Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

STEP 1 > High Yield Topics-HemOnc > Flashcards

High Yield Topics-HemOnc Flashcards

1
Q

Describe order of primary hemostasis (platelet plug formation)

A
  1. Endothelial cell damage/injury
  2. Endothelial cell releases vWF and collagen
  3. vWF binds to platelets via Gplb
  4. Platelets release ADP and Ca+2
  5. ADP binds to platelets via P2Y12 –> platelet aggregation
  6. Fibrinogen binds to platelets via Gpllb/Gpllla –> forms mesh
  • platelet plug is temporary and is unstable –> can easily dislodge
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the coagulation factors of common pathway for coagulation cascade (secondary hemostasis)?

A

10, 5, 2

*STUDY AID: “COMMON” math problem
2 x 5 = 10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the coagulation factors of intrinsic pathway for coagulation cascade (secondary hemostasis)?

A

12, 11, 9, 8 —> 10, 5, 2

  • STUDY AID: write down 12-8 (exclude 10 b/c it’s already used for the common pathway)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the coagulation factors of extrinsic pathway for coagulation cascade (secondary hemostasis)?

A

7 –> 10, 5, 2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What test is used to measure intrinsic pathway?

A

PTT

  • STUDY AID: intrinsic pathway has more coagulating factors –> PTT (more letters)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What test is used to measure extrinsic pathway?

A

PT

  • STUDY AID: extrinsic pathway has less coagulating factors –> PT (less letters)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Which coagulation cascade pathway is slower?

A

Intrinsic

  • STUDY AID: intrinsic pathway has more coagulating factors –> takes longer
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What lab test is used to measure/monitor the effect of Heparin?

A

PTT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What lab test is used to measure/monitor the effect of Warfarin?

A

PT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What lab test(s) will be elevated when common pathway coagulating factors are deficient?

A

Both PT and PTT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What lab test will be elevated in platelet disorders?

A

Bleeding Time (BT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Immune-mediated platelet destruction that is usually idiopathic and follows a recent infection; caused by antibodies against GpIIb/IIIa antibodies; it’s a diagnosis of exclusion

A

Idiopathic Thrombocytopenic Purpura (ITP)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Abnormal lab finding for ITP

A

↑ Bleeding Time

Thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Clinical presentation of ITP

A

Increased bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

“BONE MARROW” biopsy finding for ITP

A

Megakaryocytes

  • no schistocytes on PBS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

A blood disorder due to deficiency of ADAMTS-13 that typically presents in females

A

Thrombotic Thrombocytopenic Purpura (TTP)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the function of ADAMTS-13?

A

A protease that degrades vWF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Describe the pathophysiology of TTP

A

ADAMTS-13 deficiency leads to ↓ degradation of vWF –> larger vWF (attached to endothelial cells) –> ↑ platelet adhesion and aggregation –> obstructed vessels –> destruction of RBCs as they pass through vessels –> intravascular hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Abnormal lab finding for TTP

A

↑ Bleeding Time

Thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Clinical presentation of TTP

A 
Thrombocytopenia
Anemia (hemolytic; ↓ Hb, schistocytes)
Renal Dysfunction (↑ creatinine)
Fever
Neurologic symptoms (confusion)
  • STUDY AID: TTP
    “ The Terrible Pentad”
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Blood smear finding for TTP

A

Schistocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Toxin-mediated hemolysis that is caused by Shiga-like toxin from EHEC infection that typically presents in children

A

Hemolytic Uremic Syndrome (HUS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Abnormal lab finding for HUS

A

↑ Bleeding Time
Thrombocytopenia
↑ Creatinine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Why does HUS present with ↑ Creatinine?

A

Toxin mainly affects the KIDNEY (glomerulus) by formation of platelet microthrombi (which causes obstruction and schistocytes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Clinical presentation of HUS

A

Kidney failure, BLOODY diarrhea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Blood smear finding for HUS

A

Schistocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What E. coli serotype causes HUS?

