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HEMATOLOGY AND ONCOLOGY- Pathology Flashcards

1
Q

Alternative name for acanthocyte

A

Spur cell

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2
Q

What does Acantho means?

A

spiny

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3
Q

Associated pathology to Acanthocyte

A

Liver disease, abethalipoproteinemia states of cholesterol dysregulation)

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4
Q

Which pathologies are associated to Achantocytes?

A

Anemia of chronic disease
Alchol Abuse
Lead poisoning
Thalassemias

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5
Q

This type of cell is associated to G6PD deficiency

A

Bite cell

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6
Q

When do we see elliptocyte?

A

Hereditary elliptocytosis

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7
Q

Which atypic cells are seen in Megaloblastic anemia?

A

Hypersegmented PMNs

Macro- ovalocyte

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8
Q

When are Macro ovalocyte seen?

A

Megaloblastic anemia and Marrow failure

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9
Q

Which cells are characteristic of Sideroblastic anemia?

A

Ringed sideroblast

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10
Q

Which is the basis of Sideroblastic anemia?

A

Excess iron in mitochondria= pathologic

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11
Q

What explains Sideroblastic anemia?

A

The body has iron available but cannot incorporate it into hemoglobin, which red blood cells need to transport oxygen efficiently

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12
Q

Altenative name for Schistocyte

A

Helmet cell

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13
Q

When are Schistocytes seen?

A

DIC, Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic-Uremic Syndrome (HUS), traumatic hemolysis

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14
Q

Example of Traumatic hemolysis that can cause Schistocytes

A

Mechanical heart valve prosthesis

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15
Q

Which pathology is associated to Sickle cell?

A

Sickle cell anemia

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16
Q

When are Spherocyte seen?

A

Hereditary spherocytosis

Autoimmune hemolysis

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17
Q

When are Tear drop cells seen?

A

Bone marrow infiltration (eg myelofibrosis)

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18
Q

Target cells are seen in…

A

HbC disease, Asplenia, Liver disease, Thalassemia

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19
Q

What are Heinz bodies?

A

Are inclusions within red blood cells composed of denatured hemoglobin

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20
Q

How are Heinz bodies form?

A

Oxidation of Hemoglobin sulfhydryl group → denatured hemoglobin precipitation and phagocytic damage to RBC membrane → bite cells

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21
Q

Which stain is used to see Heinz bodies?

A

Crystal violet

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22
Q

When are Heinz bodies seen?

A

In G6PD deficiency

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23
Q

When are Heinz body-like inclussions seen?

A

In α thalassemia

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24
Q

What are Howell Jolly bodies?

A

Basophilic nuclear remnants foun in RBCs

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25
Q

What is the normal process of Howell Jolly bodies?

A

Howel Jolly bodies are normally removed from RBCs by splenic macrophages

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26
Q

In these situations we could see Howell Jolly bodies

A

In patients with functional hyposplenia or asplenia

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27
Q

First step to classify an anemia

A

Microcytic
Normocytic
Macrocytic

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28
Q

What detemines Microcytic anemia?

A

MCV (

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29
Q

Pathologies associated to Microcytic anemia

A

Iron deficiency (late)
ACD (Anemia of Chronic Disease)
Thalassemias
Lead poisnoning

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30
Q

Which factors may first present as normocytic anemia and the progress to a microcytic anemia?

A

Iron deficiency

Anemia of Chronic Disease

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31
Q

Which anemia is associated to Cooper deficiency?

A

Microcytic sideroblastic anemia

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32
Q

When is conseider Normocytic anemia?

A

MCV (50-100 fL)

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33
Q

How are Normocytic anemia classified?

A

Hemolytic

Nonhemolytic

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34
Q

When is called Nonhemolytic anemia?

A

Reticulocyte count normal or ↓

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35
Q

Non hemolytic Normocytic Anemias

A

ACD (Anemia of Chronic Disease)
Aplastic anemia
Chronic kidney disease
Iron deficiency (early)

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36
Q

How is Hemolytic Anemia diagnose?

A

With Reticulocyte count ↑

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37
Q

How is Hemolytic Normocytic anemia classified?

A

Intrinsic

Extrinsic

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38
Q

Intrinsic Hemolytic Normocytic anemias causes

A

RBC membrane defect (hereditary spherocytosis)
RBC enzyme deficiency (G6PD, pyruvate kinase)
HbC defect
Paroxysmal nocturnal hemoglobinuria
Sickle cell anemia

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39
Q

Extrinsic causes of Hemolytic Normocytic anemia

A

Autoimmune
Microangiopathic
Macroangiopathic
Infections

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40
Q

When is diagnose Macrocytic anemia?

A

MCV (>100 fL)

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41
Q

How are Macrocytic anemia classified?

A

Megaloblastic and non Megaloblastic

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42
Q

Megaloblastic anemias causes

A

Folate deficiency
B12 deficiency
Orotic aciduria

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43
Q

Causes of Non megaloblastic anemias

A

Liver disease
Alcoholism
Reticulocytosis

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44
Q

Which kind of anemia does Iron deficiency causes?

A

Microcytic, hypochromic anemia

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45
Q

Pathophysiology of Iron deficiency anemia

A

↓ iron due to chronic bleeding, malnutrition/absorption disorders or ↑ demand → ↓ final step in heme synthesis

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46
Q

Causes of iron deficiency due to chronic bleeding

A

GI loss

Menorrhagia

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47
Q

Causes of iron deficiency due to increase demand

A

Pregnancy

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48
Q

How are the labs in iron deficiency?

A

↓ iron
↑ Total iron-binding capacity
↓ ferritin

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49
Q

Clinical findings of iron deficiency

A

Fatigue

Conjuntival pallor

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50
Q

In microscope what are the findings?

A

Microcytosis and hypochromia

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51
Q

How can iron deficiency anemia may manifest?

A

Plummer Vinson syndrome

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52
Q

Characteristic of Plummer Vinson syndrome

A

Triad of iron deficency anemia, esophageal webs, atrophic glossitis

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53
Q

What is the defect in α Thalassemia?

A

α globin gene deletions → ↓ α globin synthesis

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54
Q

Types of deletion in α Thalassemia

A

Cis deletion

Trans deletion

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55
Q

Who suffer more cis deletion in α Thalassemia?

A

Asian population

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56
Q

In whom is more prevalent trans deletion of α thalassemia?

A

African population

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57
Q

Charactetistics of 4 allele deletion in α Thalassemia

A

No α-globin

Excess γ globin forms γ4

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58
Q

What is Hb Barts?

A

Excess γ globin forms γ4

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59
Q

Which kind of deletion in α Thalassemia is incompatible with life? Why?

A

4 allele deletion

Causes allele deletion

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60
Q

Which disease is caused by 3 allele α Thalassemia?

A

HbH disease

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61
Q

Characteristics of 3 allele α Thalassemia

A

Very little α globin

Excess β globin forms β4

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62
Q

What does Excess β globin causes?

A

HbH disease

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63
Q

What is the effect of 1-2 allele deletion in α Thalassemia?

A

No clinically significant anemia

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64
Q

Which kind of Anemia is β Thalassemia?

A

Microcytic, hypochromic anemia

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65
Q

What happens in β Thalassemia?

A

Point mutations in splice sites and promoter sequences→ ↓ β globin synthesis

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66
Q

In whom is more prevalent β thalassemia?

A

In mediterranen populations

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67
Q

Classification of β thalassemia

A

β thalassemia minor
β thalassemia major
HbS/ β thalassemia heterozygote

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68
Q

How else is β thalassemia minor known?

A

Heterozygote

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69
Q

How is β chain in β thalassemia minor?

A

Underproduced

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70
Q

Symptoms of β thalassemia minor

A

Usually asymptomatic

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71
Q

How is β thalassemia minor diagnosis confirmed?

A

By ↑ HbA2 (>3.5%) on electrophoresis

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72
Q

Alternative name for β thalassemia major

A

Homozygote

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73
Q

What is affected in β thalassemia major?

