encapsulated organisms (S. pneumo, H. flu)
  • Splenic infarct/sequestration crisis
  • Salmonella osteomyelitis
  • Painful crises (dactylitis, priapism, acute chest syndrome, avascular necrosis, stroke)
  • Renal papillary necrosis and microhematuria
65
Q

Sickle cells CoD?

A
  • Kids: encapsulated respiratory infection

- Adults: acute chest syndrome

66
Q

Sickle cell rx?

A

Hydroxyurea (increases HbF), hydration

67
Q

Warm AI Hemolytic Anemia

A

IgG

  • Chronic
  • SLE, CLL, certain drugs (alpha-methyldopa, penicilin)
68
Q

Cold AI Hemolytic Anemia

A

IgM and complement

  • Acute
  • Triggered by cold
  • Seen in CLL, mycoplasma pneumonia, mono
69
Q

AI Hemolytic Anemia lab?

A

+ Coombs

70
Q

Microangiopathic Anemia

A
  • Obstructed/narrowed vessel lumina
  • DIC, TTP/HUS, SLE, malignant HTN
  • SCHISTOCYTES
71
Q

Describe Parvovirus B19

A

SS nonenveloped DNA

linear and small

72
Q

Acute Intermittent Porphyria E?

A

Porphobilinogen Deaminase

- Increased porphobilinogen, S-ALA, coporphobilinogen in urine

73
Q

Presentation of acute intermittent porphyria?

A
  • Painful abdomen
  • Port wine colored urine
  • Polyneuropathy
  • Psych disturbances
  • Precipitated by drugs, alcohol, starvation
74
Q

Rx for acute intermittent porphyria?

A

Glucose and heme –> inhibits ALA-synthase

75
Q

Porphyria cutanea tarda E?

A

Uroporphyrinogen decarboxylase

- Urophoryrin elevated in urine (tea-colored urine)

76
Q

Porphyria cutanea tarda presentation?

A

Blistering cutaneous photosensitivity

77
Q

Labs in Hemophilia?

A

Increased PTT

78
Q

Treatment for hemophilia?

A

Desmopressin (vWF and factor VII)

  • Factor VIII concentrate (A)
  • Factor IX concentrate (B)
  • Factor XI concentrate (C)
79
Q

Vitamin K deficiency Labs?

A

Increased PT and PTT

80
Q

Difference between cogulation factor inhibitor or hemophilia?

A

PTT does NOT correct upon mixing normal plasma with patient plasma due to inhibitor (vs. hemophilia A)

81
Q

Liver failure’s effect on hemotology?

A
  • Decreased production of coag factors
  • Decreased activation of Vit K
  • Followed using PT
82
Q

Bernard-Soulier Syndrome

- QUALITATIVE Platelet Prob

A
  • Decreased GpIb –> defect in platelet-to-vWF adhesion
  • Large platelets (“big suckers”)
  • Increased BT
83
Q

Glanzmann Thromasthenia

- QUALITATIVE Platelet Prob

A
  • Decreased GpIIb/IIIa –> defect in platelet-to-platelet aggregation
  • Increased BT
84
Q

Hemolytic Uremic Syndrome Labs

- QUANTITATIVE platelet prob (decreased PC and BT)

A
  • Thrombocytopenia, microangiopathic hemolytic anemia, acute renal failure
  • KIDS w/ DIARRHEA (STEC O157:H7)
  • Rx: plasmapharesis
85
Q

Immune Thrombocytopenia

- QUANTITATIVE platelet prob (decreased PC and BT)

A

Anti-GpIIb/IIIa Ab –> splenic mac consumption of platelet-Ab complex

  • Viral illness
  • Increased megakaryocytes
  • Rx: steroids, IVIG, splenectomy
86
Q

Thrombotic Thrombocytopenic Purpura

- QUANTITATIVE platelet prob (decreased PC and BT)

A
  • Inhibition/deficiency of ADAMTS 13 = vWF metalloprotease –> decreased degradation of vWF –> large vWF multimers –> increased platelet adhesion –> increased platelet aggregation and thrombosis
87
Q

Labs on TTP?

A

Schistocytes, increased LDH

88
Q

Sx of TTP?

A

Pentad: neuro sx + renal sx + fever + thrombocytopenia + microangiopathic hemolytic anemia (schistocytes)

89
Q

von Willebrand Disease Labs

A
  • Increased BT (decreased platelet-to-vWF adhesion)
  • Increased PTT (intrinsic, vWF carries/protects factor VIII)
  • Abnormal ristocetin test (no platelet aggregation)
90
Q

vWF Disease Genetics

A

AD

91
Q

vWF Disease Rx

A

Desmopressin (releases vWF stored in endothelium [WP bodies])

92
Q

DIC Labs

A
  • Decreased platelet count
  • Increased BT
  • Increased PT
  • Increased PTT
  • Increased D-dimer (increased fibrin degradation products)
  • Schistocytes
  • Decreased fibrinogen
  • Decreased factors V and VIII
93
Q

DIC Pathogenesis

A

Widespread activation of clotting –> deficiency of clotting factors –> bleeding state

94
Q

Antithrombin Deficiency Labs

A
  • Normal PT and PTT
  • Diminishes increasing PTT following heparin administration
  • Also acquired through nephrotic syndrome
95
Q

Factor V Leiden Defect

A

Mutant factor V (G–> A DNA point mutation near cleavage site) –> resistant to degradation by protein C

  • Most common cause of hypercoagulability in Caucasians
  • Normal PT, unchanged when Protein C is added
96
Q

Protein C/S Deficnecy

A
  • Decreased ability to inactivate Va and VIIIa –> increased risk of THROMBOTIC SKIN NECROSIS WITH HEMORRHAGE AFTER WARFARIN ADMIN
97
Q

Prothrombin Gene Mutation

A

Mutation in 3’ untranslated region –> increased production of prothrombin –> increased plasma levels and venous clots

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