A

O157:H7

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

A blood disorder caused by widespread clotting factor activation; caused by many factors (sepsis, snake venom, trauma, transfusion)

A

Disseminated Intravascular Coagulation (DIC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Describe the pathophysiology of DIC

A

Sepsis/venom/trauma –> widespread clotting factor activation –> ↓ clotting factors, anticoagulant factors (protein C, protein S, antithrombin), platelets, and fibrinogen –> ↑ bleeding state

  • STUDY AID: DIC
    “ Damn I’m Clotting”
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Abnormal lab finding for DIC

A

↑ Bleeding Time
↑ PT/PTT
↑ D-Dimer

Thrombocytopenia
↓ Fibrinogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Clinical presentation of DIC

A

↑ Bleeding, sepsis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Blood smear finding for DIC

A

Schistocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

A blood disorder caused by vWF deficiency; most common inherited bleeding disorder that can present with heavy menstrual bleeding and prolonged bleeding after tooth extraction

A

Von Willebrand Disease (VWD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Inheritance pattern for Von Willebrand Disease (VWD)

A

Autosomal Dominant

  • remember that it is most common type of inherited bleeding disorder!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Describe the pathophysiology of Von Willebrand Disease (VWD)

A

↓ vWF –> ↓ factor VIII activity & ↓ platelet adhesion to vWF –> ↑ PTT (can be normal) & ↑ BT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What coagulating factor is carried and protected by vWF?

A

Factor VIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Tx for VWD

A

Desmopressin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

MOA of Desmopressin in treating vWD

A

Desmopressin ↑ vWF release from endothelium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

A bleeding disorder caused by GpIb deficiency

A

Bernard-Soulier Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What activity of platelet is defective in Bernard-Soulier Syndrome?

A

Platelet adhesion to vWF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Abnormal lab finding for Bernard-Soulier Syndrome

A

↑ Bleeding Time

Thrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Blood smear finding for Bernard-Soulier Syndrome

A

Large platelets

  • same size as RBCs!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

A bleeding disorder caused by GpIIb/GpIIIa deficiency

A

Glanzmann Thrombasthenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What activity of platelet is defective in Glanzmann Thrombasthenia?

A

Platelet to platelet aggregation due to lack of fibrinogen mesh formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Abnormal lab finding for Glanzmann Thrombasthenia

A

↑ Bleeding Time

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Blood smear finding for Glanzmann Thrombasthenia

A

No platelet clumping

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

A bleeding disorder caused by factor VIII deficiency

A

Hemophilia A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

A bleeding disorder caused by factor IX deficiency

A

Hemophilia B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

A bleeding disorder caused by factor XI deficiency

A

Hemophilia C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Inheritance pattern for each type of Hemophilia

A

Hemophilia A: x-linked recessive

Hemophilia B: x-linked recessive

Hemophilia C: AR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Abnormal lab finding for all three types of Hemophilia

A

↑ PTT

  • remember factors 8, 9, 11 are all intrinsic pathway coagulating factors!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

A bleeding disorder caused by ↓ activity of factors II, VII, IX, X, protein C, protein S

A

Vitamin K deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Abnormal lab finding for Vitamin K deficiency

A

↑ PTT

↑ PT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Conversion of fibrinogen to fibrin requires

A

Thrombin (factor IIa)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Aggregation of fibrin monomers to form fibrin mesh (fibrin polymer) requires

A

Factor XIIIa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Conversion of factor XIII to XIIIa requires

A

Thrombin (factor IIa)

  • thrombin activates both fibrinogen and XIII
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Protein C and S are regulatory ANTIcoagulant proteins that inhibit

A

Factors VIIIa and Va

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

A hyper-coagulability disorder caused by production of mutant factor V that is RESISTANT to degradation by protein C; presents with ↑ coagulating symptoms (DVT, pregnancy loss)