A

β chain is absent

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74
Q

How is anemia consider in β thalassemia major?

A

Severe anemia

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75
Q

What is required in β thalassemia major?

A

Blood transfusion

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76
Q

What could be the result of β thalassemia major?

A

Hemochromatosis due to requiring constant blood transfussions

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77
Q

Findings of β thalassemia major

A

Marrow expansion → Skeletal deformitis

“Chipmunk” facies

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78
Q

How is marrow expansion seen in β thalassemia major?

A

“Crew cut” on skull x ray

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79
Q

Complication of β thalassemia major

A

Extramedullary hematopoiesis (leads to hepatosplenomegaly)

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80
Q

From which microorganism is increased the risk of aplastic crisis in β thalassemia major?

A

Parvovirus B19 induced aplastic crisis

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81
Q

Structuraly how is HbF composed?

A

α2γ2

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82
Q

Type of Hb increased in β thalassemia major

A

Increased HbF

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83
Q

What is the benefict of increased HbF in β thalassemia major?

A

HbF is protective in tje infant and disease only becomes symptomatic after 6 months

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84
Q

Characterteristics of HbS/ β thalassemia heterozygote

A

Mild to moderate sickle cell disease depending on amoun of β globin production

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85
Q

What kind of anemia is produced by lead poisoning?

A

Microcytic, hypochromic anemia

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86
Q

Pathophysiology of Lead poisoning anemia

A

Lead inhibits ferrochelatase and ALA (Porphobilinogen synthase or ALA dehydratase, or aminolevulinate) → ↓ heme synthesis and ↑ RBC protoporphyrin

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87
Q

What other problem does Lead poisoning causes in RBC?

A

Also inhibits rRNA degradation, causing RBCs to retain aggregates of rRNA (basophilic stippling)

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88
Q

Risk factor that increases Lead poisoning

A

In Old houses with chipped paint

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89
Q

Findings of Lead poisoning

A

LEAD:
Lead lines on gingivae (Burton lines) and on methaphyses of long bones on x-ray
Encephalopathy and Erythrocyte basophilic stippling
Abdominal colic and sideroblastic Anemia
Drops- Wrist and foot drop

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90
Q

First line of treatment related to wrist and foot drop caused by lead poisoning

A

Dimercaprol and EDTA (Ethylenediaminetetraacetic acid)

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91
Q

In Lead poisoning what is the treatment for kids?

A

Succimer used for chelation for kids

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92
Q

Anemia caused by Sideroblastic anemia

A

Microcytic anemia, hypochromic

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93
Q

What is the problem of Sideroblastic anemia?

A

Defect in heme synthesis

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94
Q

Hereditary explanation of Sideroblastic anemia

A

X linked defect in δ ALA synthase gene

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95
Q

Causes of Sideroblastic anemia

A

Genetic
Acquired
Reversible

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96
Q

Causes of Acquired Sideroblastic anemia

A

Myelodysplastic syndromes

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97
Q

Reversible causes of sideroblastic anemia

A 
Alcohol is most common
Lead
vitamin B6 deficiency
Cooper deficiency
Isoniazid
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98
Q

Microscopic findings in Sideroblastic anemia

A

Ringed sideroblasts (with iron laden mitochondria) seen in bone marrow

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99
Q

Labs found ins Sideroblastic anemia

A

↑ iron, normal TIBC, ↑ ferritin

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100
Q

Treatment for sideroblastic anemia

A

Pyridoxine

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101
Q

Why is Pyridoxine administer for sideroblastic anemia?

A

Because B6 cofactor for δ ALA synthase

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102
Q

Pathophysiology of Megaloblastic anemia

A

Impaired DNA synthesis → maturation of nucleus of precursor cells in bone marrow delayed relative to maturation of cytoplasm

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103
Q

What could be one of the final results of Megaloblastic anemia?

A

Abnormal cell division→ pancytopenia

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104
Q

Causes of Folate deficiency causing Megaloblastic anemia

A

Malnutrition (eg, alcoholics), malapsorption, antifolates, Increased requirements

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105
Q

Antifolates drugs

A

Methotrexate
Trimethoprim
Phenytoin

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106
Q

Situations that increase Folate requirements?

A

Hemolytic anemia

Pregnancy

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107
Q

Findings in Folate deficiency

A

Hypersegmented neutrophils, glossitis, ↓ folate, ↑ homocysteine but normal methylmalonic acid

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108
Q

What clinical finding distinguish folate deficiency from vitamin B12 deficiency?

A

No neurologic symptoms in folate deficiency

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109
Q

Alternantive name for vitmain B12

A

Cobalamin

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110
Q

Causes of vitmain B12 deficiency

A

Insufficient intake (strict vegans), malabsorption (eg. Crohn disease), pernicious anemia, Diphyllobothrium latum (fish tapeworm), proton pump inhibitors

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111
Q

Findings of Vitamin B12 deficiency

A

Hypersegmented neutrophils, glossitis, ↓ B12, ↑ homocysteine, ↑ methylmalonic acid

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112
Q

Neurologic symptoms of Vitamin B12 deficiency

A 
Peripheral neuropathy with sensoriomotor disfunction
Dorsal columns (vibration/ propioception)
Lateral corticospinal (spasticity)
Dementia
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113
Q

Characteristics of Neurologic symptoms caused by Cobalamin deficiency

A

Subacute combined degeneration

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114
Q

What explains Subacute combined degeneration in Vitmain B12 deficiency?

A

Due to involvement of B12 in fatty acid pathways and myelin sinthesis

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115
Q

What kind of anemia does Orotic aciduria cause?

A

Megaloblastic anemia

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116
Q

Pathophysiology of Orotic aciduria

A

Inability to convert orotic acid to UMP because of defect in UMP synthase

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117
Q

In which process do we see the conversion from Orotic acid to UMP?

A

De novo pyrimidine synthesis pathway

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118
Q

Inheritance mode of Orotic aciduria

A

Autosomal recessive

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119
Q

In Whom is Orotic aciduria suspected? and how?

A

presents in children as megaloblastic anemia that canot be cured by folate or B12 with failure to thrive

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120
Q

Which deficiency could be compared with Orotic aciduria?

A

Orhithine transcarbamylase deficiency

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121
Q

What difference is classic in Orotic aciduria compared to Orhithine transcarbamylase deficiency?

A

No hyperammonemia in Orotic aciduria

Orhithine transcarbamylase deficiency presents with Increased orotic acid with hyperammonemia

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122
Q

Findings of Orotic Aciduria

A

Hypersegmented neutrophils, glossitis, orotic acid in urine

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123
Q

Treatment for Orotic Aciduria

A

Uridine monophosphate to bypass mutated enzyme

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124
Q

Main characteristic of Nonmegaloblastic macrocytic anemias

A

Macrocytic anemia in which DNA synthesis is unimpaired

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125
Q

Causes of Nonmegaloblastic macrocytic anemias

A

Liver disease
Alcoholism
Reticilocytosis
Drugs

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126
Q

Drugs that could cause Macrocytic anemias

A

5 FU
Zidovudine
Hydroxyurea

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127
Q

Findings of Intravascular hemolysis in Normocytic, normochromic anemias

A

↓ haptoglobin, ↑ LDH, schistocytes and ↑ reticulocytes on peripheral blood smear; and urobilinogen in urine

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128
Q

Examples of Intravascular hemolysis in Normocytic, normochromic anemias

A

Paroxysmal nocturnal hemoglobinuria
Mechanical destruction (aortic stenosis, prothetic valve)
Microangiopathic hemolytic anemia

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129
Q

Findings of extravascular hemolysis in Normocytic, normochromic anemias

A

Macrophages in spleem clears RBC
Spherocytes in peripheral smear, ↑ LDH plus ↑ unconjugated bilirubin, which causes jaundice (eg. hereditary spherocytosis)

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130
Q

Pathophysiology of Anemia of chronic disease

A

Inflammation → ↑ Hepcidine → ↓ release of iron from macrophages

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131
Q

What is the effect of Increased Hepcidin? and who releases it?

A

Released by liver, binds ferroportin on intestinal mucosal cells and macrophages , thus inhibiting iron transport

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132
Q

Lab Findings of Anemia of chronic disease

A

↓ iron, ↓ TIBC, ↑ ferritin

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133
Q

What could be the final stage of Anemia of chronic disease?

A

Can become microcytic, hypochromic

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134
Q

At the begining what type of anemia is Anemia of chronic disease

A

Nonhemolytic, normocytic anemia

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135
Q

What kind of anemia is Aplastic anemia?

A

Nonhemolytic, normocytic anemia

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136
Q

What causes Aplastic anemia?

A

By failure or destruction of myeloid stem cells

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137
Q

What causes destructuin of myeloid stem cells?

A

Radiation and drugs
Viral agents
Fanconi anemia
Idiopathic; may follow acute hepatitis

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138
Q

Drugs that can cause aplastic anemia

A

Benzene
Chloramphenicol
Alkylating agents
Antimetabolites

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139
Q

Viral agents related to Aplastic anemia

A

Parvovirus B19
EBV
HIV
HCV

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140
Q

What is the defect in Fanconi anemia?

A

DNA repair defect

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141
Q

What is the cause of idiopathic aplastic anemia?

A

Immune mediated, primary stem cell defect

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142
Q

Findings in Aplastic anemia

A

Pancytopenia, characterized by sever anemia, leukopenia, and thrombocytopenia
Normal cell morphology, but hypocellular bone marrow with fatty infiltration (dry bone marrow tap)

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143
Q

Symptoms of Aplastic anemia

A

Fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection

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144
Q

Possible treatment fot Aplastic anemia

A

Withdrawal of offending agent, immunosuppressive regimens, allogeneic bone marrow transplantation, RBC and platelet transfusion, G-CSF, or GM-CSF

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145
Q

Immunosuppressive regimens used in Aplastic anemia

A

Antithymocyte globulin

Cyclosporine

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146
Q

How does Chronic Kidney disease causes Nonhemolytic, normocytic anemia?

A

↓ EPO → ↓ hematopoiesis

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147
Q

Types of Intrinsic normocytic anemias

A

Extravascular

Intravascular

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148
Q

What kind of anemia is Hereditary spherocytosis?

A

Intrinsic normocytic anemias, Extravascular

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149
Q

What is the defect in Hereditary spherocytosis?

A

Defect in proteins interacting with RBC membrane skeleton and plasma membrane

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150
Q

Types of defects in proteins intertacting with RBC membrane skeleton and plasma membrane in Hereditary spherocytosis

A

Ankyrin, band 5, protein 4.2, spectrin

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151
Q

In hereditary spherocytosis, what happens if RBC has less memebrane?

A

Causes small and round RBCs with no central pallor → premature removal of RBCs by spleen

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152
Q

Which labs are identifiy in small and round RBCs?

A

↑ MHCH, ↑ red cell distribution width

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153
Q

Clinical findings of Hereditary spherocytosis

A

Splenomegaly, aplastic crisis (parvovirus B19 infection)

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154
Q

Labs found in Hereditary spherocytosis

A

Osmotic fragility test +

Normal to ↓ MCV with abundance of cells; masks microcytia

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155
Q

What screening test is useful for Hereditary spherocytosis?

A

Eosin 5 maleimide binding test

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156
Q

Treatment for Hereditary spherocytosis

A

splenectomy

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157
Q

What kind of anemia is G6PD deficiency?

A

Intrinsic normocytic anemias, Intravascular/Extravascular

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158
Q

Most common enzymatic disorder of RBCs

A

G6PD deficiency

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159
Q

Inheritance mode of G6PD deficiency

A

X linked recessive

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160
Q

Pathophysiology of G6PD deficiency

A

Defect in G6PD → ↓ glutathione → ↑ RBC susceptibility to oxidant stress

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161
Q

Classic causes of Hemolityc anemia following oxidant stress

A

Sulfa drugs, antimalarials, infections, fava beans

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162
Q

Clinical findings of G6PD deficiency

A

Back pain, hemoglobinuria a few days after oxidant stress

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163
Q

Labs found in G6PD deficiency

A

blood smear shows RBCs with Heinz bodies and bite cells

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164
Q

How is Pyruvate kinase deficiency classified?

A

Intrinsic hemolytic normocytic anemia, Extravascular

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165
Q

Inheritance mode of pyruvate kinase deficiency

A

Autosomal recessive

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166
Q

Pathophysiology of pyruvate kinase deficiency

A

Defecct in pyruvate kinase → ↓ ATP → rigid RBCs

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167
Q

How is pyruvate kinase deficiency diagnose?

A

Hemolytic anemia in a newborn

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168
Q

What kind of anemia is HbC defect?

A

Intrinsic hemolytic normocytic anemia, Extravascular

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169
Q

Pathophysiology of HbC defect

A

Glutamic acid to lysine mutation at residue 6 in β globin

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170
Q

Which has milder disease between HbSC and HbSS patients?

A

Patients with HbSC (1 of each mutant gene) have milder disease than have HbSS patients

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171
Q

What type of anemia is Paroxysmal nocturnal hemoglobinuria?

A

Intrinsic hemolytic normocytic anemia, Intravascular

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172
Q

Pathophysiology of Paroxysmal nocturnal hemoglobinuria

A

↑ complement- mediated RBC lysis

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173
Q

What is happens when ↑ complement- mediated RBC lysis during Paroxysmal nocturnal hemoglobinuria?

A

Impaired synthesis of GPI (glycosylphosphatidylinositol) anchor from decay accelerating factor that protects RBC membrane form complement

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174
Q

How is Paroxysmal nocturnal hemoglobinuria acquired?

A

Acquired mutation oin a hematopoietic stem cell

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175
Q

What risk is increased with Paroxysmal nocturnal hemoglobinuria ?

A

Increased incidence of acute leukemias

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176
Q

Triad of Paroxysmal nocturnal hemoglobinuria

A

Coombs - hemolytic anemia
Pancytopenia
Venous thrombosis

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177
Q

Labs found in Paroxysmal nocturnal hemoglobinuria

A

CD55/59 - RBCs on flow cytometry

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178
Q

Treatment for Paroxysmal nocturnal hemoglobinuria

A

Eculizumab

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179
Q

How is Sickle cell anemia classify?

A

Intrinsic hemolytic normocytic anemia, Extravascular

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180
Q

Causes a single amino acid replacement in β chain at position 6

A

HbS point mutation

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181
Q

What is subtitute in HbS point mutation?

A

Glutamic acid with valine

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182
Q

Pathogenesis of Sickle cell anemia

A

Low O2, dehydration, or acidosis precipitates sickling (deoxygenated HbS polymerizes), which results in anemia and vaso- occlusive disease

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183
Q

Why are Newborns are asymptomatic with sickle cell anemia?

A

Because of ↑HbF and ↓ HbS

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184
Q

From which infection do sickle cell anemia patients have resistance?

A

Heterozygotes (sickle cell trait) have resistance to malaria

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185
Q

Who carry HbS trait in Sickle cell anemia?

A

8% of African Americans carry the HbS trait

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186
Q

Which form do Sickle cell anemia have?

A

Sickle cells are crescent shaped RBCs

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187
Q

What is seen on X ray in sickle cell anemia?

A

“Crew cut” on skull x ray due to marrow expansion from ↑ erythropoiesis

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188
Q

Which pathologies present with “Crew cut” on skull x ray?

A

Sickle cell anemia and Thalasemias

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189
Q

Complications of Sickle cell anemia?

A 
Aplastic crisis (due to Parvovirus B19)
Autosplenectomy
Splenic sequestration crisis
Salmonella osteomyelitis
Painful crisis
Renal papilary necrosis and Microhematuria
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190
Q

What is seen in Autosplenectomy caused by sickle cell anemia?