A

Factor V Leiden

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

MCV < 80 is ____ anemia

A

Microcytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

MCV=80-99 is ____ anemia

A

Normocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

MCV > 80 is ____ anemia

A

Macrocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Anemia caused by low iron level

A

Iron deficiency anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Iron deficiency anemia is considered (micro/macro/normocytic) anemia

A

Microcytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Iron Studies (Serum Iron, Ferritin, TIBC/Transferrin) for Iron deficiency anemia

A

Serum Iron: ↓
Ferritin: ↓
TIBC/Transferrin: ↑

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

Unique clinical presentations associated with Iron deficiency anemia

A

Pica and Koilonychia (nail spooning)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What disorder presents with a triad of esophageal webs, iron deficiency, and Dsyphagia?

A

Plummer-Vinson Syndrome

  • STUDY AID: “call Plumber When I Diarrhea”
  • W: Webs (esophageal)
  • I: Iron Deficiency
  • D: Dysphagia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

Anemia caused by defective heme synthesis (porphyrin pathway), which leads to iron overload/accumulation in mitochondria

A

Sideroblastic Anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

Genetic cause of Sideroblastic Anemia

A

ALA synthase deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

Acquired causes of Sideroblastic Anemia (2)

A
  1. Isoniazid (B6 deficiency)

2. Lead poisoning (inhibits ferrochelatase and ALA dehydratase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Sideroblastic Anemia is considered (micro/macro/normocytic) anemia

A

Microcytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Iron Studies (Serum Iron, Ferritin, TIBC/Transferrin) for Sideroblastic Anemia

A

Serum Iron: ↑
Ferritin: ↑
TIBC/Transferrin: ↓

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

Blood smear finding for Sideroblastic Anemia

A

ringed sideroblasts (basophilic stippling of iron granules in mitochondria of bone marrow cells)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Ringed sideroblasts are only visible by using what stain

A

Prussian blue stain (stain iron)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Anemia caused by defective hemoglobin production

A

Thalassemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Anemia caused by defective synthesis of ALPHA subunit(s) of hemoglobin

A

α Thalassemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Anemia caused by defective synthesis of BETA subunit(s) of hemoglobin

A

β Thalassemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

Normal Hb subunits

A

α2β2

  • αα | ββ
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

__ (#) gene(s) deletion of alpha subunit of hemoglobin causes no anemia

A

1

  • αα | α\
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

__ (#) gene(s) deletion of alpha subunit of hemoglobin causes mild anemia

A

2

  • αα | \ (cis)
  • α\ | α\ (trans)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

__ (#) gene(s) deletion of alpha subunit of hemoglobin causes severe anemia

81
Q

__ (#) gene(s) deletion of alpha subunit of hemoglobin causes hydrops fetalis (can’t survive)

82
Q

(trans/cis) deletion of 2 alpha subunit genes in α Thalassemia is more common in Asians

83
Q

(trans/cis) deletion of 2 alpha subunit genes in α Thalassemia is more common in African Americans

84
Q

__ (#) gene(s) deletion of beta subunit of hemoglobin causes no anemia

A

1

  • β Thalassemia Minor
85
Q

__ (#) gene(s) deletion of beta subunit of hemoglobin causes severe anemia

A

2

  • β Thalassemia Major
86
Q

β Thalassemia is more prevalent in what ethnicty?

A

mediterranean descents

87
Q

Unique clinical feature of β Thalassemia Major

A

Chipmunk facies (due to increased hematopoiesis in other bones)

88
Q

What Hemoglobin type will be present in infants with β Thalassemia Major?

A

HbF (αα | γγ)

  • they will be asymptomatic until HbF is depleted
89
Q

What Hemoglobin type will be present in adults with β Thalassemia Major?

A

HbA2 (αα | δδ)

  • they will be symptomatic
90
Q

Thalassemia is considered (micro/macro/normocytic) anemia

A

Microcytic

91
Q

Blood smear finding for Thalassemia

A

Target Cells

92
Q

Anemia caused by chronic inflammation/infection

A

Anemia of Chronic Disease

93
Q

What peptide hormone is produced in Anemia of Chronic Disease to decrease gut absorption of iron and to stimulate iron storage?