A

Howell Jolly bodies

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191
Q

What could be the results of Autosplenectomy in sickle cell anemia?

A

↑ risk of infection with encapsilated organisms

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192
Q

What happens in childhood in patients with sickle cell anemia?

A

Early splenic dysfunction

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193
Q

What causes painful crisis in Sickle cell anemia?

A 
Vaso-occlusive
Painful hand swelling
Acute chest syndrome
Avascular necrosis
Stroke
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194
Q

What happens when vaso occlusive is present in sickle cell anemia?

A

Dactylitis

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195
Q

Most common cause of death in adult related to Sickle cell anemia

A

Acute chest syndrome

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196
Q

What is the cause of Renal papillary necrosis?

A

Due to low O2 in papilla, also seen in heterozygotes

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197
Q

In Sickle cell anemia patients, what causes microhematuria?

A

Medulalry infarcts

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198
Q

How is Sickle cell anemia diagnose?

A

Hemoglobin electrophoresis

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199
Q

Treatment of Sickle cell anemia?

A

Hydroxyurea and bone marrow transplantation

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200
Q

What happens when administer hydroxyurea in sickle cell anemia?

A

↑ HbF

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201
Q

Autoimmune hemolytic anemia is consider…

A

Extrinsic hemolytic normocytic anemia

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202
Q

How is Autoimmune hemolytic anemia classify?

A

Warm agglutinin

Cold aglutinin

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203
Q

What is the most common cause of warm and cold algutinin Autoimmune hemolytic anemia?

A

Idiopathic in etiology

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204
Q

Which immunoglobulin represents Warm agglutinin Autoimmune hemolytic anemia?

A

IgG

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205
Q

What pathologies are related to Warm agglutinin Autoimmune hemolytic anemia?

A

Chronic anemia seen in SLE, CLL, or with certain drugs

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206
Q

Example of drug that can cause Warm agglutinin Autoimmune hemolytic anemia

A

α methyldopa

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207
Q

Immunoglobulin that represents Cold agglutinin Autoimmune hemolytic anemia

A

IgM

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208
Q

What is the factor related to Cold agglutinin Autoimmune hemolytic anemia?

A

Acute anemia triggered by cold

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209
Q

Pathologies related to Cold agglutinin Autoimmune hemolytic anemia

A

Mycoplasma pneumonia infections, or infectious mononucleosis

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210
Q

Which lab test is usually positive in Autoimmune hemolytic anemia?

A

Coombs +

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211
Q

Characteristics of Direct Coombs test

A

Anti Ig antibody (Coombs reagent) added to patient’s blood

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212
Q

When is Direct Coombs test positive?

A

RBCs agglutinate if RBCs are coated with Ig

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213
Q

What happens in Indirect Coombs test?

A

Normal RBCs added to patients serum. If serum has anti Ig antibodies (Coombs reagent) added

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214
Q

What kind of anemia is Microangiopathic anemia?

A

Extrinsic hemolytic normocytic anemia

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215
Q

Pathogenesis of Microangiopathic anemia

A

RBCs are damaged when passing through obstructed or narrowed vessel lumina

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216
Q

When is Microangiopathic anemia seen?

A

In DIC, TTP-HUS, SLE amd malignant hypertension

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217
Q

Which cells are seen in Microangiopathic anemia?

A

Schistocytes (helmet cells) are seen on blood smear due to mechanical destruction of RBCs

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218
Q

How is Macroangiopathic anemia consider?

A

Extrinsic hemolytic normocytic anemia

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219
Q

Prosthetic heart valves and aortic stenosis may cause these types of anemia

A

Macroangiopathic anemia

Hemolytic anemia secondary to mechanical destruction

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220
Q

Cells seen in Macroangiopathic anemia

A

Schostocytes on peripheral blood smear

221
Q

Which anemia is cause by infections?

A

Extrinsic hemolytic normocytic anemia

222
Q

Examples of agents that cause infectious anemia

A

Malaria

Babesia

223
Q

What happens in Infectious anemia?

A

↑ destruction of RBCs

224
Q

Importance of ferritin

A

Primary iron storage protein of body

225
Q

Transport iron in blood

A

Transferrin

226
Q

In which situation is Serum iron decrease?

A 
Iron deficiency (primarily)
Chronic disease
227
Q

In this disease Serum iron is increased

A

Hemochromatosis (primarily)

228
Q

Which lab indirectly measures Transferrin?

A

TIBC (Total iron-binding capacity)

229
Q

Situation in which Transferrin is increase

A

Iron deficiency

Pregnancy/ OCP use (primarily)

230
Q

Situations when Transferrin production is increased

A

Pregnancy/ OCP use

231
Q

When is Transferrin decreased?

A

Chronic disease

Hemochromatosis

232
Q

In which pathologies is ferritin increased?

A 
Chronic disease (primarily)
Hemocromatosis
233
Q

When is ferritin decreased?

A

Iron Deficiency

234
Q

How is % transferrin saturation in Iron deficiency?

235
Q

How is % transferrin saturation in Hemochromatosis?

236
Q

How is % transferrin saturation in prenancy and OCP use?

237
Q

How is % transferrin saturation calculated?

A

Serum iron/ Total iron-binding capacity (TIBC)

238
Q

Types of Leukopenias

A

Neutropenia
Lymphopenia
Eosinopenia

239
Q

When is consider Neutropenia?

A

Absolute neutrophil count

240
Q

Causes of Neutropenia

A

Sepsis/ postinfection, drugs (including chemotherapy), aplastic anemia, SLE, radiation

241
Q

How is Lymphopenia consider

A

Absolute lymphocyte count

242
Q

Which Lymphocyte count are require in children to be classified as lymphopenia?

A

Absolute lymphocyte count

243
Q

Pathologies that cause lymphopenia

A

HIV

DiGeorge syndrome, SCID (severe combined immunodeficiency), SLE, corticosteroids, radiation , sepsis, postoperative

244
Q

Causes of Eosinopenia

A

Cushing syndrome, corticosteroids

245
Q

How do Corticosteroids affect Leukocytes?

A

Cause neutrophilia, but eosinopenia and lymphopenia

246
Q

What is the effect of Corticosteroids to Neutrophils?

A

↓ activation of neutrophil adhesion molecules, imparing migration out of the vasculature to sites of inflammation

247
Q

What is the effect of corticosteroids to Eosinophils?

A

Sequester eosinophils in lymph nodes

248
Q

What is the effect of corticosteroids to Lymphocytes?

A

Cause apoptosis

249
Q

What are porphyrias?

A

Are hereditary or acquired conditions of defective heme synthesis that lead to the accumulation of heme precursors

250
Q

What happens in lead poisoning?

A

Lead inhibits specific enzynes needed in heme synthesis, leading to similar conditions to Porphyrias

251
Q

Which is the affected enzyme in lead poisoning?

A

Ferrochelatase and ALA dehydrate

252
Q

What is the accumulated substrate in Lead posisoning?

A

Protoporphyrin, δ ALA (blood)

253
Q

Presenting symptoms of Lead poisoning

A

Microcytic anemia, GI and kidney disease

254
Q

How do children get Lead poisoning?

A

Exposure to lead paint

255
Q

Clinical manifestation of Lead poisoning in children

A

Mental deterioration

256
Q

How do Adults acquired Lead poisoning?

A

Enviromental expossure (battery/ ammunition/ radiator factory)

257
Q

Clinical pressentation of Lead poisoning in adults

A

Headache, memory loss, demyelination

258
Q

Porphobilinogen deaminase is the affected enzyme in this pathology

A

Acute intermittent deaminase

259
Q

Which enzyme is affected in Acute intermittent deaminase?

A

Porphobilinogen deaminase

260
Q

Accumulated substrate in Acute intermittent deaminase

A

Porphobilinogen, δ ALA, coporphobilinogen (urine)

261
Q

Symptoms of Acute intermittent deaminase

A 
5 P's:
Painful abdomen
Port wine-colored urine
Polyneuropathy
Psychological disturbances
Precipitated by drugs, alcohol, and starvation
262
Q

Treatment for Acute intermittent deaminase

A

Glucose and heme, which inhibit ALA synthase

263
Q

Uroporphyrinogen decarboxylase is the affected enzyme in this pathology

A

Porphyria cutanea tarda

264
Q

What enzyme is affected in Porphyria cutanea tarda?

A

Uroporphyrinogen decarboxylase

265
Q

Accumulated substrate in Porphyria cutanea tarda

A

Uroporphyrin (tea- colored urine)

266
Q

Presenting symptom of Porphyria cutanea tarda

A

Blistering cutaneous photosensitivity

267
Q

Most common porphyria

A

Porphyria cutanea tarda

268
Q

When is ALA synthase activity increased?

269
Q

When is ALA synthase activity decreased?

270
Q

What does PT test?

A

Test function of common and extrinsic pathway

271
Q

Test function of common and extrinsic pathway

272
Q

Which factors are part of extrinsic pathway?

A

I, II, V, VII and X

273
Q

Test function of common and intrisic pathway

274
Q

What does PTT evaluates?

A

Test function of common and intrinsic pathway

275
Q

Which factors are part of intrinsic pathway?

A

All factors except VII and XIII

276
Q

When extrinsic and common pathway have a defect, how is manifested?

277
Q

When is PT increased?

A

In extrinsic and common pathway defects

278
Q

When intrinsic and common pathway have a defect, how is manifested?

279
Q

When is PTT increased?

A

In intrinsic and common pathway defects

280
Q

Which coagulation times are alterated in hemophilia A or B?

281
Q

How is hemophilia A or B classified?

A

Intrinsic pathway coagulation defect

282
Q

What is deficient in hemophilia A?

A

Deficiency of factor VIII

283
Q

Which labs are abnormal in Hemophilia A?

284
Q

What is deficient in hemophilia B?

A

Deficiency of factor IX

285
Q

Which labs are abnormal in Hemophilia B?

286
Q

Clinical presentation of Hemophilia A or B

A

Macrohemorrhage in hemophilia- hemarthroses, easy bruising, ↑ PTT

287
Q

What is hemarthroses?

A

Bleeding into joints

288
Q

Treatment for Hemophili A

A

Recombinant factor VIII

289
Q

Which labs are abnormal in Vitamin K deficiency?

A

↑ PTT, ↑ PT

290
Q

How is Vitamin K consider?

A

General coagulation defect

291
Q

How is the bleeding time in Vitamin K deficiency?

292
Q

What is affected in Vitamin K deficiency?

A

↓ synthesis of factors II, VII, IX, X, protein C, protein S

293
Q

How is defect in platelet plug formation manifested?

A

↑ bleeding time

294
Q

How is platelet abnormalitie manifested?

A

Microhemorrhage: mucous membrane bleeding, epistaxis, petechiae purpura, ↑ bleeding time, possible ↓ platelet count

295
Q

How is Platelet count and Bleeding time in Bernard Soulier syndrome?

A

PC ↓ and BT ↑

296
Q

Platelet disorders

A

Bernard Soulier syndrome
Glanzmann thrombasthenia
Immune thrombocytopenia
Thrombotic thrombocytopenia purpura

297
Q

What is the problem in Bernard Soulier syndrome?

A

Defect in platelet plug formation

298
Q

This syndrome is characterized by ↓ GpIb

A

Bernard Soulier syndrome

299
Q

What is the consequence of ↓ GpIb?

A

Defect in platelet to vWF adhesion

300
Q

What is seen in Bernard Soulier syndrome?

A

Defect in platelet to vWF adhesion

301
Q

What is the main point in Glanzmann thrombasthenia?

A

Defect in platelet plug formation

302
Q

Which lab is affected in Glanzmann thrombasthenia?

A

Increased bleeding time, normal platelet count

303
Q

Syndrome Characterized by ↓ GpIIb / IIIa

A

Glanzmann thrombasthenia

304
Q

What is affected with ↓ GpIIb / IIIa?

A

Defect in platelet to platelet aggregation

305
Q

What is seen in Glanzmann thrombasthenia blood smear?

A

Shows no platelet clumping

306
Q

Labs found in Immune thrombocytopenia

A

↓ Platelet count

↑ Bleeding time

307
Q

What is the defect in immune thrombocytopenia?

A

Anti GpIIb/IIIa antibodies

308
Q

What is the result of Anti GpIIb/IIIa antibodies?

A

Splenic macrophage consumption of platelet/antibody complex

309
Q

What may triggers immune thrombocytopenia?

A

By viral illness

310
Q

What is the effect of immune thrombocytopenia in platelets?

A

Decreased platelet survival

311
Q

Finding of immune thrombocytopenia

A

Increased megakaryocytes on bone marrow biopsy

312
Q

Lab findings in thrombotic thrombocytopenic purpura

A

↓ Platelet count

↑ Bleeding time

313
Q

What is the explanation of thrombotic thrombocytopenic purpura?

A

Inhibition or deficiency of ADAMTS 13 (vWF metalloprotease)

314
Q

What is the result of Inhibition or deficiency of ADAMTS 13 (vWF metalloprotease)?

A

↓ Degradation of vWF multimers

315
Q

Pathogenesis of thrombotic thrombocytopenic purpura

A

↑ large vWF multimers →↑ platelet adhesion → ↑ platelet aggregation and thrombosis

316
Q

What is the final result of thrombotic thrombocytopenic purpura?

A

↓ Platelet survival

317
Q

Labs found in thrombotic thrombocytopenic purpura

A

Schistocytes, ↑ LDH

318
Q

Symptoms of thrombotic thrombocytopenic purpura

A

Pentad of neurologic and renal symptoms, fever, thrombocytopenia, and microangiopathic hemolytic anemia

319
Q

Treatment for thrombotic thrombocytopenic purpura

A

Exchange transfusion and steroids

320
Q

Labs affected in von Willebrand disease

A

Increase bleeding time

Normal or increased PTT

321
Q

Defects in von Willebrand disease

A

Intrinsic pathway coagulation defect

Defect in platelet plug formation

322
Q

What is the result of ↓ vWF in Intrinsic pathway coagulation defect?

A

Normal or ↑ PTT (depends on severity; vWF acts to carry/ protect factor VIII)

323
Q

What explains decrased of vWF lead to increased PTT?

A

vWF acts to carry/ protect factor VIII

324
Q

What is the result of ↓ vWF in Defect in platelet plug formation?

A

Defect in platelet to vWF adhesion

325
Q

How is von Willebrand disease consider?

A

Mild but most common inherited bleeding disorder

326
Q

Inheritance mode of von Willebrand disease

A

Autosomal dominant

327
Q

How is von Willebrand disease diagnose?

A

In most cases by ristocetin cofactor assay (↓ agglutination is diagnositic)

328
Q

Treatment for von Willebrand disease

A

DDAVP (Desmopressin), which releases vWF stored in endothelium

329
Q

Mixed platelet and coagulation disorders

A

von Willebrand disease

DIC

330
Q

Labs found in Disseminated intravascular coagulation

A

↓ Platelet Count
↑ Bleeding time
↑ PT
↓ PTT

331
Q

Pathophysiology of Disseminated intravascular coagulation (DIC)

A

Widespread activation of clotting lead to a deficiency in clotting factors, which creates a bleeding state

332
Q

Causes of DIC

A 
STOP Making New Thrombi
Sepsis 
Trauma
Obstetric complications
acute Pancreatitis
Malignancy
Nephrotic syndrome
Transfussion
333
Q

Findings of Disseminated intravascular coagulation

A

Schistocytes, ↑ fibrin split products (D- dimers), ↓ fibrinogen, ↓ Factor V and VIII

334
Q

Hereditary thrombosis syndromes leading to hypercoagulability

A

Factor V Leiden
Prothrombin gene mutation
Antitbrombin deficiency
Protein C or S deficiency

335
Q

Most common cause of inherited hypercoagulability in whites

A

Factor V Leiden

336
Q

What is the Factor V Leiden?