94
Q

Where is Hepcidin made?

95
Q

What chronic disorders are associated with Anemia of Chronic Disease?

A

Kidney Disease
Autoimmune Disease
Rheumatologic Disease

  • STUDY AID: “listen to CD in KAR”
  • CD: anemia of Chronic Disease
  • K: Kidney Disease
  • A: Autoimmune Disease
  • R: Rheumatologic Disease
96
Q

Anemia of Chronic Disease is considered (micro/macro/normocytic) anemia

A

Normocytic

97
Q

Iron Studies (Serum Iron, Ferritin, TIBC/Transferrin) for Anemia of Chronic Disease

A

Serum Iron: ↓
Ferritin: ↑
TIBC/Transferrin: ↓

98
Q

An intrinsic hemolytic anemia that presents with high hemoglobin level in urine due to a gene mutation

A

Paroxysmal Nocturnal Hemoglobinuria (PNH)

99
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH) is caused by mutation in what gene?

100
Q

Describe pathophysiology of Paroxysmal Nocturnal Hemoglobinuria (PNH)

A

PIGA gene mutation –> ↓ DAF (CD55) and ↓ MIRL (CD59) on RBCs –> RBCs are susceptible to MAC (membrane attack complex) lysis –> hemolysis –> hemoglobinuria esp. at night

101
Q

Lab findings of Paroxysmal Nocturnal Hemoglobinuria (PNH)

A

Pancytopenia
Negative Coombs Test

  • STUDY AID: PNH
  • Pancytopenia
  • Negative Coombs Test
  • Hibernating (occurs at night)
102
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH) is considered (micro/macro/normocytic) anemia

A

Normocytic

103
Q

An intrinsic hemolytic anemia that is caused by G6PD deficiency

A

G6PD Deficiency Anemia

104
Q

G6PD Deficiency Anemia is usually symptomatic after

A

oxidative stress

105
Q

What are the most common causes of G6PD Deficiency Anemia that increase oxidative stress?

A
  • Dapsone
  • Sulfa Drugs
  • Primaquine (malaria tx)
  • Fava beans
106
Q

What is the function of G6PD?

A

G6PD converts NADP to NADPH

107
Q

Describe pathophysiology of G6PD Deficiency Anemia

A

↓ G6PD –> ↓NADPH (necessary to reduce glutathione) –> ↓ reduced glutathione –> ↑ free radicals –> oxidative damage –> denatured hemoglobin

108
Q

G6PD Deficiency Anemia is considered (micro/macro/normocytic) anemia

A

Normocytic

109
Q

Blood smear finding for G6PD Deficiency Anemia

A
  1. Heinz bodies (denatured hemoglobin)

2. Bite cells (RBCs bitten by macrophages to remove heinz bodies)

110
Q

Inheritance pattern of G6PD Deficiency Anemia

A

X-linked recessive

111
Q

An intrinsic hemolytic anemia that is caused by defective RBC cytoskeleton proteins, leading to the loss of biconcave shape of RBCs

A

Hereditary Spherocytosis

112
Q

What cytoskeleton proteins are defective in Hereditary Spherocytosis?

A

Spectrin
Ankyrin
Bands

113
Q

Hereditary Spherocytosis is considered (micro/macro/normocytic) anemia

A

Normocytic

114
Q

Lab findings of Hereditary Spherocytosis

  • Know this cold!
A

↑ MCHC (dense RBCs)

↑ osmotic fragility (diagnostic test)

115
Q

Clinical presentations of Hereditary Spherocytosis

A
  • Splenomegaly (RBCs can’t easily pass through spleen due to its shape)
  • Negative Coombs test
  • Jaundice
116
Q

Blood smear finding for Hereditary Spherocytosis

A
  • Spherocytes

- Howell-Jolly bodies (nuclear remnants)

117
Q

How do you distinguish howell-jolly bodies from heinz bodies?