A

Production of mutant factor V that is resistant to degradation by activated protein C

337
Q

Pathophysiology of Prothrombin gene mutation

A

Mutation in 3’ untranslated region→ ↑ production of prothrombin ↑ plasma levels and venous clots

338
Q

How is Antithrombin deficiency gotten?

A

Inherited deficicency of antithrombin

And Acquired

339
Q

What effect does antithrombin deficiency has on PT, PTT, or thrombin time?

A

Has no direct effect on them

340
Q

Hois PTT affected by Antithrombin deficiency?

A

No direct effect on it, but diminishes the increase in PTT following heparin administration

341
Q

How can antithrombin deficiency be acquired?

A

Renal failure/ nephrotic syndrome → antithrombin loss in urine → ↑ factors II and X

342
Q

What is the result of Protein C or S deficiency?

A

↓ ability to inactivate factors V and VIII

343
Q

What risk is increased with Protein C or S deficiency?`

A

For Thrombotic skin necrosis with hemorrhage following administration of warfarin

344
Q

Components of Blood transfusion therapy

A

Packed RBCs
Platelets
Fresh frozen plasma
Cryoprecipitate

345
Q

What is the dosage effect of Packed RBCs?

A

↑ Hb and O2 carrying capacity

346
Q

Clinical use for Packed RBCs

A

Acute blood loss, severe anemia

347
Q

Dosage effect of Platelets

A

↑ Platelet count (↑ 5000/mm3/ unit)

348
Q

Which is the clinical use for Platelets as blood transfusion therapy?

A

Stop significant bleeding (thrombocytopenia, qualitive platelet defects)

349
Q

What is the purpose to administer fresh frozen plasma?

A

↑ coagulation factor levels

350
Q

Clinical use for Fresh frozen plasma

A

DIC, cirrhosis, warfarin overdose, exchange transfusion in TTP/HUS

351
Q

Contains fibrinogen, factor VIII, factor XIII, vWF, and fibronectin

A

Cryoprecipitate

352
Q

Clinical use for Cryoprecipitate

A

Treat coagulation factor deficiencies involving fibrinogen and factor VIII

353
Q

What is the dosage effect of Cryoprecipitate?

A

Contains fibrinogen, factor VIII, factor XIII, vWF, and fibronectin

354
Q

Which are the blood transfussion risks?

A

Infection transmission (low)
Transfusion reactions
Iron overload
Hypocalcemia (citrate is a calcium chelator)
Hyperkalemia (RBCs may lyse in blood units)

355
Q

What is Leukemia?

A

Lymphoid or myeloid neoplasms with widespread involvement of bone marrow

356
Q

Where are tumor cells ussually found in Leukemia?

A

In peripheral blood

357
Q

What is Lymphoma?

A

Discrete tumor masses arising from lymph nodes

358
Q

How is lymphoma often presented?

A

Blur definitions

359
Q

What is leukemoid reaction?

A

Acute inflammatory response to infection

360
Q

Labs in Leukemoid reaction

A

↑ WBC count with ↑ neutrophils and neutrophil precursors such as band cells (left shift)
↑ Leukocyte ALP

361
Q

Contrast of Leukemoid reaction with CML

A

Both have ↑ WBC count with left shift, but ↓ Leukocyte ALP

362
Q

Characteristics of Hodgkin lymphoma

A

Localized, single group of nodes; extranodal rarecontigous spread

363
Q

Which is the stronger predictor of prognosis in Hodgkin lymphoma?

A

Contigous spread

364
Q

Who has better prognosis between non Hodgkin and Hodgkin lymphoma?

A

Hogdkin lymphoma has better prognosis

365
Q

Which cells are characteristic of Hodgkin lymphoma?

A

Reed Sternberg cells

366
Q

Groups of age that present more Hodgkin lymphoma?

A

Bimodal distribution- young adulthood and > 55 years

367
Q

Which sex is at higher risk of Hodgkin lymphoma?

A

More common in men except Nodular sclerosing type

368
Q

What factor is associated to Hodgkin lymphoma?

A

50 % of cases associated with EBV

369
Q

Clinical findings of Hodgkin lymphoma

A

Constitutional (“B”) signs/ symptoms -low- grade fever, night sweats, weight loss

370
Q

Characteristics of Non Hodgkin lymphoma

A

Multiple, peripheral nodes; extranodal involvement common; non contigous spread

371
Q

Which cells are characterisitic of Non Hodgkin lymphoma?

A

Majority involve B cells (except those of lymphoblastic T cell origin)

372
Q

Group of age for Non Hodgkin lymphoma

A

Peak incidence for certain subtypes at 20-40 years old

373
Q

Factors associated to Non Hodgkin lymphoma

A

May be associated with HIV and immunosuppresion

374
Q

Clinical findings of Non Hodgkin lymphoma

A

Fewer constitutional sign/ symptoms

375
Q

What are the Reed-Sternberg cells?

A

Distinctictive tumor giant cell seen in Hodgkin disease

376
Q

Characteristics of Reed-Sternberg cells

A

Binucleate or bilobed with the 2 halves as mirror images (“owl eyes”)

377
Q

Which CD do Reed Sternberg cells have?

378
Q

Cell origin of Reed-Sternberg cells

379
Q

Are Reed-Sternberg cells necessary for diagnosing Hodgkin disease?

A

Necessary but not sufficient for a diagnosis of Hodgkin disese

380
Q

Which microscopic characteristics have better prognosis for Hodgkin disease?

A

Strong stromal or lymphocytic reaction against RS cells

381
Q

Most common form of Hodgkin lymphoma

A

Nodular sclerosing

382
Q

Which sex group is more affected by Nodular Sclerosing Hodgkin lymphoma?

A

Women and men equally

383
Q

Which type of Hodgkin lymphoma has better prognosis?

A

Lymphocyte rich form

384
Q

Which types of Hodgkin lymphoma have poor prognosis?

A

Lymphocyte mixed or depleted forms have poor prognosis

385
Q

How are Non Hodgkin lymphoma classified?

A

Neoplasm of Mature B cells

Neoplasm of mature T cells

386
Q

Non Hodgkin lymphoma of mature B cells

A

Burkitt lymphoma
Diffuse large B cell lymphoma
Mantle cell lymphoma
Follicular lymphoma

387
Q

In whom is more common Burkitt lymphoma?

A

Adolescents or young adults

388
Q

Which gene is affected in Burkitt lymphoma?

A

t (8; 14)- translocation of c-myc (8) and heavy chain Ig (14)

389
Q

Microscope characteristics of Burkitt lymphoma

A

“Starry sky” apperacne, sheets of lymphocytes with interpersed macrophages

390
Q

What is associted to Burkitt lymphoma?

391
Q

Clinical manifestations of Burkitt lymphoma

A

Jaw lession in endemic form in Africa

Pelvis or abdomen in sporadic form

392
Q

Usual group of age of presentation of Diffuse large B cell lymphoma

A

Usually Older adults, but 20% in children

393
Q

Gen affected in Diffuse large B cell lymphoma

394
Q

Most common type of non Hodgkin lymphoma in adults

A

Diffuse large B cell lymphoma

395
Q

Who are more affected by Mantle cell lymphoma?

A

Older males

396
Q

Gen affected in Mantle cell lymphoma

A

t (11; 14)- translocation of cyclin D1 (11) and heavy chain Ig (14)

397
Q

CD found in Mantle cell lymphoma

398
Q

Who are at higher risk for Follicular lymphoma?

399
Q

Gen affected in Follicular lymphoma?

A

t (14; 18)- translocation of heavy chain Ig (14) and bcl-2

400
Q

How is the course of Follicular lymphom?

401
Q

What is the effect of bcl2?