A
  • Heinz bodies are denatured hemoglobin so they contain iron –> needs special stain to be visualized
  • Howell-Jolly bodies are nuclear remnants so they do NOT contain iron
118
Q

Anemia caused by B12 or folate deficiency, leading to ↓ DNA synthesis

A

Megaloblastic Anemia

119
Q

What are the three common causes of B12 deficiency?

A
  1. Pernicious Anemia
  2. GI surgery (bariatric surgery, ileum resection)
  3. Malabsorption (crohn disease, celiac)
  • B12 storage is big, and therefore B12 deficiency due to malnutrition is rare and can take a long time to develop!
120
Q

What autoimmune disorder is caused by antibodies against gastric parietal cells or Intrinsic Factor?

A

Pernicious Anemia

121
Q

B12 deficiency is considered (micro/macro/normocytic) anemia

A

Macrocytic

122
Q

Lab findings of B12 deficiency anemia

A

↑ Homocysteine

↑ MMA

123
Q

What clinical symptom is only present in B12 deficiency anemia and not in folate deficiency anemia?

A

Neurological symptoms (due to lack of B12 needed to synthesize myelin sheath)

  • STUDY AID: think of dirty medicine’s MMA (knocking off ileum and the head)
124
Q

B12 absorption requires

A

Intrinsic Factor (IF)

125
Q

What cell produces Intrinsic Factor (IF)?

A

Gastric parietal cells

126
Q

B12 absorption occurs in what part of GI tract?

127
Q

What are the two common causes of folate deficiency?

A
  1. Malnutrition

2. Drugs (Methotrexate)

128
Q

Folate deficiency is considered (micro/macro/normocytic) anemia

A

Macrocytic

129
Q

Lab findings of folate deficiency anemia

A

↑ Homocysteine

  • normal MMA
130
Q

Blood smear finding for Megaloblastic Anemia (both types)

A

Hyper-segmented neutrophils

131
Q

How do you distinguish Von Willebrand disease from Bernard-Soulier since they both increase BT (affect primary hemostasis)?

A

Von Williebrand disease have normal platelet count and is the most common inherited bleeding disorder

132
Q

How do you distinguish ITP from Glanzmann thrombathenia since they are both caused by defective GpIIb/GpIIIa?

A

ITP: thrombocytopenia, normal PBS

Glanzmann Thrombasthenia: normal platelet count; no platelet clumping on PBS

133
Q

What intrinsic hemolytic anemias (2) are caused by a mutation in B-globin gene?

A
  1. Sickle Cell Anemia

2. HbC disease

134
Q

What single amino acid substitution occurs in sickle cell anemia?

A

Glutamic Acid –> Valine

  • missense mutation
135
Q

Mutant HbA in sickle cell anemia is called

136
Q

Describe pathogenesis of sickle cell crisis

A
  1. O2 unloading state (low O2 state, high altitude, acidosis, ↓2,3-BPG)
  2. Sickling (HbS polymerization/agglutination)
  3. Anemia, vaso-occlusion, pain
137
Q

Treatment for sickle cell crisis

A
  • Hydroxyurea (↑HbF)
  • Hydration
  • Gardos channel blockers (↓polymerization of HbS)
138
Q

How can sickling of RBCs in sickle cell anemia patients affect spleen?

A

Sickle cells can sequester in the spleen due to their abnormal shape and cause splenic infarction

  • functional asplenia
139
Q

Sickle cell anemia patients are at increased risk for what organisms?