A

Inhibits apoptosis

402
Q

How is Follicular lymphom presentation?

A

With painless “waxing and waning” lymphadenopathy

403
Q

Non Hodgkin Lymphoma of mature T cells

A

Adult T cell lymphoma

Mycosis fungoides

404
Q

In which patients os more often seen Adult T cell lymphoma?

405
Q

What causes Adult T cell lymphoma? with what is associated?

A

By HTLV-1

associated with IV drgu abuse

406
Q

Who are mainly affected by Adult T cell lymphoma?

A

Populations of Japan, West Africa, and the Caribean

407
Q

How is Adult T cell lymphoma manifested?

A

Adult present with cutaneois lesions

408
Q

How are bones affected by Adult T cell lymphoma?

A

Lytic bone lesions, hypercalcemia

409
Q

How else is Mycosis Fungoide known?

A

Sézary syndrorme

410
Q

Group of age affected by Mycosis Fungoide

411
Q

How is Mycosis Fungoide presented?

A

Adults present with cutaneous patches plaques/ tumors with potential to spread to lymph nodes and viscera

412
Q

What is seen in Sézary syndrome?

A

Circulating malignant cells seen in Sézary syndrome

413
Q

How is the course of Sézary syndrome?

414
Q

What CD is seen in Mycosis Fungoides?

415
Q

Origin Cells from Multiple Myeloma

A

Monoclonal plasma cell

416
Q

What apperance do monoclonal plasma cell have?

A

“fried egg” apperance

417
Q

From where does Multiple Myeloma arise?

A

In the marrow

418
Q

What does Multiple Myeloma produce?

A

Large amounts of IgG (55%) or IgA (25%)

419
Q

Most common primary tumor arising within bone in the eldery

A

Multiple myeloma

420
Q

Main Group of age affected by Multiple myeloma

A

> 40-50 years old

421
Q

Which factors are associated to Multiple myeloma?

A 
↑ susceptibility to infection
Primary amyloidosis (AL)
422
Q

What is seen in imaging studies in Multiple myeloma?

A

Punched out lytic bone lesions on x ray

423
Q

What in seen in serum protein electrophoresis in Multiple Myeloma?

424
Q

What is seen in urine in Multiple Myeloma?

A

Ig light chains in urine (Bence Jones protein)

425
Q

Protein found as Ig light chains in urine

A

Bence Jones protein

426
Q

What is seen in smear in Multiple Myeloma?

A

Rouleaux formation

427
Q

What is Rouleaux formation?

A

RBCs staked like poker chips in blood smear

428
Q

Characteristics of plasma cells seen in Multiple Myeloma

A

Numerous plasma cells with “clock face” chromatin and intracytoplasmic inclusions containing immunoglobulin

429
Q

What difference does Multiple Myeloma has with Waldenstrom macroglobulinemia?

A

M spike= IgM (→ hyperviscocity symptoms)

No lytic bone lesions

430
Q

With which disease is Monoclonal gammopathy of undetermined significance related to?

A

Asymmptomatic precursor to Multiple myeloma

431
Q

What is Monoclonal gammopathy of undetermined significance?

A

Monoclonal expansion of plasma cells with serum monoclonal protein

432
Q

How much patients with Monoclonal gammopathy of undetermined significance develop Multiple Myeloma?

A

1-2% per year

433
Q

Findings of Multiple myeloma

A 
CRAB
hyperCalcemia
Renal insufficiency
Anemia
Bone lytic lesions/ Back pain
434
Q

What are Myelodysplastic syndromes?

A

Stem cell disorders involving inneffective hematopoiesis → defects in cell maturation of all non lymphoid lineages

435
Q

What causes Myelodysplastic syndromes?

A

Caused by de novo mutations or environmental exposure

436
Q

Which environmental exposure cause Myelodysplastic syndromes?

A

Radiation, Benzene, chemotherapy

437
Q

What risk do Myelodysplastic syndromes have?

A

Risk of transformation to AML

438
Q

What is Pseudo Pelger Huet anomaly?

A

Neutrophils with bilobed nuclei (two nuclear masses connected with a thin filament of chromatin) typically seen after chemotherapy

439
Q

Pseudo Pelger Huet anomaly is associated to this disease

A

Myelodysplastic syndrome

440
Q

Pathophysiology of Leukemias

A

Unregulated growth of leukocytes in bone marrow → ↑ or ↓ number of circulating leukocytes in blood and marrow failure

441
Q

What is the result of Leukemias?

A

Anemia (↓ RBCs), infections (↓ mature WBCs) and hemorrhage (↓ platelets)

442
Q

Which structures could be infiltrated by Leukemic cells?

A

Liver, Spleen and lymph nodes

443
Q

Types of Leukemias

A

Lymphoid neoplasms

Myeloid neoplasms

444
Q

Who are lymphoid neoplasms?

A

Acute lymphoblastic leukemia/ lymphoma (ALL)
Small lymphocytic lymphoma (SLL)/ Chronic lymphocytic leukemia (CLL)
Hairy Cell leukemia

445
Q

At which age does Acute Lymphoblastic leukemia commonly appears?

446
Q

How is T cell ALL presented?

A

Mediastinal mass (leukemic infiltration of the thymus)

447
Q

Which disease is associated to ALL?

A

Down syndrome

448
Q

Findings of ALL

A

Peripheral bloof and bone marrow have ↑↑↑ lymphoblasts

449
Q

Special biomarkers for ALL

A

TdT+

CD10+

450
Q

What is the use for TdT+?

A

Marker of pre-T and pre-B cells

451
Q

What does CD10 identifies?

A

pre-B cells only

452
Q

Which Lymphoid neoplasm is the most responsive to therapy?

A

Acute lymphoblastic leukemia/ lymphoma (ALL)

453
Q

Acute lymphoblastic leukemia/ lymphoma (ALL) may spread to…

A

CNS and testes

454
Q

Mutations presented in Acute lymphoblastic leukemia/ lymphoma (ALL)

A

t (12; 21)

455
Q

Prognosis of t (12;21) mutation

A

Better prognosis

456
Q

How else is Chronic lymphocytic leukemia known?

A

Small lymphocytic lymphoma (SLL)

457
Q

At what age does CLL commonly appears?

A

> 60 years

458
Q

What CD are seen in CLL?

A

CD20+, CD5+ B cell neoplasm

459
Q

How is the progression of SLL?

A

Progresses Slowly

460
Q

Symptoms of SLL

A

Often asymptomatic

461
Q

What is seen in peripheral blood smear in CLL?

A

Smudge cells

462
Q

What else is found in CLL?

A

Hemolytic anemia

463
Q

What is the difference between CLL and SLL?

A

SLL same as CLL except CLL has increasedperipheral blood lymphocytosis or bone marrow involvement

464
Q

Who are at higher risk for Hairy cell leukemia?

465
Q

How is Hairy cell leukemia consider?

A

Mature B cell tumor in the elderly

466
Q

Characteristic of cells in Hairy cell leukemia?

A

Cells have filamentous, hairy like projections

467
Q

Which stain is positive in Hairy cell leukemia ?

A

TRAP (tartrate resistant acid phosphate)

468
Q

By which method is TRAP replaced inthe study of Hairy cell leukemia?

A

With flow cytometry

469
Q

What do Hairy cell leukemia causes?

A

Marrow fibrosis→ dry tap on aspiration

470
Q

Treatment for Hairy cell leukemia

A

Cladribine (2-CDA), an adenosine analog (inhibits adenosine deaminase)

471
Q

Who are Myeloid neoplasms?

A

Acute myelogenous leukemia (AML)

Chronic Myelogenous leukemia (CML)

472
Q

Age of onset of Acute myelogenous leukemia?

A

Median onset 65 year

473
Q

In which disease are Auer rods seen?

A

Acute myelogenous leukemia

474
Q

What are Auer rods?