A

Encapsulated organisms (strep. pneumo, H. flu, Neisseria meningitis, Salmonella)

140
Q

What is transient arrest of erythropoiesis due to bone marrow failure

A

Aplastic crisis

141
Q

Sickle cell anemia patients are prone to “aplastic crisis” due to infection by

A

Parvovirus B19

142
Q

Blood smear finding for Sickle Cell Anemia

A

Sickle cells

143
Q

X-ray finding for Sickle Cell Anemia

A

Crew cut on skull (due to marrow expansion from increased erythropoiesis)

144
Q

Heterozygotes (only one B-chain is affected) for sickle cell anemia

A

Sickle Cell Trait

145
Q

Patients with sickle cell trait have resistance against

146
Q

Hematologic lab findings for sickle cell trait

A

NORMAL hematologic values as general population

147
Q

What single amino acid substitution occurs in HbC disease?

A

Glutamic Acid –> Lysine

  • missense mutation
148
Q

Blood smear finding for HbC disease

A

Target cells

149
Q

What are the substrates/intermediates of Heme synthesis pathway?

A 
Glycine
Succinyl CoA
Aminolevulinic Acid (ALA)
Porphobilinogen
Hydroxymethylbilane
Uroporphyrinogen III
Coproporphyrinogen III
Protoporphyrin 
Heme

*STUDY AID: Get Some Additional Points Having Understood the Correct Pathway for Heme

150
Q

What is the rate limiting step of heme synthesis pathway, enzyme, and required cofactor?

A

Glycine + Succinyl CoA —> Aminolevulinic Acid (ALA)

  • Enzyme: ALA Synthase
  • Cofactor: B6
151
Q

What heme intermediate is produced in the mitochondria and gets transported out into cytosol?

A

Aminolevulinic Acid (ALA)

  • STUDY AID: going “a la” cytosol
152
Q

What heme intermediate is produced in the cytosol and gets transported back into mitochondria?

A

Coproporphyrinogen III

  • STUDY AID: “Cop” me some mitochondria
153
Q

What is the last step of heme synthesis pathway, enzyme, and required cofactor?

A

Protoporphyrin —> Heme

  • Enzyme: Ferrochelatase
  • Cofactor: Fe+2
154
Q

What enzyme converts Aminolevulinic Acid (ALA) to Porphobilinogen?

A

ALA Dehydratase

155
Q

What enzyme converts Porphobilinogen to Hydroxymethylbilane?

A

Porphobilinogen Deaminase

156
Q

What enzyme converts Uroporphyrinogen III to Coproporphyrinogen III?

A

Uroporphyrinogen Decarboxylase

157
Q

Congenital sideroblastic anemia is caused by

A

ALA synthase deficiency

158
Q

Acquired sideroblastic anemia is caused by lead poisoning inhibiting what two enzymes?

A
  1. ALA dehydratase

2. Ferrochelatase

159
Q

Children are exposed to lead via

A

lead paint

160
Q

Adults are exposed to lead via

A

batteries, ammunition

161
Q

X-ray finding for Lead poisoning

A

Lead lines (deposition) on Metaphysis on long bones

162
Q

Blood smear finding for sideroblastic anemia caused by lead poisoning or ALA synthase deficiency

A

Basophilic stippling or ringed sideroblasts

163
Q

Porphyria (heme synthesis disorder) caused by Porphobilinogen Deaminase dysfunction

A

Acute Intermittent Porphyria

164
Q

Clinical presentations of Acute Intermittent Porphyria

A
  • Port wine colored urine
  • Painful abdomen
  • Polyneuropathy
  • P450 inducers
  • Psychological symptoms
  • STUDY AID: The 5 P’s
165
Q

Porphyria (heme synthesis disorder) caused by Uroporphyrinogen Decarboxylase dysfunction

A

Porphyria Cutanea Tarda

166
Q

What are the acquired cause of Uroporphyrinogen Decarboxylase dysfunction?