A

Peroxidase + cytoplasmic inclusions seen mostly in M3 AML

475
Q

What is seen in blood smear in Acute myelogenous leukemia?

A

↑↑↑ circulating myeloblasts on peripheral smear

476
Q

Risk factor for Acute myelogenous leukemia

A

Prior exposure to alkylating chemotherapy, radiation, myeloproliferative disordes, Down syndrome

477
Q

Type of mutation seen in Acute myelogenous leukemia

A

t(15; 17)→ M3 AML subtype

478
Q

M3 AML subtype responds to…

A

responds to all-trans retinoic acid (vitamin A)

479
Q

What do trans retinoic acid (vitamin A) causes in M3 AML subtype?

A

Inducing differentiation of myeloblasts

480
Q

What pathology could be present in M3 AML subtype?

481
Q

What induces DIC in M3 AML subtype?

A

Chemotherapy due to release of Auer rods

482
Q

Peak incidence of Chronic Myelogenous leukemia (CML)

A

45-85 years

483
Q

Median age of diagnosing Chronic Myelogenous leukemia (CML)

484
Q

Which chromosome is related to Chronic Myelogenous leukemia (CML)?

A

Philadelphia chromosome

485
Q

Philadelphia chromosome

A

t (9;22), bcr-abl

486
Q

What does Philadelphia chromosome define?

A

Myeloid stem cell proliferation

487
Q

How is Chronic Myelogenous leukemia (CML) presented?

A

↑ neutrophils, metamylocytes, basophils

488
Q

Clinical finging of Chronic Myelogenous leukemia (CML)

A

Splenomegaly

489
Q

What could be the probable evolution of Chronic Myelogenous leukemia (CML) ?

A

May accelerate and transform to AML or ALL (“blast crisis”)

490
Q

What is a blast crisis?

A

When Chronic Myelogenous leukemia transforms to AML or ALL

491
Q

Other characteristic of Chronic Myelogenous Leukemia

A

Very low leukocyte alkaline phosphatase (LAP) as a result of low activity in mature granulocytes

492
Q

What difference does Chronic myelogenous leukemia has with Leukomoid reaction

A

CML has ↓ Leukocyte Alkaline phosphatase

Leukemoid reaction presents with ↑ Leukocyte Alkaline phosphatase

493
Q

Which is the treatment for Chronic myelogenous leukemia?

A

Responds to imatinib

494
Q

What is imatinib?

A

A small molecule inhibitor of the bcr-abl tyrosine kynase

495
Q

Which translocation is Philadelphia chromosome?

496
Q

Associated disorder to Philadelphia chromosome

A

CML (bcr-abl hybrid)

497
Q

Pathology associated to t(8:14)

A

Burkitt lymphoma

498
Q

What is activated in Burkitt lymphoma?

A

c-myc activation

499
Q

Translocation of Mantle cell lymphoma

500
Q

Associated disorder to cyclin D1 activation

A

Mantle cell lymphoma

501
Q

Disorder associated to t(14; 18)

A

Follicular lymphomas

502
Q

What is activated in Follucular lymphomas?

A

bcl-2 activation

503
Q

Pathology associated to t(15; 17)

A

M3 type pf AML (responsive to all trnas retinoic acid)

504
Q

Proliferative disorders of dendritic cells from nonocyte lineage

A

Langerhans cell histiocytosis

505
Q

Alternative name for dendritic cells

A

Langerhans cell

506
Q

How is Langerhans cell histiocytosis presented?

A

Presents in child as lytic bone lesions and skin rash or as recurrent otitis media with mass involving the mastoid bone

507
Q

What is the problem with dendritic cells in Langerhans cell histiocytosis?

A

Cells are functionally immature and do not efficiently stimulate primary T lymphocytes via antigen presentation

508
Q

What is expressed by cells in Langerhans cell histiocytosis?

A

S-100 (mesodermal origin) and CD1a

509
Q

Histologic characteristic of Langerhans cell histiocytosis

A

Birbeck granules (“tennis rackets”)

510
Q

Chronic myeloproliferative disorders

A

Polycythemia vera
Essential thrombocytosis
Myelofibrosis
CML

511
Q

Non-receptor tyrosine kinase mutated in Chronic myeloproliferative disorders

512
Q

What is the function of JAK2?

A

Is involved in hematopoietic growth factor signaling

513
Q

Which myeloproliferative disorder in not implicated in mutations of JAK2?

514
Q

Main characteristics of Polycythemia vera

A

Hematocrit > 55%, somatic (non heraditary) mutation in JAK2 gene

515
Q

How is Polycythemia often presented?

A

As intense itching after hot shower

516
Q

Rare but classic symptom of Polycythemia vera

A

Erythromegalia

517
Q

What is Erythromegalia?

A

Severe, burning pain and reddish or bluish coloration

518
Q

What causes Erythromegalia in Polycythemia vera?

A

Episodic blood clots in vessels of the extremities

519
Q

What induces a secondary polycythemia?

A

Via natural or artificial ↑ in EPO levels

520
Q

Which pathology is similar to Essential thrombocytosis?

A

Polycythemia vera

521
Q

What is the main problem of Essential thrombocytosis?

A

Specific for overproduction of abnormal platelets

522
Q

What is the result of Overproduction pf abnormal platelets?

A

Bleeding, thrombosis

523
Q

What does Bone marrow contains in Essential thrombocytosis?

A

Enlarged megakaryocytes

524
Q

Main characteristic of Myelofibrosis

A

Fibrotic obliteration of bone marrow

525
Q

Cell characteristics on Myelofibrosis

A

Teardrop RBCs and immature forms of the myeloid line

526
Q

In this chronic myeloproliferative disorder we see bcr-abl transformation

527
Q

What does bcr-abl transformation leads to in CML?

A

↑ cell division and inhibition of apoptosis

528
Q

Imatinib is the drug of choice in this myeloproliferative disorder

529
Q

Comercial name for Imantinib

530
Q

In blood counts how are cell affected in Polycythemia vera?

A

↑ RBCs
↑ WBCs
↑ Platelets

531
Q

What mutation is seen in Polycythemia vera?

532
Q

What cells are affected in Essential thrombocytosis?

A

↑ Platelets

533
Q

Is JAK2 mutated in Essential thrombocytosis?

A

Yes, in 30-50%

534
Q

How are blood counts in Myelofibrosis?

A

↓ RBCs

Variable WBCs and Platelets

535
Q

Is JAK2 mutated in Myelofibrosis?

A

Yes, in 30-50%

536
Q

How are blood count in CML?

A

↓ RBCs
↑ WBCs
↑ Platelets

537
Q

Is JAK2 mutated in CML?

538
Q

Which gene is mutated in CML?

A

Philadelphia chromosome

539
Q

Types of Polycythemias

A

Relative
Appropriate absolute
Inappropriate absolute
Polycythemia vera

540
Q

In relative polycythemia what is the problem?

A

↓ plasma volume

541
Q

What leads to relative polycythemia?

A

↓ plasma volume (dehydration, burns)

542
Q

What is the result of appropriate absolute polycythemia?

A

↑ RBC mass

↑ EPO levels

543
Q

What is the main cause of appropriate absolute polycythemia?

A

↓ O2 saturation

544
Q

What pathologies are associated to appropriate absolute polycythemia?

A

Lung disease, congenital heart disease, high altitude

545
Q

What is seen in inappropriate absolute polycythemia?

A

↑ RBC mass

↑ EPO levels

546
Q

Main causes of inappropriate absolute polycythemia

A 
Renal Carcinoma
Wilms tumor
Cyst
Hepatocellular carcinoam
Hydronephrosis
547
Q

Main cause of inappropriate absolute polycythemia

A

Due to ectopic EPO

548
Q

What are the alterations seen in Polycythemia vera?

A

↑ Plasma volume
↑↑ RBC mass
↓ EPO levels

549
Q

Why is EPO decreased in Polycythemia vera?

A

Due to negative feedback

HEMATOLOGY AND ONCOLOGY (4 decks)

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