167
Q

Clinical presentations of Porphyria Cutanea Tarda

A

Blistering hyperpigmentation

Photosensitivity

168
Q

Porphyria Cutanea Tarda is exacerabated by

A

Alcohol consumption

169
Q

The most common type or porphyria

A

Porphyria Cutanea Tarda

  • this means it’s higher yield!
170
Q

Lymphoid or myeloid neoplasm with widespread involvement of bone marrow; tumor cells are usually found in peripheral blood

171
Q

Describe Dirty Medicine’s spectrum for “Leukemias”

A

ALL CML AML CLL

  • ALL and CLL for “lateral” sides of the spectrum
  • CML and AML for “medial” sides of the spectrum
172
Q

Describe Dirty Medicine’s way of distinguishing b/w LYMPHOID vs. MYELOID leukemias on CBC w/ diff

A

Lymphoid Leukemia: ↑ lymphocytes (T-cells, B-cells)

Myeloid Leukemia: ↑ Neutrophils

173
Q

Describe Dirty Medicine’s way of distinguishing b/w ACUTE vs. CHRONIC leukemias

A

Acute leukemia: Immature cells

Chronic leukemia: Mature cells

174
Q

Describe Dirty Medicine’s spectrum for the most affected age group for each “Leukemia” type

A

ALL CML AML CLL

10 40 50 80

175
Q

Which leukemia is + for TdT marker?

176
Q

Which leukemia has Philadelphia chromosome (t: 9:22)?

177
Q

Which leukemia is + for myeloperoxidase stain?

178
Q

Which leukemia has Auer rods visible on blood smear?

179
Q

Tx for ALL

A

Cytarabine (aka. ARA-C)

180
Q

Tx for CML

A

Bcr-abl tyrosine kinase inhibitors

- Imatinib

181
Q

Tx for AML

A

All-trans retinoic acid (ATRA)

aka. Vitamin A

182
Q

B cell, T cell, and NK cells all arise from

A

lymphoid stem cell

183
Q

Erythrocyte, platelet, granulocytes, and macrophage all arise from

A

myeloid stem cell

184
Q

Which leukemia is associated with Down syndrome?

185
Q

Polycythemia vera is a disorder of ↑ RBCs due to acquired mutation of

186
Q

Clinical presentations of Polycythemia vera

A
  • Aquagenic pruritus (itching after shower)

- Erythromelalgia (severe, burning pain and red/blue coloration of extremities)

187
Q

Lab finding of Polycythemia vera

A

↑ RBCs

↓ EPO (erythropoietin)

188
Q

How do you distinguish b/w primary vs. secondary polycythemia vera?

A

Primary: ↑ RBCs and ↓ EPO
Secondary: ↑ RBCs and ↑ EPO

  • secondary polycythemia vera is caused by ↑ EPO
189
Q

Tx for polycythemia vera

A

Phlebotomy

Ruxolitinib (JAK 1/2 inhibitior)

190
Q

Uncontrolled proliferation and bone marrow infiltration by malignant PLASMA cells

A

Multiple Myeloma

191
Q

Clinical presentation of Multiple Myeloma

A
  • hyperCalcemia
  • Renal involvement
  • Anemia
  • Back Pain
  • STUDY AID: CRAB
192
Q

Describe how multiple myeloma can cause anemia

A

Bone marrow infiltration by malignant PLASMA cells → suppression of hematopoiesis → leukopenia, thrombocytopenia, anemia

193
Q

Describe how multiple myeloma can cause renal dysfunction

A

Overproduction of monoclonal immunoglobulin and/or light chains –> ↑ excretion –> kidney damage

194
Q

Which type of immunoglobulin is overproduced in multiple myeloma?

A

IgG > IgA

  • IgG in 55% of cases!
195
Q

X-ray finding for Multiple Myeloma

A

“Punched out” holes (bone lytic lesions) in the skull

196
Q

Blood smear finding for Multiple Myeloma

A

Rouleaux formation

  • RBCs are stacked in a row
197
Q

Bone marrow biopsy finding for Multiple Myeloma

A

> 10% monoclonal plasma cells with clock-face chromatin

198
Q

CML can progress into what other type of leukemia?

A

AML

*20% chance of CML to become AML!

STEP 1 (19 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions